About Thalassaemia - Khmer by Thalassaemia International Federation (TIF)

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1/12/2008

9:48 AM

Page 1

esovePAenH epþateTAelItMrUvkarrbs;GñkCMgWtaLasesmIu. ral;kic©xitxMBiBN’naGMBICMgW nig lkçN³rbs;va RtUv)aneFVItamrebobgay² ehIy)anbBa©úÚlbEnßmnUvkareCOnelOnEpñkKøInik nig viTüasaRsþfμI² Edl)anrkeXIjkñúgb:unμanqñaMcugeRkayenH kñúgkarBüa)al nig karBarCMgWtaLasesmIu. karpþl;nUvBt’manfμI² nig RtwmRtUvdl;GñkCMgWtaLasesmIuCaTUeTA kñúgsklelak CakmμvtßúmYykñúgcMeNamkmμvtßúcMbg² nig CaehtuplnaMeGaybegáItCa shB½n§GnþrCatiCMgWtaLasesmIu enHeLIg. esovePAenHRtUv)anERbsMrYlCaexmrPasa nig e)aHBum<pSayeday mnÞIreBTükumarCati 100/ mhavifI shB½n§rusSIú/ xN½ÐTYleKak/ PñMeBj/ km<úCa GnuBaØatisiT§ibkERbBIÉksaredIm nig pþl;mUlniFieday shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ Thalassaemia International Federation (TIF) P.O. Box 28807, 2083 Nicosia, 31 Ifigenias Str., 2007 Nicosia, Cyprus Website: http://www.thalassaemia.org.cy

ERbsMrYlCaexmrPasa evC¢bNÐit Can supl

evC¢bNÐit tU eBCra

RKUeBTüÉkeTseraKkumar nig elahitsaRsþ mnÞIeBTükumarCati PñMeBj

naykrgTTYlbnÞúkbec©keTs mnÞIreBTübEg¥kkMBg;RtEbk extþéRBEvg

ISBN : 9963-623-40-9

ERbsMrYlCaexmrPasa nig e)aHBum<enAPñMeBj km<úCa qñaM 2008

Cover text.qxp

1/12/2008

9:48 AM

Page 2

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ sUmEføgGMNrKuNy:ag RCaleRCAcMeBaHkarpþl;viPaKTand¾sb,úrsBIshB½n§ Cooley’s anemia énshrdæGaemrik sMrab;eKalbMNgénkare)aHBum<pSayenH.

rkSasiT§iRKb;y:ag KμanEpñkNamYyénesovePAenHGacRtUvplitCafμI rkSaTukkñúgRbB½n§Gacrk)an b¤ bBa¢ÚneTAkñúgTMrg;NamYy b¤ edaybMNgNamYy dUcCaeGLicRtUnik emkanik ftcMlg mIRkUhVIlm_ b¤ tamEbbepSg²edayKμankarGnuBaØat Calayl½kçN_GkSrBIshB½n§GnþrCatiCMgWtaLasesmIu nig GñkniBn§. e)aHBum<enAnIkUsSa/ sIuRBUs (Printed in Nicosia, Cyprus)

GMBIGñkniBn§ evC¢> Gg;RDULa eGElhVehrIuyU (Dr. Androulla Eleftheriou) )anTTYl sBaØab½Rt]tþmsikSa nig eRkay]tþmsikSaBIsaklviTüal½yLúgdugkñúgvis½y GtisuxumCIvsaRsþ (Microbiology) nig visaNUsaRsþ (Virology). elakRsI )anTTYlrgVan;CaGaharUbkrN_mYycMnYn BIGgÁkarsuxPaBBiPBelak nigBI KN³kmμkarhVÚlR)ay (Fulbright Commission)/ ehIyelakRsI)ankan; tYnaTICaGñkRsavRCavkitþiys enAsaklviTüal½yevC©saRsþ cRkPBGgÁeKøs. GaharUbkrN_fñak;bNiÐtrbs;elakRsIRtUv)anbMeBjenAmCÄmNÐlRtYtRta CMgW (Centres of Disease Control) naTIRkugGatøg;ta shrdæGaemrik. evC¢> eGElhVehrIuyU )ankan;tYnaTICaRbFanmCÄmNÐl visaNUbEg¥k (Virus Reference Centre) énRksYgsuxaPi)al RbeTssIuRBUs (Cyprus) cab;taMgBIqñaM 1990 ehIyCaGñkrYmcMENkcMbg kñúgkarbegáItmCÄmNÐlenH pgEdr. elakRsI)anerobcM nig)ancUlrYmy:agskmμkñúgsikçasala snñisiT nigKMeragTaMgfñak;Cati nigGnþrCatiCaeRcIn. bc©úb,nμelakRsIbMerIkarCaTIRbwkSa GgÁkarsuxPaBBiPBelak elIvis½yCMnajrbs;Kat;. karcab;GarmμN_sMxan; nigkargarsikSaRsavRCavrbs; evC¢> eGElhVehrIuyU KWkñúgvis½yénkarbgáeraKedayvisaNU (Viral infections) kñúgCMgWtaLasesmIu kMritF¶n;. tamry³karsikSaRsavRCavrbs;Kat; elakRsI)anTMnak;TMngCamYy RKUeBTü nig GñkviTüasaRsþxagkarRsavRCavCaeRcInEdleFVIkarkñúgvis½yenH RBmTaMgCamYyGñkCMgWtaLasesmIuenAbNþaRbeTsnanakñúgBiPBelak. cab;BIqñaM 1993 evC¢> eGElhVehrIuyU )aneFIVshkarCaGñksμ½RKcitþCamYy shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦. tamry³kare)aHBum<pSayrbs;xøÜn s>G>t bMeBjkargareFVIeGayR)akdfaral; Bt’manevC¢saRsþGMBICMgWtaLasesmIugaynwgrk)anedayRsYl. i

sarsMxan;CagenH s>G>t pþl;»kasdl;buKÁlikeBTüeGaysikSabnþGMBI CMgWtaLasesmIutamry³sikçasilaGb;rM. kñúgqñaM 1997 tMENgfμImYyCaGñksMrbsMrYlviTüasaRsþrbs; s>G>t RtUv)an begáIteLIgedIm,IemIlxusRtUvkic©karxagelIenH. evC¢> eGElhVehrIuyU RtUveKEtgtaMgeLIgedayÉkcän© - elakRsI)anTTYlyktYnaTIenHeday hrT½yrIkrayGs;BIdYgcitþ. cab;taMgBIeBlenaHmk evC¢> eGElhVehrIuyU )ankan;tYnaTICaGñksMrbsMrYlviTüasaRsþenHedaybMeBjkargarrbs;elakRsI edayELkBItYnaTIpøÚvkarkñúgrdækar. tamry³kargarrbs;elakRsICamYy s>G>t/ evC¢> eGElhVehrIuyU )anGnuvtþKMeragCaeRcIn TaMgkñúgRsuk nig GnþrCati kñúgshRbtibtþikareday CitsñiT§CamYybNþaRKUeBTü nigsmaKmCMgW taLasesmIunanaBaseBj BiPBelak. elakRsI)anniBn§ nig e)aHBum<pSayesovePAmYycMnYnkñúgnam s>G>t RBmTaMgmYycMnYneTot edayshRbtibtþikarCamYyGgÁkarsuxPaB BiPBelak nig GgÁkardéT²eTot GMBIRbFanbTCaeRcInEpñkviTüasaRsþ ehIy elakRsIk¾CaniBn§naykTsSnavdþIRbcaMRtImasrbs; s>G>t.

esckþIEføgGMNrKuN xJúMsUmEføgGMNrKuNcMeBaHkarpþl;viPaKTanBI evC¢> mIxaEGl Gg;háasÞIejaTIs TIRbwkSaevC¢saRsþrbs; shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ Edl)anBinitüesovePAenH ehIy)anpþl;karENnaMCMnaj nig Gnusasn_ RBmTaMgkarTUnμand¾mantMél. xJúMsUmEføgGMNrKuNcMeBaHkarpþl;viPaKTanBIGñkCMnajkarevC¢saRsþxageRkam EdlCasmaCik smaCIka énRkumRbwkSaPi)alrbs; s>G>t Edl)anBinitüEpñk nanatamÉkeTsCMnajrbs;BYkeK ehIy)anpþl;Gnusasn_ RBmTaMgkarTUnμan d¾mantMél. saRsþacarü cn B½rT½r evC¢> b:Ul EtlhV½r GaKarEpñkelahitsaRsþ sklviTüal½yLúgdug 98 Chenies Mews London WC1E 6HX

GñkÉkeTselahitsaRsþ mCÄmNÐltaLasesmIu RksYgsuxaPi)al nIkUsüa/ sIuRBUs (Nicosia, Cyprus)

evC¢> m:alkUl vkX½r

evC¢> nIkUs sá½rDIs

GñkÉkeTsebHdUgsaRsþ viTüasßan hatF½r GaKar sIusIl PøImIg mnÞIreBTüsklviTüal½yLúgdug

RKUeBTükumar-GñkÉkeTsRkeBj GaKarEpñkeraKkumar mnÞIreBTü m:akarIuyU RksYgsuxaPi)al nIkUsüa/ sIuRBUs (Nicosia Cyprus)

Grafton Way, London WC1E 6AU, UK

iii

saRsþacarü nIka kaBEBllInI GUes<dal m:akC½r b:UlIKøInIkU/ )a:DIKøIGUn RkaenlI vIy:a sV½rsa 35/ mILaNU 20122 GIutalI (Milano 20122 Italy)

CMnYy]btßmÖd¾mantMélBI evC¢> s> RKIsÞÚ nigelakRsI G> KarI GñkviTüasaRsþ mnÞIrBiesaFn_ TaMgBIrnak;CabuKÁlikmCÄmNÐltaLasesmIu enAnIkUsüa RksYgsuxaPi)alsIuRBUs Edlpþl;søay nigtaragmYycMnYnEdleRbIkñúg esovePAenH. CMnYy]btßmÖCMnajd¾mantMélBI evC¢> ehELn EBrI buKÁlik s>G>t kñúgkarBinitüepÞógpÞat; nig kñúgkarerobcMsTÞanuRkménesovePAenH.

kar]TÞis sUm]TÞisesovePAenH cMeBaHGñkCMgWtaLasesmIuRKb;²Kña enABaseBj BiPBelak EdlGñkTaMgenaH xJúM)ansÁal; nig)anCYb ehIyxøHeTotxJúMsÁal; Et minTan;)anCYbenAeLIy RBmTaMgrab;ryBan;nak;epSgeTot EdlkMBugrs;enA BaseBjBiPBelak EdlxJúMnwgKμan»kas)anCYb. karebþCJarbs;BYkeKedIm,I rs;enA nigxMBuHBar )anpþl;eGayxJúMnUvcMNg; nigkMlaMgcitþ edIm,IcUlrYmkñúgkar ts‘URbyuT§nwgCMgWenH. esckþIkøahand¾FMeFg ehIynigkarGt;Fμt;KμanTIbBa©b; rbs;BYkeK)anbeRgonxJúMfaetIRtUvts‘URbyuT§edaymincuHcaj;EbbNa.

matikaerOg

CMBUkTI 1

buBVkfa lixitBIGñkniBn§ Qam nig taLasesmIu Qam - {sÞwgCIvit} tYnaTIrbs;Qam FatupSMrbs;Qam QamTaMgmUl RbePTQam ¼RkumQam CMgWQam CMgWtaLasesmIukMritF¶n;

CMBUkTI 2

xii xiv 1 1 1 3 4 7 9 10

Bn§útMNBUC nig taLasesmIu

EhSn (Genes) = Bn§ú PaBminRbRktIkgúñ eKalikaQamRkhmEdlnaMeGayekItCMgW taLasesmIukMritF¶n; eGm:UkøÚb‘Ín nig Edk Bn§úEdlRKb;RKgkarsMeyaKkøÚb‘Ín eraKsrIrsaRsþ rbs;CMgWtaLasesmIu (Pathophysiology of

15 15 17

Thalassemia)

kgVHExSEbtta (β - chains) ehIyelIsExSGal;haV (α - chains) kMNt;eraKvinicä½yCMgWtaLasesmuI viFIsaRsþEpñkelahitviTüa EdleKeRbICaTUeTA edIm,IeFVIeraKvinicä½ytaLasesmuIkMritF¶n;

20 23

CMBUkTI 3

CMBUkTI 4

karBüa)al Ebtta taLasesmuIkMritF¶n;

karBüa)aledaykarbBa©ÚlQam

etIeBlNaRtUvcab;epþImBüa)aledaybBa©ÚlQam Fana[)ankarbBa©ÚlQammansuvtßiPaB ehSNUTIbRkumQam (Blood group genotype) etIQamGVIRtUvbBa©Úl kMNt;rbbbBa©ÚlQam vaytMélRbsiT§iPaBénrbbbBa©ÚlQam karBüa)alRbtikmμbBa©ÚlQam

29 30 31 32 37 39 44

elIsbnÞúkCatiEdk nig karkMcat;CatiEdkecj

EdkenAkñúgsarBagÁkayGñkmansuxPaBl¥ EdkkñúgCMgWtaLasesmIu etIkarelIsCatiEdkbMpøajragÁkayya:gdUcemþc Desferrioxamine (DFO) b¤ Desferal etIeKcab;epþImBüa)alkMcat;CatiEdkeBlNa meFüa)ayepSg²eTotkñúgkarcak;bBa©ÚlfñaM DFO vaytMélRbsiT§iPaBénkarBüa)aledayfñaM DFO eRbIkMrit Ferritin edIm,IkMNt;kMriteRbI DFO CatiEdkkñúgTwkenam plvi)akcMeBaHkareRbIfñaM DFO ehIyvabgðajeLIgEbbNa karmanKP’ karsMrbtamcMeBaHkarBüa)aledayfñaM DFO fñaMkMcat;CatiEdk epSg²eTot RbsiT§iPaBrbs;fñaM Deferiprone plrMxanrbs;fñaM L1

49 50 52 53 55 58 61 62 65 65 71 71 72 72 73

vii

plrMxanepSg²eTotrbs; Deferiprone kareRbIfñaMkMcat;CatiEdkedaypÁúMcUlKña fñaMfμI Deferasirox CaRbePTRKab;elbtammat; pøÚveRbIR)as;fñaM etI Deferasirox dMeNIrkardUcemþc RbsiT§PaBrbs;fñaM plrMxanrbs;fñaM Deferasirox bMrameRbIR)as; (Contra-indications) karmanépÞeBaH nig kareRbIfñaM Deferasirox

CMBUkTI 5

bBaðaevC¢saRsþbNþalmkBItaLasesmuI nig karBüa)al GeNþIkrIkFM karkat;GeNþIkecj nig karbgáeraK plvi)akcMeBaHebHdUg nig RkeBjbeBa©jkñúg (Endocrine) plvi)akcMeBaHebHdUg tamdanmuxgarebHdUg plvi)akcMeBaHRkeBjbeBa©jkñúg

CMBUkTI 6

viii

74 76 77 78 79 79 79 80 81

82 82 84 86 86 88 91

karbgáeraKkñúgCMgWtaLasesmuI

101

karbgáeraKbNþalmkBIkarbBa©ÚlQam karbgáeraKedayvIrusrlakeføImRbePT B

103

(Hepatitis B = HBV)

104

rlakeføImRbePT vIrusepSg²eTot Pñak;garbgáeraKfμI

107

C (Hepatitis C = HCV)

112 114

CMBUkTI 7

CMBUkTI 8

karRBYy)armμN_fμI cMeBaHPñak;garbgáeraKcas; suvtßiPaBQam suvtßPi aB nig manQameRbIR)as; enAbNþaRbeTskMBgu GPivDÆn_ segçbbBaðaEdlGacekItman cMeBaHGñkCMgwtaLasesmuI kMritF¶n;Edlmin)anBüa)al

114

taLasesmIukMrti mFüm nig taLasesmIuepSg²eTot

119

kareFVIeraKvinicä½y karBüa)al taLasesmIukMritmFüm karkMcat;CatiEdk bBaðaEpñkevC¢saRsþkñúgCMgWtaLasesmIukMritmFüm taLasesmIubNþalmkBI {eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘ÍnEdlmanrcnasm<½n§køay lkçN³GUm:UhSIúháÚt rbs;eGm:UkøÚb‘ÍnxusFmμta PaBpÁúMKñaén {eGm:UkøÚb‘ÍnxusFmμta} CamYy Ebtta taLasesmIu Gal;hVa ¬Alpha¦ taLasesmIu

121

rbbBüa)alEdl)aneFVI nig CMhan naeBlGnaKt karbnSaMxYrq¥wg GVIeTACakarbnSaMxYrq¥wg ? etImaneRKaHfñak;GVIxøH ? etIeKGacbnßyeRKaHfñak;[enAticbMput)andUcemþc ? CMhanepSg²eTot cMeBaHkarbnSaMxYrq¥wgEdlmanPaBsIuKña BitR)akd ¬Cab;sac;Qam¦

115

117

118

123 124 125

128 128

129 130

131 131 131 132 133

136

CMBUkTI 9

esckþIsnñidæan bnSaMxYrq¥wgedayeRbIQamTgsuk

137

CMhanepSg²eToteq<aHeTArkkarBüa)al »sfCMrujbegáIt eGm:UkøÚb‘ÍnTarkkñúgépÞ (HbF inducers)

139

Büa)aledayBn§ú (Gene therapy) eraKvinicä½yrkPaBsIuKñaén HLA munkarbgákMeNIt

140

CMBUkTI 10 bBaðaecaTEpñkcitþsgÁm cMeBaHGñkCMgW cMeBaH«BukmþayGñkCMgW cMeBaHGñkCMgW nig «Bukmþay TTYlBt’man nig karKaMRT GñkCMgWtaLasesmIukMritF¶n; Cakumar CaekμgCMTg; nig CamnusSeBjv½y

CMBUkTI 11 Bt’bEnßm karrs;ran nig KuNPaBCIvit rbbGahar nig taLasesmIu kILa nig taLasesmIu visSmkal nig taLasesmIu karGaBah¾BiBah¾ nig RKYsar

CMBUkTI 12 eGBIedmIsaRsþ nig karkarBarCMgWtaLasesmIu eGBIedmIsaRsþ karkarBar sarsMxan;énkarkarBar x

137

139

140

141 141 141 142 143

144

147 147 149 149 150 150

152 152 152 152

rebobEdltaLasesmIucMlgCalkçN³tMNBUC GñkpÞúktaLasesmIukMritRsal ehtuGVIGñkcaM)ac;RtUvdwgfa etIxøÜnGñk CaGñkpÞúkCMgW b¤ Gt; eGm:UkøÚb‘ÍnxusFmμta nig CMgWeGm:UkøÚb‘ÍnepSg²eTot Gal;hVa taLasesmIu EsVgyl;faetIrUbGñk CaGñkpÞúk b¤ Gt;eT nrNaxøHKYreFVIetsþQam ? PaBrYmExSelahitCamYyKña CMerIssMrab;KUrRskrRbQmmuxnwgeRKaHfñak; eFVIetsþTarkkñúgépÞrkCMgWtaLasesmIu karbBaÄb;KP’

155

sTÞanuRkm

169

Bt’mansMxan;² cMNIGahar nig CMgWtaLasesmIu eRbI T2* sMrab;vas;kMritbnÞúkCatiEdkkñúgebHdUg taragvaytMél nig karBüa)al eKhTMB½rsMxan;² Gas½ydæanTMnak;TMngsMxan;² nig Bt’man

156 156 156 158 160 163 163 164 164 167

178 187 189 199 201

buBVkfa shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ RtUv)anbegáIteLIgkñúgqñaM 1986 kñúgbMNgelIkkMBs;eKalkarN_ karBar nig karEfTaMBüa)aleGay)anRtwmRtUv cMeBaHCMgWtaLasesmIu CMuvijBiPBelak. karbegáIt nig karBRgIksmaKmCatiCMgWtaLasesmIu ehIynigkarGPivDÆn_ elIkmμviFIGb;rMdl;RKUeBTüCMnaj EdlbMeBjkargarkñúgvis½yCMgWtaLasesmuI edaybNþúHbNþalbnþ KWsßitenAkñúgbNþaeKalbMNgd¾sMxan; Edltamry³kar enH s>G>t b:gR)afñasMerceKaledATaMgenaH. kñúgbribTenH s>G>t CYyBRgwg dl;RkumGñkCMgW¼matabitafñak;Cati EdlBYkeKCaGgÁd¾sMxan; dak;sm<aFeTAelI GaCJaFrsuxaPi)al eGayelIkkMBs;eKalneya)ayCati edIm,IkarBareGayman RbsiTi§PaB nig karEfTaMBüa)aleGay)anRtwmRtUv cMeBaHCMgWtaLasesmuI. smaKmCMgWtaLasesmuI eday\tmnÞilpgEdr manParkic©CaKnøwHkñúgkar eXasna bBa©úHbBa©ÚlTak;TajGarmμN_CasaFarN³ CaEpñkd¾sMxan;mYyén kmμviFINamYykñúgkarRtUtRtaCMgWtaLasesmIu. s>G>t )anKUsbBa¢ak;pgEdrBIsarsMxan;énkarbegáItkmμviFIelIkkMBs;Gb;rM edayerobcMsikçasila snñisiT fñak;Cati tMbn; nig CalkçN³GnþrCati ehIy)an e)aHBum<pSaynUvÉksarCaRcIn Edl)anbkERbCaeRcInPasa cab;BIBt’man mUldæansMrab;GñkCMgW matabita nig saFarNCnTUeTA edayRKbdNþb;GMBICMgW karBüa)al nig karkarBar rhUtdl;GtßbTviTüasaRsþCMnajsMrab;RKUeBTü EdlCab;Bak;B½n§kñúgkarEfTaMBüa)al CMgWtaLasesmIu. esovePAenH nwgpþl;viPaKTany:agsMxan;dl;karxMRbwgERbgTaMgLaykñúgkar pþl;CUnGñkCMgW nig matabita nUvRbPBfμI²d¾man tMél Bt’manBit Edlbgðaj eLIgkñúglkçN³samBaØ² rMelcedayrUbPaB edayRKbdNþb;RKb;Tid§PaB xii

CMgWtaLasesmuI/ epþImBImUlehtu nig FmμCatiénCMgW rhUtdl;karBüa)al karkarBar TMhMrMCYlcitþ nig bBaðakñúgsgÁm. s>G>t sUmsMEdgkardwgKuNCaxøaMgcMeBaHGñksMrbsMrYlviTüasaRsþrbs; shB½n§ evC¢> Gg;RDULa eGElhVehrIuyU cMeBaHkarsMerckargaredayxitxM y:agGs;BIsmtßPaBkñúgkarsresresovePAenH. CakarBit xJúMmanCMenOeday mansgÇwmfakic©kary:aghμt;ct;kñúgkareFVIeGay)anfμI²Tan;ehtukarN_enHnwg CYypþl;eGayGñkCMgW nig matabitanUvkþIsgÇwm nig karKaMRTbnþEdlBYkeK RtUvkaredIm,IRbQmmux RbyuT§CamYyCMgWtaLasesmIu.

)a:NUs Gg;KøWhSÚs (Panos Englezos) RbFanshB½n§GnþrCatiCMgWtaLasesmIu (TIF President)

xiii

lixitBIGñkniBn§ RbiymitþCaTIrab;Gan/ eBlEdlRbFanRkumRbwkSashB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ )an sMNUmBrxJúMeGayBicarNaniBn§esovePAmYyGMBICMgWtaLasesmuI edayepþat eTAelItMrUvkarrbs;GñkCMgW nig matabita xJúM)ansÞak;esÞIr hak;dUcCakargarmYy KYreGayrujra. b:unμanqñaMknøgeTAenH mankare)aHBum<pSayCaRcInRbkbeday eCaKC½y nigmanRbCaRbiyPaB RtUv)ansresredayGñkviTüasaRsþBUEk l,Il,ajEdlBUEkkñúgvis½y CMgWtaLasesmIu. cab;BIeBlenaHmkPaB eCOnelOnkñúgRKb;Tid§PaBEdlTak;TineTAnwgkarRtYtRtaCMgWtaLasesmuI ekItmanCaeRcIn nig RbkbedayGtßn½y. xJúM)ansMerccitþTTYlykkargard¾lM)ak ehIycab;sresresovePAenHeday bBa©ÚlnUvPaBeCOnelOnfμI²TaMgenaH b:uEnþRbkbedayehtupl. Cabzm xJúMCab;citþCaxøaMgCamYy s>G>t ehIyebþCJaCYyelIkkMBs;vtßúbMNgrbs; s>G>t. elIsBIenHeTot xJúMmanesckþIRsLaj;CaBn;eBkcMeBaHBiPBRKYsar CMgWtaLasesmIu edaymanGarmμN_faxJMúCaEpñkmYykñúgcMeNamBYkeK. xJúM)anBüayamy:aglM)akedIm,IbegáItesovePAenH CadMbUgsMrab;GñkCMgW nig matabita Edlpþl;CUnnUvBt’manBit fμI²Tan;ehtukarN_GMBICMgWtaLasesmuI karBüa)al nig karkarBar. ral;karxMRbwgERbgRtUv)aneFVIeLIgpgEdredIm,I bgðajBt’manTaMgenHkñúgPasasamBaØ nig c,as;las;. karenHminEmnsut EtgayRsYleFVIeLIy CaBiesskñúgCMBUkxøHEdlxJúMxVayxVl;fakarRbIR)as;Pasa samBaØeBkGacmanplGaRkk;dl;Gtßn½yBit. eTaHy:agNak¾eday sgÇwmfa sTÞanuRkmEdl)anpþl;CUnenAPaKxagcugénesovePAenH RBmTaMgrUbPaB nig RkahVikCaeRcIn nwgCYyGñkGaneGay)anyl;TaMgRsugnUvGtþbTNa xiv

EdlminGacsresrCaPasasamBaØ)an. GñkGannwgkt;sMKal;fa karBnül;bEnßmRtUv)anpþl;CUncMeBaHCMBUkedayELk. karsMerccitþpþl;Bt’manlMGit RtUv)aneFVIkñúgPaKxøH² CMBUkRKbdNþb;PaKxøH énrebobBüa)al EdlxJúMyl;favamansMxan;cMeBaHGñkCMgWsMrab;CIvitrs;enA nig KuNPaBCIvitrbs;eK. kñúgEpñkxøHeTot dUcCakñúgPaKTak;TgnwgebHdUg manBakübec©keTseBTüCaeRcInEdlxJúMminGacsMrYlCaPasasamBaØ )aneRcIneLIy. RKb;Bt’mankñúgesovePAenH suT§Et)andkRsg;ecjBIr)aykarN_Edl e)aHBum<pSayCaeRcIn ehIyshB½n§GnþrCatiCMgWtaLasesmIuGacpþl;CUn GñkGan)an tamkaresñIsMucMeBaHGtßbTNamYyEdlRtUv)anykmksikSakñúg esovePAenH. esovePAenH rYmCamYyesovePAelgEhÁm {elg nig eronGMBItaLasesmuI (Play and learn about thalassemia)} nig esovePArUbkMEbøgmYyeTot {GMBICMgWtaLasesmuI (About thalassemia)} EdlkMBugEterobcM b:uEnþnwg ecjpSayqab;²enH nwgCYyGñkCMgWRKb;v½y nig matabitarbs;BYkeKsÁal;c,as; CMgWtaLasesmIu ehIynigkarBüa)alva. edaysgÇwmEdrfa kare)aHBum<pSayTaMgenHnwgbMeBjtYnaTIy:agman GtßRbeyaCn_dl;GñkCMgW nig matabitarbs;BYkeKeGayTMlab;CamYyvaküs½BÞ evC¢saRsþCaeRcInEdlBYkeKnwgCYbeBlTMnak;TMngCamYybuKÁlikeBTü b¤ GanesovePAviTüasaRsþ nig TsSnavdþInanaGMBICMgWtaLasesmuI. GñkCMgWrauMér:kñúgbNþaRbeTsEdlmaneKalneya)ayKaMRTCasaFarN³ xv

ehIymankarRtYtRtataLasesmuI nig CMgWBnæú (Genetic disorders) déTeTot CaTUeTATTYl)ankarGb;rMl¥GMBICMgWrbs;BYkeK ehIyBYkeKEsVgrkBt’mansþIBI karvivDÆn_fμI²CaRbcaM. k¾b:uEnþ GñkCMgWkñúgbNþaRbeTskMBugGPivDÆn_vij CaTUeTA RtUvkarCMnYykñúgkarTTYl)anBt’manfμI²TaMgenaH Bt’manEdlcaM)ac;CYyBYkeK RbyuT§CamYyCMgWeKpÞal;pg RBmTaMgBRgIkkarRbyuT§CMrujeGayrd§aPi)aldak; eGaymaneKalneya)aynanasMrab;karBüa)aleGay)anRtwmRtUv. CaTIbBa©b; esovePAenH)anrYmbBa©ÚlCMBUktUcmYy faetIRtUvkarBarkMeNItTark BICMgWtaLasesmuIy:agdUcemþc vaCakmμvtßúcMbgrbs;TsSnvis½ysuxPaB saFarN³ k¾dUcCal½kçx½NÐFanaKuNPaBBüa)alpþl;CUnGñkCMgW Edlman Rsab;. kMeNItbEnßmnUvkumarmanCMgW nwgkat;bnßyRbPBFnFanEdlman sMrab;pþl;karEfTaMBüa)aleGay)anRtwmRtUvdl;GñkCMgWEdlmanRsab;.

evC¢> Gg;RDULa eGElhVehrIuyU

xvi

CMBUkTI 1

red = oxygenated blood blue = deoxygenated blood

Qam nig taLasesmIu taLasesmIu (Thalassemia) KWCaCMgWmYyRkumEdleFVI[xøÜnmnusSeyIg minGacplitQammanKuNPaBx<s;[)anbrimaNRKb;RKan;. dUceyIgnwg )aneXIjenAxageRkamxøÜnmnusSrs;)an KWGaRs½yedaymanQam dUcenH sMxan;RtUvyl;nUvbBaðaEdlmanplb:HBal;elIQam nig meFüa)ayEdl xøÜnmnusSeyIgeqøIytb. Qam - {sÞwgCIvit} QamCavtßúravRTRTg;CIvit EdldwknaMsarFatuciBa©wmeTAkan; srIragÁ nig Calika kñúgsarBagÁkay ehIynaMykmkvijnUvkaksMNl;sMrab;beBa©j ecal. mnusSeBjv½yEdlmansuxPaBl¥ manQamRbmaN 5 - 6 lIRt KWRbEhl 7 - 8° énTMgn;xøÜnTaMgmUl. QameFVIdMeNIrBaseBjkñúgxøÜn edaysarebHdUgEdlCasñb;rujqøgkat; bNþajbMBg;TaMgLayEdleKehAfa srésQam (Blood Vessels). srésQamman 3 EbbxusKña KWsrésQamRkhm srésQamexμA nig srésQamtUcl¥it (Capillaries) nImYy²suT§EtmanTMhM nig muxgar xus²Kña ¬emIlrUb 1a, 1b¦ . srésQamTaMg 3 rYmKñaehAfa RbB½n§srésQam (Circulatory system) ¬emIlrUb 1a¦ . tYnaTIrbs;Qam QambMeBjmuxgarsMxan;²CaeRcIn ³ (i) dwkCBa¢n Ú GuksIuEhSn (Oxygen) ³ sarBagÁkayRtUvBwgEp¥kelIQam edIm,IdwkCBa¢ÚnsarFatumkciBa©wmsMxan;²EdlxøÜnR)aNeyIgRtUvkar

ehIyykeTAvijnUvkaksMNl;Bul EdlRtUvbeBa©jecalEdlxøÜnR)aN eyIgminRtUvkar. ]TahrN_ edIm,ImanCIvitrs; nig bMeBjmuxgar)an ekasika nig GtisuxumR)aNTaMgGs;RtUvkar GuksIuEhSn - Ca]sμ½nman kñúgxül;EdleyIgdkdegðIm. Qamcab;ykGuksIuEhSnBI sYt ehIynaM eTA[EpñkepSg²énsarBagÁkay. (ii) cab;yk ]sμ½nkabUnik (Carbon dioxide) Ca]sμ½nmYyepSgBIGuksIuEhSnEdlCakaksMNl;ekItecjBIekasika. QamnaMkabUnDIGuksIutRtLb;mkbeBa©jecaleTAxageRkAvijenAsYt. dUcKñaenHEdr Qamk¾RbEm:RbmUlkaksMNl;epSg²eTotdUcCa G‘uyer: (Urea) nig GasIutG‘uyrik (Uric Acid) eTAbeBa©jecalenA tMrgenam nig eføIm. cugbBa©b; kaksMNl;TaMgenHbeBa©jBIQameTAkñúgTwkenam nig lamk ¬emIlrUb 1c¦. 1c

Exchanges between the blood and tissue fluid across the capillary

dwkCBa¢ÚnsarFatuKImIBiess ehAfa G½rm:Un (Hormone) EdlRKb;RKg muxgarénRbB½n§sarBagÁkaysMxan;² dUcCa RkeBjbeBa©jkñúg (Endocrine gland) RbB½n§srIragÁbnþBUC nig pøÚvePT. (iv) dwknaMsarFatuciBa©wmeTAEpñkepSg² énsarBagÁkaydUcCa RbUetGIun xøaj; sár Edl)anmkBIkarbMEbkcMNIGaharenAkñúgRbB½n§rMlayGahar (iii)

¬emIlrUb 1d, 1e¦ . (v) CYysarBagÁkayRbyuT§RbqaMgnwgCMgW nig karqøgeraKepSg² tamry³ ekasikaEdlbegáItEpñkRbB½nækarBar ehAfa RbB½næsuaMnwgeraK (Immune system).

FatupSMrbs;Qam QamRtUv)anplitenAkñúg xYrq¥wg (Bone marrow) ¬emIlrUb 1f¦ CaCalikaeXIjmanenAEpñkkNþal énq¥wg (Central cavity). cMeBaHkumar ekasika QammanplitenAtamCalikaCaeRcIn EtmnusS FMvij manplitEtenAkñúgxYrénq¥wgk,al q¥wgkgxñg q¥wgCMnI nig q¥wgRtKak.

RbB½n§rMlayGahar

ekasika

xYrq¥wg

QamTaMgmUl QamTaMgmUlpSMeLIgeday 2 Epñk ³ (i) EpñkminEmnCaRKab;Qam KWCaEpñk KμanpÞúkRKab;Qam nig (ii) EpñkCaRKab;Qam KWEpñkpÞúksuT§EtRKab;Qam. sarBagÁkayeyIg pSMeLIgBIPaKl¥itCaeRcInEsnekadi ehAfa ekasika. ekasikaeRbob)annwgdMu\dæsg;GaKardUecñaHEdr. ekasikamanTMhMtUcemIlnwg EPñkTeTmineXIjeT. CalikaPaKeRcInmanekasikas¥itpþúMCab;Kña b:uEnþkñúgQam vij ekasikavaGENþt hUrcuHeLIg kñúgsarBagÁkay. ekasikanImYy²man 3 EpñksMxan;² KW EpñkkNþal eKehAfa sñÚl (Nucleus) EpñkCMvu ji sñlÚ eKehAfa sIutUpøas (Cytoplasm) ehIynigsMbkB½T§CMuvijeKehAfa Pñas (Membrane) ¬emIlrUb 1g¦. rcnasm<½n§tUcCagenHCaeRcIn mankñúgEpñkTaMgbIxagelI ehIynImYy²manmuxgarBiessedayELkrbs;va. ya:gNak¾eday mYyPaK FMkñúgekasikanImYy²suT§swgEtCaTwkrYmCamYyRbUetGIun xøaj; sár nuyekøGiuc GasIut (Nucleic Acid) m:UelKulrlaykñúg Twk (Dissolved molecules) GIuy:ugGsrIragÁ (Inorganic ions). RbUetGIunCakMlaMgclkr énekasikarbs;eyIg. kñúgsarBagÁkay rbs;eyIg manRbUetGIunxus²Kña 100>000 RbePT. muxgarxøHrbs;RbUetGIunkñúgekasika rYmman ³ * sagsg;rcnasm<n ½ m§ yY PaKFMrbs;ekasika * edIrtYCakatalIkr (Enzymes) sMrab; RbtikmμKimI EdleFVI[CivitRbRBwtþeTA)an * RKb;RKgTMnak;TMng rvagbNþaépÞsMbk ekasika * RKb;RKgTidæPaBBn§útMNrBUC (Expression of genes) *

pþti ykFatuBn§tú N M BUC (Genetic material)

EpñkminCaRKab;QaménQam KWCaFaturavBN’elOgbnþic eKehAfa pøasμa (Plasma) Edlmandl;eTA 55° énQamTaMgGs;. pøasμapSMeLIgBITwk nig GMbilRBmTaMgRbUetGIunsMxan;²EdlvaEckcayBaseBj sarBagÁkay dUcCa ³ Gal;b‘uymIn (Albumin) CaRbUetGIuncMbgkñúgQam. køÚb‘uylIn (Globulins) rYmman hÁama:køÚb‘uylIn (Gamma globulin) pSMeLIgedayGgÁbdibkçrab;mWun EdlCYysarBagÁ kayRbyuT§RbqaMgnwgCMgW nig karqøgeraKepSg². hVIRb‘ÍNUEhSn (Fibrinogen) CYykñúgkMNkQamkMu[ QamhUrecjeBlmanrbYsNamYy. (ii) EpñkCaRKab;Qamvij pSMeLIgedayekasika 3 RbePT ³ eKalikaQam Rkhm b¤ eGrIRtUsIut (Erythrocytes) eKalika Qams b¤ LWkUsIut (Leucocytes)/ pøaEkt b¤ RtumbUsIut (Thrombocytes) ¬emIlrUb 1h¦ eKalikaQamRkhm b¤ eGrIRtUsIut sarBagÁkaymanpÞúkeKalikaQamRkhm (Red Blood Cells) RbmaN 4>500>000 - 5>000>000¼mm3 Qam - esÞIrEt 45° énbrimaNQam srub. eKalikaQamRkhmmanGayumFümEvgCageKkñúgcMeNamRKab;Qam déTeTot KW 100 - 120 éf¶. muxgarcMbgrbs;eKalikaQamRkhm KWdwknaMGuksIuEhSneTABaseBj sarBagÁkay edayP¢ab;GuksIuEhSneTAnwgsmasFatumYyehAfa eGm:UkøÚb‘Ín (Hemoglobin) EdlbnÞab;mkvanaMGuksIuEhSneTAeGayekasikanImYy² énsarBagÁkay. eKalikaQamRkhmmanpÞúkm:UelKuleGm:UkøÚb‘ÍnCaeRcIn RbmaNCa 300 lan CaehtueFVI[QammanBN’Rkhm. tamBiteTA eKalikaQamRkhmmanpÞúkeBjedayeGm:UkøÚb‘Ín EdleFVI[vaminGacpÞúk GVIepSgeTot)andUcekasikaepSg²eTot dUcCa sñÚl (Nucleus) CaedIm (i)

smasFatupSMrbs;Qam

neutrophil

eosinophil

Basophil

¬emIlrUb 1h¦. Pñas b¤ sMbkxageRkArbs;eKalikaQamRkhmmanPaBTn;dUcBBuHsab‘U. karenHeFVIeGayvagayRsYlbt;Ebn RKb;Tis edayKμanEbk CaBiesseBlva qøgkat;srésQamtUc²bMput (Capillaries) edIm,IdwknaMGuksIuEhSneTAkEnøg NaEdlRtUvkar. eKalikaQamRkhmmanpÞúkkatalIkrcMnYneRcInCa kabUnic GanIRdas (Carbonic anhydrase) EdlrYmcMENksMxan;dl;kardwkCBa¢Ún]sμ½nkabUnik BICalikaeTAsYt. eKalikaQam>s b¤ LWkUsIut ¬emIlrUb 1h¦ vamanEt 1° énQam. BYkvamannaTICaGayuCIvitedayedIrtYCaExSbnÞat; karBarCYrmuxRbqaMgnwgkarratt,atBIPñak;garbgáCMgW dUcCa )ak;etrI vIrus pSit nig )a:ra:sIut. eKalikaQam>s (White Blood Cells) CaRkumekasikaepSg² Kña RkumnImYy²rYmcMENktamviFIxus²KñaedIm,IRbqaMg nig karBarkarqøgeraK nig karbMpøicbMpøajCalika. tamFmμta eKEbgEckBYkvaeTAtamlkçN³rUbragÁ rbs;va ³ RkanuyLÚsIut (Granulocytes) b¤ ekasikaEdlmansñlÚ eRcIn eKehAdUcenH edaysarsñÚlvamansPaBCaRKab; nig manEkøbeRcIn. eKEbgEckRkumenHeTAtamBN’vaeBlbMBak;BN’ enAkñúgmnÞIrBiesaFn_ ³ - NWRtUhVIl (Neutrophils) ¬emIlrUb 1i¦ ¬man 72° éneKalika Qam>sTaMgGs;¦ vamanBN’exov eBlbMBak;BN’. - eGGUsIuNUhVIl (Eosinophils) ¬emIlrUb 1j¦ ¬man 1/5° éneKalika Qam>sTaMgGs;¦ vamanBN’RkhmeBlbMBak;BN’. - )asUhVIl (Basophil) ¬emIlrUb 1k¦ ¬man 0/5° éneKalikaQam>s TaMgGs;¦ vamanBN’sVay eBlbMBak;BN’.

monocyte

eKalikaQam>s epSg²eTotman ³ - m:UNUsIut (Monocyte) ¬emIlrUb 1l¦ ¬man 4° éneKalikaQam>s TaMgGs;¦. - LaMhVÚsIut (Lymphocyte) ¬emIlrUb 1h¦ ¬man 24° éneKalikaQam>s TaMgGs;¦. eKalikaQam>s manTMhMFMCageKalikaQamRkhm EtvamancMnYnticCag RbEhl 7>000¼mm3 énQam ehIyCIvitvak¾xøICagEdr manry³eBlEt 18 - 36 ema:gb:ueNÑaH . pøaEkt (Platelets) mantYnaTIsMxan;EtmYyenAkñúgQam vaepþIm[mandMeNIr kMNkQam ¬ERbQameTACaqñúkP¢it¦ karBarsarBagÁkaykMu[)at;bg;Qam tamsrésQamEdldac;. pøaEkt KWCaeKalikaQamEdlmanTMhMtUcCageK ¬emIlrUb 1h¦. vamancMnYnRbmaN 200>000¼mm3 énQamehIyman GayuRbEhl10 éf¶. xusBIeKalikaQamRkhm BYkeKalikaQam>s nigpøaEkt suT§Etman sñÚl nig sIutUpøas . eKalikaQamTaMg 3 RbePTenH ¬eKalikaQamRkhm eKalika Qam>s nig pøaEkt¦ ekItecjBIekasikaedIm (Precursor) dUcKña ehAfa eGma:tUb:UGIueyTic esþmEsl (Hematopoietic stem cell). ekasika edImenHEbkCakUnecAelOnNas;. RKan;Etkñúgry³eBl 4 s)aþh_/ ekasika edIm cMnYn 10 GacbMEbk)anCaRKab;QamRkhmcMnYn 30 Esnekadi RKab; Qam>s cMnYn 30 ekadi nig pøaEktcMnYn 1/2 Esnekadi - EdlRKb;RKan;lμm eTACMnYsral;RKab;QamkñúgsarBagÁkay. RbePTQam ¼RkumQam RkumQamcMbgman 4 RbePT ³ A, B, AB nig O kt;sMKal;edayRbePT RbUetGIun b¤ ehAmüa:geTotfa cMNucsMKal; (Marker) b¤ Gg;TIEhSn (Antigen) sßitenAelIPñaséneKalikaQamRkhm. QammnusSeyIgmñak;² cat;cUl

what platelets do

eTAkñúgRbePTNamYyénQamTaMg 4 RkumcMbgenH A, B, AB b¤ O KWfa mnusSmñak;²maneKalikaQamRkhmEtmYyRbePTkñúgbNþaRkumTaMgenH ³ RkumQam A - eKalikaQamRkhmmancMNucsMKal; A (Marker A) elIPñas rbs;BYkva. RkumQam B - eKalikaQamRkhmmancMNcu sMKal; B (Marker B) elIPañ s rbs;BYkva. RkumQam AB - eKalikaQamRkhmmancMNucsMKal;TaMgBIr A nig B (A and B Markers) elIPñasrbs;BYkva. RkumQam O - eKalikaQamRkhmKμancMNucsMKal;TaMgBIr A nig B elI Pñasrbs;BYkvaeT. eKalikaQamRkhm GacpÞúkpgEdrnUvGg;TIEhSnmYyepSgeTot EdlKμan Tak;TgnwgRkumQam KW Gg;TIEhSnerhSús [Rhesus (Rh) Antigen]. QamEdlmancMNucsMKal;erhSús (Rh Marker) eKehAfa erhSúsviC¢man ÉQamEdlKμancMNucsMKal; erhSús eKehAfa erhSúsGviC¢man. eK)anRsavRCavrkeXIjeKalikaQamRkhmCag 20 RbePTepSgeTot b:uEnþmanEtRbePTxagelIeT EdlsMxan;bMput ehIyeKyl;c,as;. manehtuplxøH²EdleKRtUvkardwgBIRbePTQamrbs;xøÜn. sarsMxan; KWsßit enARtg;fabuKÁlmñak;GacnwgRtUvTTYlQamBIbuKÁlmñak;eTot dUcCakarbBa©Úl QamCaedIm ¬emIltarag 1w¦. kñúgkarbBa©ÚlQam QamGñkpþl; ¬Gñkbric©aKQam¦ nigQamGñkTTYl ¬GñkRtUvbBa©ÚlQam¦ RtUvEtsIuKña ¬cUlKña)an¦ edIm,IkMu[sarBagÁkayGñkTTYlQamRbqaMgnwgQamEdl)anbBa©ÚleTA. dMeNIrénkarpÁÚrpÁg[sIuKñaénRkumQam nig Gg;TIEhSn erhSús rbs;Gñkbric©aK CamYyGñkTTYl eKEtgEteFVIenAmnÞIrBiesaFn_énmCÄmNÐlpþl;Qam ehIy kareFVIrebobenHeK[eQμaHfa RkUsμac; (Cross matching) b¤ karsakl,grk PaBRtUvKña (Compatibility testing). RbsinebIRkumQam nig ktþaerhSúsmin dUcKña sarBagÁkayGñkTTYlQamnwgyl;fa QamEdlbBa©ÚleTACaPñak;gar

ebotebon ehIysarBagÁkaynwgkMcat;vaecj. PaBRbwg 1w ERbgrbs;sarBagÁkay vaykMcat;QamBIeRkAEdlminsIu KñaenaH nwgeFV[I ekItCaCMgFW n¶ F; r¶ ehIymandl;søab; ebI eFVIeraKvinicä½y nigBüa)alminTan;eBlevla. CMgWQam manCMgWCaeRcInbNþalmkBImanPaBminFmμtaenAkñúgQam. PaBminFmμta TaMgenH cat;fñak;eTAtamEpñkénQamEdlb:HBal; dUcCa CMgWeKalikaQam Rkhm CMgWeKalikaQam>s b¤ CMgWkMNkQam. CMgWeKalikaQamRkhm kñúgenaHmanCMgWtaLasesmIuCaedIm. CMgWmYykñúg bNþaCMgWeKalikaQamRkhmEdlCYbjwkjab;CageK KWeraKxVHeKalikaQam Rkhm (Anemia) CaeraKEdlsarBagÁkayeyIgmaneKalikaQamRkhm b¤ eGm:UkøÚb‘ÍnmankMritTabxusBIFmμta. eraKsBaØaTUeTAéneraKxVHeKalikaQam Rkhm KWPaBesøksøaMgGs;kMlaMgBlM edaysarQamBMu)andwknaMGuksIuEhSn eTAsarBagÁkay[)anRKb;RKan;. RbePTeraKxVHeKalikaQamRkhmEdlCYb jwkjab;CageKenaH KWbNþalmkBIxVHCatiEdk bNþal[xYrq¥wgminGacplit cMnYneKalikaQamRkhm)anRKb;RKan;. GñkCMgWEbbenH CaTUeTAnwgCaes,Iy edayRKan;EteRbIfñaMCatiEdkbMeBjbEnßm. b:uEnþ man eraKxVHeKalikaQamRkhmepSg²eTot EdlvaF¶n;F¶rCag. ]TahrN_ eraKxVHeKalikaQamRkhmbNþalmkBIkarEbkRKab;Qam (Hemolytic anemia) edaysarBagÁkayeyIgbMpøajeKalikaQamRkhm kñúgkMritelOn. kñúgCMgWtaLasesmIukMritF¶n; (Thalassemia major) eKalikaQamRkhmekItPøamRtUv)anbMpøajPøam dUcenHxYrq¥wgminGacpliteKalika QamRkhm[RKb;cMnYn mkCMnYs)aneLIy. CMgWTaMgenHmanlkçN³tMNBUCBI «Bukmþaypþl;mk[kUn ehIyvaF¶n;F¶rNas;. Gs;CaeRcInqñaMmkehIyEdleK KitfaminGacBüa)al)an ehIyGñkCMgWTaMgenaHnwgsøab;taMgBIv½yekμgeday 9

eCosminrYc. Et\LÚvenH eyIgdwgfakarBüa)alRtwmRtUv GñkCMgWGacmanCIvit Fmμta)an. CMgWtaLasesmIukMritF¶n; taLasesmIukMritF¶n; ehAmüa:geTotfa eraKxVHeKalikaQamRkhm emDIETra:en (Mediterranean anemia) b¤ eraKxVHeKalikaQamRkhmkULI (Cooley’s anemia). eKFøab;EtKitfa vamanEtenACMuvijsmuRT emDIETra:en GBa©wgehIy)anCaeKehAfa eraKxVHeKalikaQamRkhm emDIETra:en b¤ taLasesmIu [Thalassemia (Thalassa + anemia)] Bakü “Thalassa” )ann½yfa {smuRT} vamankMeNItBIPasaRkik rYmBakümk)an esckþIfa eraKxVHeKalikaQamRkhmenAbNþaRbeTsCMuvijsmuRT - smuRT emDIETr:aen. CMgWenHeKehAfa eraKxVHeKalikaQamRkhmkULI k¾)aneday yktameQμaHevC¢bNÐitCMnajEpñkeraKkumar eFam:as kULI CaCnCati GaemrikkaMgEdlrYmCamYyevC¢bNÐit EBrl_ lI )anerobrab; nig raykarN_ elIkdMbUg nUvlkçN³rUbPaBKøInikkñúgqñaM1927 eRkayBI)aneFVIkarsikSaelIGñk CMgWTaMgLaymanedImkMeNItCaCnCati GIutalI. taLasesmIukMritF¶n; CaRbePTtaLasesmIuEdlF¶n;F¶rCageK ehIyCaRbFan bTcMbgénesovePAenH. taLasesmIumanCaTUeTAenAtMbn;ekþAkñúgBiPBelak. eTaHbIya:gNak¾eday kareFVIcMNakRsukrbs;mnusS CMuvijBiPBelak)annaM taLasesmIueTARKb;TIkEnøgEdlBImunBMuman. CaBiessbNþaRbeTsPaKxag eCIg dUcCa Gg;eKøs shrdæGaemrik kaNada nig GaLWm:g; mankrNICMgWenH ekIneLIgKYr[kt;sMKal; bNþalmkBIGenþaRbevsn_BIGWr:ubxagt,Úg nig GasIu.

CMBUkTI 2

DNA

genome

Bn§útMNBUC nig taLasesmIu taLasesmIu KWCaCMgWEdlTak;TgnwgBn§úehIytBUC )anesckþIfa qøgBI«Buk mþay eTAkUntamry³ Bn§ú (Genes). vaminqøgtamQam xül; Twk b¤ qøg tamkarb:HBal;ragÁkay b¤ pøÚvePTCamYyGñkCMgWeLIy vaminbgáedaykgVHcMNI Gahar b¤ sßanPaBevC¢saRsþxVHGnam½yenaHEdr. EhSn (Genes) = Bn§ú RkumBn§ú KWCaBnøkBUCsMrab;sarBagÁkaymYyedayELkerog²xøÜn edaypþl;nUv Bt’manCIvsaRsþTaMgGs;EdlcaM)ac;sMrab;RtYtBinitükarrIkFMFat; nig karvivtþn_ eBjmYyCIvitrbs;sarBagÁkayenaH vaCaRkumGgÁCIvsaRsþtMNBUC. BinÞúnImYy²CaPaKsMxan;rbs;sarFatuKImImü:agehAfa edGuksIurIbUnuyekøGiuk GasIud (Deoxyribonucleic acid = DNA). DNA ¬emIlrUb 2a¦ Carcnasm<½n§mYydUckaMCeNþIr EdlmanemCeNþIrBIrRsb KñaP¢ab;edaykaMCeNþIrCaeRcIn. kaMnImYy²mansarFatuKImI BIrpþúMCab;KñaCaKUr eKehAfa )as (Bases). )asnImYy² tagedayGkSrxus²Kña (C, G, A, T) . GkSr C sMrab; sIutUsIun (Cytocine)/ G sMrab; háanIn (Guanine)/ A sMrab; GaednIn (Adenine)/ T sMrab; TImIn (Thymine). )asTaMgbYnenHcab;KUrKñakñúg lkçN³kMNt;c,as;las; ³ A KUrCamYy T ehIy C KUrCamYy G ¬emIlrUb 2a¦. BYk)astMerobKñatamlMdab;lMedayCakaMCeNþIrpþl;nUvkUttMNrBUC (Genetic code) rbs;sarBagÁkay. DNA rbs;sarBagÁkaypÁúMcUlKñaCaeRcIn eTA eK[eQμaHfa bNþMúBn§ú b¤ ehSenam (Genome) ¬emIlrUb 2b¦.

RkUm:UhSÚm

ehSenamrbs;mnusSmanpÞúkBn§úeRcInBan;. DNA pþl;karENnaM[mankar sagsg;m:UelKulTaMgGs; EdlTTYlxusRtUvdl;muxgarCIvsaRsþrbs;sarBagÁkay dUcCa sagsg;RbUetGInu . dMeNIrénkarplitm:eU lKulmYyecjBIBn§Tú ak;Tg eTAnwgynþkarüCIvsaRsþkñúgekasika KW {karGan} Bt’manEdlpÞúkkñúgBn§ú. Bt’manenaH)ancMlgcUlkñúgm:UelKulmYy Rsedognwg DNA eKehAfa RNA [b¤ehAfa rIubUnuyekøGiuk GasIud (Ribonucleic Acid)]. dUc DNA Edr/ RNA manpÞúk)asháanIn GaednIn nig TImIn (G, A and T) Et sIutUsIun )asTIbYnkñúg RNA RtUvCMnYseday G‘uyra:sIul [Uracil (U)]. RNA xusBI DNA Rtg;fa RNA manrcnasm<½n§EtmYy KμanBIrdUcCeNþIrenaHeT. muxgarrbs; RNA KWCaGñknaM sar (mRNA) naMBt’manBI DNA eTAynþkarüCIvsaRsþrbs;ekasika EdlTTYl xusRtUvkñúgkarbegáItplitplCIvsaRsþ dUcCa RbUetGIunCaedIm . kñúgsarBagÁkaymnusStRmUv[manBn§úmYycMnYnFM edIm,IGnuvtþn_nUvmuxgarCIv saRsþsμúRKsμajCaeRcIn. Bn§úTaMgenH)anRtUvrkSaCab;KñakñúgekasikaCaExS DNA d¾EvgEdleKehAfa RkUm:UhSÚm (Chromosomes). ekasikanImYy² rbs;mnusS ¬elIkElgEtekasikaemCIvitburs nig RsþI¦ manc,ab;cMlg RkUm:UhSÚmBIr mYymkBImþay mYyeTotmkBI«Buk. RbePTsarBagÁkayxusKña cMnYnRkUm:UhSÚmk¾xusKñaEdr. mnusSmanRkUm:UhSÚm 23 KUr b¤ 46 RkUm:UhSÚm ¬emIlrUb 2c¦. cMnYn 22 KUr ¬44 RkUm:UhSÚm¦ man lkçN³dUcKñaTaMgePTRbus TaMgePTRsI eKehAfa GUtUsUm (Autosomes) ÉmYyKUrenAsl;Ca RkUm:UhSÚmePT (Sex chromosomes) CaGñkkMNt;ePT. kñúgRkUm:UhSÚmmYyKU²rbs;mnusSmñak;²manmYy)anmkBImþay/ mYyeTot)an mkBI«Buk. eRkaykarRsavRCavCaeRcInqñaM eKGacsÁal;Bn§úCaeRcInEdlkalNavaTTYl rgkarb:HBal; vabgá[manCMgWepSg²ehAfa CMgWBn§ú (Genetic disorders).

CMgWBn§ú EbgEckeTACabYnRbePT ³ 1> PaBminFmμtarbs;RkUm:UhSÚm ³ TaMgenHekIteLIgeBlRkUm:UhSÚmTaMgmUl b¤ mYyEpñkFMrbs;RkUm:UhSÚm)an)at;bg; ekIneTV b¤ ERbRbYl . 2>CMgWrbs;Bn§úEtmYy ³ TaMgenHbgáeLIgeBlmankarERbRbYlpøas;bþÚrenARtg; Bn§bú gá[mankarERbRbYl b¤)at;bg;karsMeyaKnUvplitpl ecjBIkUdrbs;Bn§úenaH. 3> CMgWb:HBal;elIBn§úeRcIn ³ vaekItecjBIkarERbRbYl pøas;bþÚrénBn§úCaeRcIn rEmgekItmkCamYyehtuplEpñk briyakas. )anesckþIfa manktþaCaeRcInRtUvekItman RsbKña munnwg[ekItmanCaCMgW. 4> CMgWTak;Tgnwg mItUkugRDI (Mitochondria) ³ CMgWbgáedaykarERbRbYlpøas;bþÚrkñúgrcnasm<½nætUcmYy EdlminEmnCa RkUm:UhSÚm DNA eT vasßitenAkñúgsIutUpøasrbs;ekasika eKehAfa mItUkugRDI vaCasßanIy_sþúkfamBlrbs;ekasika. taLasesmIu KWCaGVIEdleKdwgfaCa CMgWrbs;Bn§úEtmYy vaqøgBI«Bukmþay eTAkUn tamEbbEpnNamYyénEbbEpnTaMgbYnCamUldæanEdlRBHsgÇeQμaH eRhÁK½r emnEdl (Gregor Mendel) )anerobrab;elIkdMbUgkñúgqñaM 1865. EbbEpnTaMgbYn mandUcxageRkam ³ (i) GUtUsUm:al dUmINg; (Autosomal dominant) (ii) GUtUsUm:al er:esssIuv (Autosomal recessive) (iii) Giuc-lIg dUmINg; (X-linked dominant) (iv) Giuc-lIg er:esssIuv (X-linked recessive) BaküTaMgenHeKeRbIenAkñgú Bn§vú Ti üa edIm,Ierobrab;lkçN³lT§plKønI ki [ehVNTU bI (Phenotype)] EdlekItecjBIPaBminFmμtarbs;Bn§ú [ehSNUTIb

mItUkugRDI

. ebI ehVNUTIbekItecjBIehSNUTIbEtxagNamYy «Buk b¤ mþay karenHCa GUtUsUm:al dUmINg;. ebIekItecjBIehSNUTIbTaMg«Buk TaMgmþay enaHeKehAfa GUtUsUm:al er:esssIuv. dUcKñaenHEdr ebIehVNUTIb ekItecjBI ehSNUTIbtamry³PaBminFmμtarbs;RkUm:UhSÚmkMNt;ePTEtmYy «Buk b¤ mþay eKehAfa Giuc-lIg dUmINg; EtebIekItecjBIRkUm:UhSÚmkMNt;ePTTaMgBIr TaMg«Buk TaMgmþay eKehAfa Giuc-lIg er:esssIuv. taLasesmIu CaCMgWrbs;Bn§úEtmYyEdlqøgBI«BukmþayeTAkUn tamEbbEpn tMNBUC GUtUsUm:al er:esssIuv. CMgW GUtUsUm (Autosomal disease) GacekItTaMgePTRsI nig RbusdUcKña edayehtufaPaBminRbRktIekItmanelI GUtUsUm (Autosomes) NamYy EtminekItelIRkUm:UhSÚmEdlkMNt;ePTrbs;kumareT. er:esssIuv (Recessive) mann½yfa kumarRtUvTTYlBn§úbgáCMgWBI«Bukpg nig BImþaypg edIm,I[ekItCasPaBKøInikF¶n;F¶r ehAfa taLasesmIukMritF¶n;. buKÁlNaEdlTTYlBn§úbgáCMgWBImþaypg BI«Bukpg eKehAfa GUm:UhSIúháÚt (Homozygotes). kñúgkrNI Ebtta taLasesmIu ( β– Thalassemia) eKehAfa GñkCMgW Ebtta taLasesmIu GUm:UhSIúháÚt (Homozygous β– Thalassemia) eKGacehAmüa:geTotfa taLasesmIukMritF¶n; (Thalassemia major)/ eraKxVHeKalikaQamRkhmemDIETra:en (Mediterranean anemia) b¤ eraKxVHeKalikaQamRkhmkULI (Cooley’s anemia). buKÁlNaEdlTTYlBn§úFmμtamYyBImñak;én«Bukmþay ehIyBn§úmYyeTotbgáCMgW BImñak;epSgeTotén«Bukmþay eKehAfa eGetr:UhSIúháÚt (Heterozygotes). ehIydUcKñaenHEdr kúñgkrNI Ebtta taLasesmIu eKehAfa Ebtta taLasesmIu eGetr:UhSIúháÚt (Heterozygous β – Thalassemia). manBaküepSgeTotEdleKeRbIcMeBaHGñkpÞúkBn§úbgáCMgW EtGt;maneraKsBaØa KW (Genotype)]

taLasesmIukMritRsal (Thalassemia minor b¤ Thalassemia trait). GñkCMgWEbbenH nwgminekItCaeraKsBaØaGVIeT Etya:gNak¾eday eKnwgbBa¢ÚnBn§ú bgáCMgWenaHeTAkUnrbs;eK. PaBminRbRktIkñúgeKalikaQamRkhmEdlnaMeGayekItCMgWtaLasesmIukMritF¶n; dUc)anerobrab;kñúgCMBUkTI1/ taLasesmIu KWCaCMgWBn§útBUCénQam [cMCagenH eTot KWCaCMgWmYybNþalmkBIPaBminRbRktIkñúgkarsMeyaKm:UelKul eGm:UkøÚb‘Ín kñúgeKalikaQamRkhm. eGm:UkøÚb‘Ín nig Edk eGm:UkøÚb‘Ín ¬emIlrUb 2e¦ KWCaRbePTm:UelKulcMruHBiess EdlCaRbUetGIun mankñúgeKalikaQamRkhm EdlmantYnaTIdwknaMGuksIuEhSneTAkan;kEnøg EdlRtUvkarkñúgsarBagÁkay. eKalikaQamRkhmnImYy² manm:UelKul eGm:UkøÚb‘Ín 300lan. m:UelKuleGm:UkøÚb‘ÍnmYyEckCa 2 Epñk ³ (i) RbUetGIunmYy ehAfa køÚb‘Ín (Globin) begIát)anCaExSRbUetGIuncMnYnbYn tMerobCaKUrnwgKña ¬emIlrUb 2e + f¦. vamanExSRbUetGIunmYycMnYn dUcCa ExSRbUetGIunRbePT Gal;hVa (alpla = a ) nig ExSRbUetGIunEdlminEmn RbePT Gal;hVa (β ,γ , , ,E ) tMerobKñaCaKUr a β , a γ , a , a nig a E . (ii) bNþMú EGm-Edk (Haem-Iron) Carcnasm<½n§ragCargVg;EdlsMeyaK enAkñúg sIutUsul (Cytosol) nig mItUkugRDI (Mitochondria) rbs;ekasika. m:UelKulEdkmYymankñúgbNþMú EGm-EdkCaGñkpþl;lT§PaB[mankardwk CBa¢ÚnGuksIuEhSnkñúgsarBagÁkay. enHedaysarEtEdkgayP¢ab; nig gay pþac;GuksIuEhSn edaybegáItCameFüa)ayd¾manRbsiT§iPaBkñúgkarnaM GuksIuEhSneTACalika nig ekasika. 2

2 2

Haemoglobin

2 2

mnusSFMFmμtamanEdk 4 Rkam enAkñúgxøÜn ehIy 75° ¬RbEhlCa 3 Rkam¦ RtUv)aneRbIedIm,IsMeyaKeGm:UkøÚb‘Ín. karplit nig sMeyaKeGm:UkøÚb‘Ín TTYlkarRKb;RKgBIBn§úmYycMnYn KW BYk Bn§úGal;hVa (a- genes) sßitelI RkUm:UhSÚmelx 16 cMENk Bn§úEbtta (β- genes)/ Bn§úháama: (γ- genes) nig Bn§úEdlta ( - genes) sßitelI RkUm:UhSÚmelx 11. manBn§úbYn kMNt;ExSGal;hVa (a- chains) nigmanBn§úBIrkMNt;ExSEbtta (B- chains) ¬emIlrUb 2g¦. edayminKitBIcMnYnBn§úEdlTTYlxusRtUvkñúgkarRtYtBinitükar sMeyaKExSGal;hVa nig ExSEbtta KW ExSTaMgBIrRtUv)anplitkúñgbrimaN esμIKñaya:gBitR)akd. 2g

The α- and β- globin gene clusters on chromosomes 16 and 11, respectively

manRbePTeGm:UkøÚb‘ÍnepSg² )anmkBIkarpÁÚrpÁgExSRbUetGIunxus²Kña EdlRtUv )anplitenAdMNak;kalnImYy²énCIvit dUcbgðajkñúgtaragxageRkam ¬emIlrUb 2h¦. 2h

The sites of erythropoiesis during development and the different globins produced at each stage

Bn§úEdlRKb;RKgkarsMeyaKkøÚb‘Ín PaBminRbRktIBitR)akdEdlnaMeGayekIt Ebtta taLasesmIukMritF¶n; KWenARtg;Bn§úEdlRKb;RKgkarplit ExSRbUetGIun Ebtta kñúg køÚb‘Ín rbs; eGm:UkøÚb‘Ín. CalT§plBIPaBminRbRktIrbs;Bn§úenH vaKμanplit ExSEbtta b¤ manbnþic bnþÜc EdleFVI[eKalikaQamRkhmmanExSRbUetGIunEtmYyRbePT KW ExS Gal;hVa. GtulüPaBenH raraMgdl;karpliteGm:UkøÚb‘ÍnFmμta EdlTamTar [man ExSEbtta nig ExSGal;hVa edIm,IdMeNIr[)anRtwmRtUv. CalT§pl karpliteKalikaQamRkhmEdleKehAfa eGrIuRtUb:UGIueyhSIús (Erythropoiesis) ¬ecjBIBaküRkik Erythra )anesckþIfa {eKalikaQamRkhm}/ poiesis )anesckþIfa {plit}¦ RtUvTTYlrgkar b:HBal;ya:gF¶n;F¶r. dUc)anCMrabxagelI PaBminRbRktIrbs;Bn§úTTYlxusRtUvkñúgkarplit ExSEbtta rbs;eGm:UkøÚb‘Ín KWbgáedaykarpøas;bþÚrEtmYyenAkñúgKUr)asmYy ¬karpøas;bþÚrcMNuc¦ b¤ edaybMENkTaMgLayrbs;Bn§ú)anbg; ¬rlub)at;¦. 17

eK)ansÁal;karpøas;bþÚr Cag 200 epSg²KñaEdlGacb:HBal;eTAdl;dMeNIr karFmμtarbs;Bn§úsMrab; EbttakøÚb‘Ín (β- globin). GaRs½yedayRbePT énkarpøas;bþÚr karb:HBal;karsMeyaK køÚb‘Ín mankMrittictYc b¤ xøaMgkøa ehIynaMeGayekIttaLasesmIukMritRsal b¤ kMritF¶n;. karpøas;bþÚrxøHmanplb:HBal;mFüm (β++) rhUtdl;b:HBal;bgÁÜr (β+) eTAelIkarplit ExSEbtta/ rIÉkarpøas;bþÚrxøHeToteFVI[)at;karplit ExSEbtta Etmþg (βo). dUcenHmnusSEdlTTYlBn§ú β+ BI«Bukmþay TMngCavivtþn_eTArktaLasesmIu kMritmFüm. pÞúyeTAvij GñkTaMgLayEdlTTYlBn§ú βo BI«Bukmþay nwgxVH EbttakøÚb‘Ín esÞIrEtTaMgRsug ehIynwgvivtþn_eTArkTMrg;F¶n;F¶réntaLasesmIu esÞIrEt 100°. eTaHya:gNak¾eday eBlTTYlBn§úcMruHcUlKña/ vaminCakar gayRsYlkñúgkarTsSn_TayehVNUTIb EdlEsþgecjmkeLIy dUcCa β+β++ b¤ β+βo b¤ β++β+o. cMeBaH Ebtta taLasesmIu ( β - Thalassemia) ]TahrN_ lT§PaBbgá GacCa ³ βo/βo :

taLasesmIukMritF¶n; (Thalassemia major) β+/ β+ : taLasesmIukMritF¶n; (Thalassemia major) β++/β++ : taLasesmIukMritmFüm (Thalassemia intermedia - TMrg;mFüménCMgW) β+/ β++ : taLasesmIukMritF¶n; ¼ kMritmFüm (Thalassemia major / intermedia)

ehSNUTIb ¼ ehVNUTIb (Genotype / Phenotype) dUc)anerobrab;xagelI vamansarsMxan;Nas;kñúgkarEsVgrkkarpøas;bþÚrBn§ú BiessedayELk (Genotype) EdlmanTMnak;TMngCamYyPaBF¶n;F¶rEpñkKøInik énCMgW (Phenotype). karbUkpSMcUlKñaepSg²énBn§úxusKñaTaMgbI (β++, β+ nig βo) EdlmankñúgtaLasesmIu eFVI[kMritF¶n;F¶rénCMgWxus²Kña. TnÞwmnwg enHEdrenARbeTsepSg² manlkçN³énkarpSMBn§úcUlKña ehIynigPaBjwkjab; 18

énkarpøas;bþÚrbn§úedayELkrbs;eK. ]TahrN_ énkarpøas;bþÚr (Mutations) kMritF¶n; ¬0¦/ minsUvF¶n; ¬+¦/ mFüm ¬++¦ bgðajdUcxageRkam ³ β++ Mutations

β+ Mutations

βo Mutations

-101, -87, -88, -28

lVS1-110

lVS1-1

lVS1-106

lVS11-745

lVS11-1

lVS1-5

IVS1-[-25] C39

ktþaBn§úmYycMnYnepSgeTot k¾GacCYybnßyPaBF¶n;F¶rénCMgW)anEdr dUcCa ³ (i) karsMeyaKExSGal;hVa (a - chains) fycuH. Ca]TahrN_ GñkCMgW taLasesmIukMritF¶n;xøH Kat;TTYlkarpøas;bþÚrEdlbnßykarsMeyaK ExSGal;hVaEfmBIelIkarfycuHénkarsMeyaKExSEbtta. kñúgkrNIenH GtulüPaBrvagExSGal;hVa nig ExSEbttaminsUvxøaMg dUcenHCMgWk¾manTMrg; minF¶n;F¶rEdr. (ii) kMeNInkarsMeyaKExSháama: (γ - chains). QammnusSFM Fmμta man eGm:UkøÚb‘ÍnTarkkñúgépÞ (Foetal hemoglobin = HbF) RbmaN 1° ekItBIExSGal;hVamYyKUr nig ExSháama:mYyKUr (a γ ). cMeBaHGñkCMgW taLasesmuI karplit ExSháama: (γ - chains) )anekIneLIgeday sarBagÁkayxitxMsMrbsMrYldl;kgVHxatkarplit eGm:UkøÚb‘Ín Ga (HbA) edaybegáInkarplit HbF. eTaHya:gNak¾eday karekIneLIgenHvaBMu RKb;RKan;eTACMnYsmuxgarrbs; HbA Edl)at;bg;eLIy. manGñkCMgW taLasesmuIxøHGacTTYlCatMNBUCpgEdrnUvlkçx½NÐmYyehAfa tMNBUC GciéRnþy_én HbF (Hereditary Persistance of Fœtal Hemoglobin b¤ HPFH). eBlenaHkMrit HbF mankñúgkMritx<s;ehIymanCab; eBjmYyCIvit rbs;mnusSFM. GñkCMgWk¾GacTTYlCatMNBUCpgEdrnUvkarpøas;bþÚr 2 2

EdlmanP¢ab;mkCamYynUvkareP£acBn§úrbs; háama:køÚb‘Ín (γ globin genes) ¬]TahrN_ γ Xmn polymorphism¦ b¤ P¢ab;mkCamYynUvlkçxNÐepSgeTotdUcCa β- Thalassemia EdlGaceFVI[mankarekIneLIg karsMeyaK HbF elImnusSFM ¬BI 5 20°¦ ehIykarenHvabnßyPaBF¶n;F¶rrbs;taLasesmuIedaybnßy GtulüPaBénExSkøÚb‘Ín (Globin chains). eraKsrIrsaRsþ rbs;CMgWtaLasesmIu (Pathophysiology of (Mutations)

Thalassemia)

kgVHExSEbtta ( β- chains) ehIyelIsExSGal;hVa (a- chains) kñúgGMLúgeBlmanépÞeBaH Qamrbs;TarkpÞúkeGm:UkøÚb‘ÍnRbePTBiess müa:geQμaHfa eGm:UkøÚb‘ÍnTarkkñúgépÞ (Fœtal hemoglobin = HbF). dUc)anCMrabxagelI/ HbF ekItBIExSGal;hVamYyKUr nig ExSháama:mYyKUr (a γ ). HbF enHbMeBjmuxgardwknaMGuksIuEhSn kñuúgsarBagÁkaydUc eGm:UkøÚb‘ÍnFmμtaEdlbMeBjmuxgarkñúgxøÜnkumar nig mnusSFMEdr. eRkayBITark RbsUt/ HbF bnþbMeBjmuxgarFmμtarhUtdl;TarkGayu 6 Ex eTIbvacab;epþIm CMnYsbnþicmþg²edayeGm:UkøÚb‘Ínrbs;mnusSFM (HbA). HbA ekItBIExSGal;hVa BIr nig ExSEbttaBIr (a β ). cMeBaHtaLasesmIukMritF¶n; BMumankarplitExSEbttaeT b¤ manplit EdrEttictYcbMput eFVI[KμankarsMeyaKecjCa HbA Fmμta)an ehIy b:HBal;ya:gF¶n;F¶rdl;smtßPaBrbs;eKalikaQamRkhm kñúgkardwk naMGuksIuEhSn. dUecñaH xøÜnkumareqIøytb edaybnþkarplit HbF. Etya:gNak¾eday vaBMuGacplit)anbrimaNRKb;RKan; edIm,IeqøIytb tamesckþIRtUvkarrbs;sarBagÁkaykumarEdlecHEtFMFat; nig edIm,ICMnYsmuxgardwkCBa¢ÚnGuksIuEhSnrbs; HbA (a β ) )aneLIy. karmanExSEbttabnþci bnþcÜ b¤kK¾ aμ nesaH k¾manplGviCm¢ anepSgeTot 2 2

Edr. eBlEdlsarBagÁkayeyIgbnþplitExSGal;hVaCaFmμta EtExSEbtta minRKb;RKan;edIm,Icab;KUKña ExSGal;hVaecHEtekIneLIg²kkkujeRcIn. karkk kujenHvarMxandl;karpliteKalikaQamRkhmrbs;sarBagÁkay edaykat; bnßykarplitrhUtdl; 95°. edaymaneKalikaQamRkhmeBjGayuEt bnþicbnþÜc sarBagÁkayecjCasPaBesøksøaMgxøaMg. elIsBIenHeTot ExSGal;hVaelIslubkkpMþúkñúgeKalikaqamRkhmeBjGayu kñúgcrnþQam eFVI[Pñasrbs;vagayxUcxat ehIyEbkFøayEdlritEteFVI[ PaBesøksøaMgkan;EtF¶n;F¶r. dMeNIrEbkeKalikaQamRkhmeKehAfa eGm:UlIs (Hemolysis) ¬mkBIBaküRkik Hem )anesckþIfa {Qam}/ lysis )anesckþIfa {Ebk}¦. eGm:UlIs bgá[ b‘ÍlIruyb‘Ín (Bilirubin) mankMrit x<s;CagFmμta. b‘ÍlIruyb‘Ín CasarFatuKImIBN’elOgEdl)anmkBIkarrMlay eGm:UkøÚb‘Ín (Hemoglobin) ehIyEdlRtUv)anbeBa©jedayeKalikaQam RkhmxUcxatkñúgdMeNIrrbs; eGm:UlIs. b‘ÍlIruyb‘Ín mankMritx<s;eFVI[EPñk nig Es,kGñkCMgWtaLasesmIukMritF¶n; mansPaBBN’elOg eKehAfa “Icteric”. EsþgecjCalT§plénkgVHeKalikaQamRkhm GñkCMgWtaLasesmIukMritF¶n; mansPaBesøksøaMg/ Gs;kMlaMg ehIyebHdUgedIrjab;eRBaHkgVHGuksIuEhSn. karenHnwgeFVI[ebHdUgrIk. GñkCMgWmankarFMFat;ragÁkayyWtya:v ehIyman karlM)akeFVIskmμPaBragÁkay. PaBesøksøaMgmanCaRbcaM mineqøIytb nwgkarBüa)al. kumareTACaexSay elVIy ehIymYrem:A nig yMxusFmμta. kgVHeKalikaQamRkhmF¶n;F¶r k¾nwgjauMg[manynþkarkarBarmYycMnYn ekIteLIgkñúgsarBagÁkay sMxan;CageK KWbegáInskmμPaBxYrq¥wg. dUc)anCMrab ehIyfa eKalikaQamRkhmplitenAkñúgxYrq¥wg. edIm,IRbwgTb;Tl;nwgkar fycuHéneKalikaQamRkhmkñúgcrnþQam xYrq¥wgbegáInskmμPaBelIsBIFmμta RbmaNCa 30 dg edIm,IpliteKalikaQamRkhm[)aneRcIn. karbegáIn skmμPaBenHk¾)anCMruj[q¥wg RtUvEtrIkFMEdr bgá[q¥wgk,almanTMrg;xusBI

Excess α Globin Chains

Major cause of anemia

Precipitate in early nucleated erythroid progenitors in marrow

Lead to ineffective erythropoiesis

Precipitate in mature red cells

Lead to hemolysis

Undergo proteolysis

-- absorbance o..o..o

radioactivity

-- absorbance o..o..o

radioactivity

Fmμta eFμjelIlyecjxøaMg ehIynwgkarrmYlénq¥wgCMnI nig q¥wgkgxñg . q¥wgmansPaBesþIgCagFmμta (Osteopenia) ehIypuy CYnkalgaynwg)ak;. eKalikaQamRkhmRtUveRcaHenAGeNþIk (Spleen) EdlCasrIragÁTTYlxusRtUv kñúgkarkMeTcecalral;eKalikaQamRkhmEdlcas;RKb;GayuRtUvxUc. Etya:gNak¾eday cMeBaHGñkCMgWtaLasesmuI GeNþIkRtUvra:b;rgkMeTceKalika QamRkhmd¾eRcInsem,Im edaysarxYrq¥wgmanskmμPaBplitxøaMg. CalT§pl GeNþIkxøÜnÉgRtUvEtskmμCagFmμta edayRbwgkMeTceKalika QamRkhmEdlelIs ehIyvak¾cab;epþImrIkFM eKehAfa Hypersplenism. bEnßmelIkarkMeTceKalikaQamRkhmEdlcas; GeNþIkEdlskmμxøaMg k¾kMeTcpgEdrnUveKalikaQams/ pøaEkt nig eKalikaRkhmekμgx©I/ karenHva rwtEteFVI[kgVHRKab;QamRkhmkan;EtF¶n;F¶reLIg. eKalikaQamRkhm EdleFVIdMeNIrkñúgcrnþQamGñkCMgWtaLasesmIu CaeKalikamanragxusFmμta edaysarxVHm:UelKul eGm:UkøÚb‘ÍnFmμta ehIyrEmgeTACab;kl;enAkñúgGeNþIk eFVI[GeNþIkkan;EtFM. TIbBa©b; GeNþIkenHÉgb:unb:gcat;viFankarN_ Tb;kgVH eKalikaQamRkhmrbs;sarBagÁkayedayplitRKab;QamRkhmedayxøÜn ÉgEtmþg ¬karbegáItRKab;QamRkhmenAeRkAxYrq¥wg¦ karenHGeNþIkrwtEt rIkkan;EtFM. dUcenH karelIsExSGal;hVa ehIyKμantulüPaBcMnYnrvagExSGal;hVa nig ExSEbtta CabuBVehtucMbgbgá[ekItCMgWtaLasesmuIkMritF¶n; CakarERbRbYl muxgarEdlCab;Tak;TinCamYyCMgW. sarBagÁkayxitxMTb;Tl;nwgkarkgVHeKalikaQamRkhmF¶n;F¶redayRsUb ykCatiEdkBIcMNIGahar tambMBg;rMlayGaharkan;EteRcIn. eTaHya:genHkþI vaRKan;EteFVI[sPaBkarN_kan;Etya:b;eTAeTot ³ GñkCMgWtaLasesmuITTYlrg kgVHeKalikaQamRkhmedaysarkarplitRKab;QamRkhmBMuRKb;RKan; BMu EmnedaysarkgVHCatiEdkeT. karRsUbCatiEdkkan;EteRcIn vaeFVI[sarBagÁkayRbQmmuxnwgeRKaHfñak;fμI KWelIsbnÞúkCatiEdk. 22

kMNt;eraKvinicä½yCMgWtaLasesmuI kumarekItmkmantaLasesmuI nwgminbgðajeraKsBaØa[eXIjeLIy. sUm,IEt etsþmnÞIrBiesaFn_k¾braC½ykñúgkarrkeraKvinicä½yEdr CaBiessebIeKmin)aneFVI etsþ«Bukmþay min)aneFVIetsþmuneBlTarkekIt ehIykñúgRKYsarKμankUnman CMgWenH. ehtupllM)akeFVIeraKvinicä½ytaLasesmuIkñúgdMNak;kalGayudMbUg rbs;kumaredaysarvaman HbF kñúgbrimaNRKb;RKan;edIm,IFana)annUv tulüPaBkñúgcMnYnExSkøÚb‘Ín ¬Gal;hVa nig háam:a¦ EdlCaGñkbegáIt HbF KWeFVI[kumartUc²eCosputBIdMeNIrGsmtßPaBkñúgkarpliteKalikaRkhm dUc)anerobrab;xagedImmkehIy. vaGacmanlT§PaBeFVIeraKvinicä½ytaLasesmuI enAGayudMbUg)aneday meFüa)aybec©keTsm:UelKul EdlsMKal;nUvkarpøas;bþÚr (Mutations) Edlkumar)anTTYlCalkçN³tMNrBUCBI«Bukmþay. eTaHya:gNak¾eday kareFVIetsþenHTMngCaGnuvtþn_eBlsgS½yBiessNamYy Ca]TahrN_ eBlTarkekItBI«BukmþayEdlCa GñkpÞúkBn§úbgáCMgW (Carriers). CaGkusl eTaHbICamankmμviFIBinitüRsavRCav (Screening programme) elITarkeTIb ekItya:gl¥k¾edayk¾kareFVIetsþrkeraKvinicä½yCMgWtaLasesmuIkMritF¶n; swgEt minGac[snñidæan)anenAdMNak;kalGayudMbUgeLIy. eTaHya:gNak¾eday karBinitüRsavRCav GacmanRbeyaCn_kñúgkareFVIeraKvinicä½y rkRbePTkøay epSg² dUcCa HbE b¤ HbS. kñúgkrNIPaKeRcIn taLasesmuIkMritF¶n;GaceFVIeraKvinicä½y)anmunGayu 2 qñaM. EttaLasesmuIkMritmFüm enAEtminGaceFVIeraKvinicä½y)ankñúgry³eBld¾yUr. taragxageRkam bgðajBIlT§plénkarsikSaEdleFVIeLIgedayGñkRsavRCav mYycMnYnelIbBaðaenH.

Gayu énkarbgðajeraKvinicä½yrbs;kumartaLasesmuIkMritF¶n; b¤ kMritmFüm (from Modell and Berdoukas 1984)

Gayu ¬qñaM¦ <1 1-2 >2

taLasesmuIkMritF¶n; 75 - 62° 35 - 29° 11 - 9°

taLasesmuIkMritmFüm 4 - 11° 11 - 30° 22 - 59°

viFIsaRsþEpñkelahitviTüa EdleKeRbICaTUeTAedIm,IeFVIeraKvinicä½y taLasesmuIkMritF¶n; (i) snÞTsSn_elahitviTüa ³ RKab;QamTaMgenHRtUv)anvas;Evgeday ]bkrN_eGLicRtUnic eKeRbIvaedIm,Ivas;TMhM nig maDeKalikaQamRkhm nig brimaNeGm:UkøÚb‘Ín (Hb) EdlmankñúgeKalikaRkhm. kMNt;eraK vinci y½ä taLasesmu)I aneBlNaTMhM nig maDeKalikaQamRkhm ehIynig kMhab;eGm:UkøÚb‘ÍnkñúgeKalikaRkhmTaMgenaHmankarcuHfyya:gxøaMg eday kMrit eGm:UkøÚb‘Ínrvag 2- 6 Rkam¼d>l ¬g/dl¦. xageRkamenH KWCa snÞTsSn_ elahitviTüamYycMnYn EdleKeXIjmanTUeTAelIGñkCMgW taLasesmuI ³ tMélmFüm KMlat ¬Range¦ Hb g/dl 6/8 3/9 - 9/3 ¬brimaNmFümén Hb kñúgbrimaNQammYyrgVal;¦ MCH pg

¬TMgn;mFümén

20/9

15 - 26

65/8

57 - 75

30/9

26 - 34

kñúgeKalikaQamRkhmnImYy²¦

MCV fl

¬TMhMmFümrbs;eKalikaQamRkhm¦ MCHC g/dl

¬brimaNmFümén 24

kñúgbrimaNeKalikaQamRkhmmYyrgVal; ¦

cMnYneKalikaQam>s emIleTAhak;dUcCaekIneLIg edaysarvtþmand¾eRcIn elIslb;éneKalikaQamRkhmekμgx©I ¬vaenAmansñÚl¦ Edl]bkrN_rab; RKb;QamGacyl;RcLMfaCaeKalikaQam>seTAvij. eTaHya:gNak¾ eday karRcLMenHgaynwgEjk)anedayGegátEpñkmnÞIrBiesaFn_bnÞab;mk eTot. (ii) karBinitüQamedayBaselIkBa©k; (Blood film) nig rUbrageKalika QamRkhm (RBC morphology) ³ BinitüedaymIRkUTsSn_ eKalikaQam RkhmmansPaBesøkCag (Hypochromic) ¬emIlrUb 2k¦ ehIy tUcCagFmμta (Microcytic). sMxan;bMputenaH PaKeRcInmanragxus Fmμta xøHtUcxøHFM (Anisocytosis)/ xøHmanragreqjrqUj (Poikilocyte). (iii) eGLicRtUhV½rEr:séneGm:UkøÚb‘Ín (Hb electrophoresis) ³ enHCadMeNIrmYyEdlEjkRbUetGIunepSg² EdlbegáItCam:UelKul eGm:UkøÚb‘Ín (Hb) dUcCa HbA, HbA nig HbF. eraKvinicä½ytaLasesmIu GaceFVI)aneBlkMrit HbF x<s;CagFmμta ehIyGacERbRbYlenAcenøaH 20 - 90°. HbA EdlFmμtavamanrhUtdl; 3° éneGm:UkøÚb‘Ín ³ GacKμan GacfycuH GacenAFmμta b¤ k¾ekIneLIgbnþic. (iv)viFIviPaKm:UelKul (Molecular methods) ³ enHKWCameFüa)ayBiess edIm,IbBa¢ak; b¤ TTYl)anBt’manBitR)akdkñúgkareFVIeraKvinicä½yedayGegát elI DNA/ ]TahrN_edIm,IkMNt;karpøas;bþÚr (Mutations) EdlbgáCMgW. eRkABIbBa¢ak;)annUveraKvinicä½y vak¾pþl;Bt’man[dwgpgEdrBIsPaBF¶n;F¶r EpñkKøInikrbs;CMgW. karGegátBinti üRKab;Qam k¾dcU CakarBinti ükarpøas;brÚþ rbs;Bn§ú a/ β nig γ KWCaCMhand¾sMxan;kMNt;eraKvinicä½ytaLasesmuIpg nig kMNt;karBüa)al pg. eTaHbIeraKvinicä½y Ebtta taLasesmIukMritF¶n; EtgEtgayRsYl

karlM)akk¾GacekItmanEdr CaBiessenAbNþaRbeTskMBugGPivDÆn_ Edl manGRtaQWeRcIn dUcCaCMgWRKuncaj;GaceFVI[sμúRKsμajdl;kareFVIeraK vinicä½y. ]TahrN_ RKuncaj;Gacbgá[xVHeKalikaQamRkhm nig GeNþIk rIkFM ehIyeTaHCakarrkeXIjEpñkelahitviTüarbs;mnÞIrBiesaFn_vaxusKña k¾eday vaGaccaM)ac;faRtUvBüa)alCMgWedayfñaMRKuncaj;sin munnwgeFVIkar vaytMéleLIgvijnUvsPaB nig eraKvinicä½yrbs;CMgWtaLasesmIu. CMgWepSg²eTot k¾Gacbgá[xVHRKab;QamRkhm nig GeNþIkrIkFMEdr ehIy HbF ekIneLIgpgEdr ehIykareFVIeraKvinicä½yEjk KWCakarcaM)ac;eday Binti üKønI ki nigviPaKenAmnÞrI BiesaFn_bEnßm. vamansarsMxan;Nas;EdlRtUv bBa¢ak;eraKvinicä½ytaLasesmuI[)anBitR)akdmuneFVIkarBüa)al. CMgW eGm:UkøÚb‘Ín (Hb) kMritF¶n;epSg² CMgW ExSGal;hVa køÚb‘Ín

CMgW ExSEbtta køÚb‘Ín

(a - globin chain disorders)

(β - globin chain disorders)

a - thalassemias

Sickle cell disorders

¬Gal;hVa taLasesmuI¦

¬CMgW ekasikasIukxWl¦

HbH disease

sickle cell anemia (HbSS)

a - thalassemia hydrops foetalis

HbS/β - thalassemia

(= Hb Bart’s hydrops foetalis)

HbSC disease

HbSD disease

CMgW sIukxWl epSg²eTot

β - thalassemias

¬Ebtta taLasesmuI¦

β - thalassemia major

¬Ebtta taLasesmuIkMritF¶n;¦

β - thalassemia intermedia

¬Ebtta taLasesmuIkMritmFüm¦

HbE/β - thalassemia

CMgW taLasesmuI kMrepSg²eTot

karBüa)al Ebtta taLasesmuIkMritF¶n; ry³eBl 3 TsvtSr_cugeRkayenH karBinitüGegátEpñkKøInik nigkarRsavRCav )anbgðajfa taLasesmuIkMritF¶n;CaCMgWmYyGacBüa)al)an. karsikSa)an bgðajfa karBüa)aledaybBa©ÚlQamCaeTogTat;nUvQamEdlBinitü)an RtwmRtUv nig mansuvtßiPaBedaycMraj;ykCatiEdkya:gmanRbsiT§iPaB nig eTogTat; )anbegáInKuNPaB nigkarrs;ranmanCIvitrbs;GñkCMgW)anya:geRcIn . rebobBüa)alEdleKENnaMenH eKp©g;eTAelIkarRbyuT§nwgGRtakgVHeKalika QamRkhmkñúgCMgWtaLasesmuI nig plvi)akrbs;va ehIynigp©g;eTAelI karkarBarkMu[manelIsCatiEdkkñúgCalikabnþicmþg² EdlbNþalmkBICMgW xøÜnÉgpg nigBIkarBüa)aledaykarbBa©ÚlQampg EdleRbIsMrab;Tb;Tl; nwgkgVHeKalikaQamRkhm.

CMBUkTI 3 karBüa)aledaykarbBa©ÚlQam karbBa©ÚlQamedayeTogTat; )anrYmcMENkd¾FMeFgdl;KuNPaB nig Gayu kalCIvitGñkCMgWtaLasesmuIkMritF¶n; ehIyCaTidæPaBrYménkarBüa)al taLasesmuI cab;taMgBITsvtSr_qñaM 1960. ebIminmancMNat;karedayRbsiT§iPaBeT kgVHRKab;QamRkhmF¶n;F¶r nig karrIkFMRCulénlkçN³xYrq¥wgrbs; taLasesmuIkMritF¶n; nwgnaMeTArk ³ karrIkFMFat;yWtya:v karxusTMrg;minFmμtaénq¥wgmux nig q¥wgepSg²eTot q¥wgBukpuy nig kar)ak;q¥wg eføIm nig GeNþIkrIkFM karxUcxatdl;skmμPaBragkayFmμta karbBa©ÚlQamya:geTogTat;elImUldæaneFVIeLIgGs;mYyCIvit GacTb;Tl; b¤ QaneTAdl;karBar)ankMu[ekItmaneraKsBaØa ¬rhUtdl;mankarBüa)al taLasesmukI rM ti F¶ne; GayCadac;NamYydak;[Gnuvtþn¦_ . ya:gNak¾eday manktþaCaeRcInRtUvEtykmkKitKUr eBlcab;epþImkarBüa)aledaykarbBa©Úl Qam. CMBUkenH nwgBiPakSaGMBI ³ 1> etIeBlNaKYrcab;epþImkarBüa)aledaybBa©ÚlQam. 2> eFVIdUcemþcedIm,IFana[)ankarbBa©ÚlQamRbkbedaysuvtßiPaB. 3> etIeKRtUvbBa©ÚlQamRbePTGVI. 4> eFVIdUcemþceTIb)anQamsmRsbtamrebobrbbénkarBüa)al edaybBa©ÚlQam .

etIeBlNaRtUvcab;epþImBüa)aledaybBa©ÚlQam karBüa)aledaybBa©ÚlQamRtUvcab;epþImEteBlNa eraKvinicä½ytaLasesmuI kMritF¶n;RtUv)anbBa¢ak;c,as;. dUc)anniyayxagelI/ eraKvinicä½ytaLasesmuI kMritF¶n; Ep¥keTAelI ³ (i) etsþmnÞIrBiesaFn_/ ]TahrN_ viFIviPaKm:UelKul/ eGLicRtUhV½rEr:s éneGm:UkøÚb‘Ín nig etsþmnÞIrBiesaFn_epSgeTot dUcCa High Pressure Liquid Chromatography (HPLC). (ii) karviPaKeTAelIBn§ú (Genetic analysis) edIm,IsÁal;RbePTénkarpøas;bþÚr kñúgCMgWtaLasesmIu Ebtta nig Gal;hVa k¾dUcCavtþman Xmn1 restriction enzyme site EdlCakMNt;sMKal;mYyCYy[eyIgBüakrN_ BIsPaBF¶n;F¶énCMgW nig kMNt;BIrebobrbbBüa)alEdlsmRsbbMputcMeBaH GñkCMgWnImYy². PaBF¶n;F¶rénkgVHRKab;QamRkhm EtgEtRtUv)anvaytMéledayEp¥kelI mUldæanénkMrit eGm:UkøÚb‘Ín (Hb) kñúgQam. eKKitCaRkamkñúgQammYy edsIulIRt ¬d>l = 1¼100 lIRt¦. eGm:UkøÚb‘Ín gayRsYlvas;EvgkñúgmnÞIr BiesaFn_edayeRbI ma:sIunrab;RKab;Qam. viFIcas;dUcCabec©keTs salI (Sahli technique) k¾Gacvas;Evg eGm:UkøÚb‘Ín KYr[Tukcitþ)anEdr. kMrit eGm:UkøÚb‘Ín Fmμta CaTUeTAeKKitfaenAcenøaH 13 - 16 Rkam¼d>l ¬g/dl¦ cMeBaHburs ehIy 11 - 14 Rkam¼d>l cMeBaHRsþI nig kumar. TaMgburs TaMgRsþI eGm:UkøÚb‘Ín enAcenøaH 8 - 11 Rkam¼d>l tMNag[kgVHeKalika QamRkhmkMritmFüm. ebIkgVHeKalikaQamRkhm kMritF¶n; eGm:UkøÚb‘Ín ticCag 8 Rkam¼d>l. GñkCMgWRtUvcab;epþImbBa©ÚlQamEteBlNataLasesmIu RtUv)anbBa¢ak;tamry³eraKvinicä½yedaymnÞIrBiesaFn_ nig karsikSam:UelKul ¬dUc)anerobrab;xagelI¦ ehIyeBl 29

Edl ³ kMrit eGm:UkøÚb‘Ín (Hb) RtUv)ankt;RtaticCag 8 Rkam¼d>l kñúgGMLúgeBl BIrdgbnþbnÞab;Kña XøatBIKñaCag 2 s)aþh_. CYnkalGñkCMgWGacmankMlaMg BlM ehIyrIkFMFat;FmμtaeTaHCakMrti eGm:UkbÚø nÍ‘ enAcenøaH 6 - 7 Rkam¼d>l k¾eday EtkarsMerccitþfaminbBa©ÚlQamkñúgeBlenH TamTarnUv bTBiesaFn_KøInikd¾FMeFg nig karBinitüedayykcitþTukdak;. kMrti eGm:UkøÚb‘Ín elIsBI 7 Rkam¼d>l b:Eu nþPa¢ b;CamYysBaØarUbkay dUcCa ³ - TMrg;muxERbRbYl - minFMFat; nig kareLIgTMgn;tictYc - )ak;q¥wg - karplitRKab;QamenAeRkAxYrq¥wg bgðajedayeføIm nig GeNþIkrIkFM ebImanbBaðaTaMgenH karbBa©ÚlQamminRtUvBnüaeBleLIy. Fana[)ankarbBa©ÚlQammansuvtßiPaB QamRtUvEtmanRKb;RKan; b¤ gayrk)an edaykarbBa©ÚlQam KWCakarBüa)aleFVIeLIgGs;mYyCIvitcMeBaH taLasesmuI GaC£aFrsuxaPi)alRtUvCMrujeKalneya)aypþl;QamedIm,I Fana[)annUvkarpÁt;pÁg;Qamdl;GñkCMgW)anRKb;RKan; nig CaGciéRnþy_. karBar[)ankMu[mankarcMlgPñak;garbgáeraK karbBa©ÚlQamRtUveFVIeLIgedaysuvtßiPaB tamEdlGaceFVIeTA)ansMrab; GñkCMgW. enH)anesckþIfa eRKaHénPñak;garbgáeraKdUcCa vIrus/ )ak;etrI b¤ )a:ra:sIut EdlqøgtamQampþl;eTAGñkCMgW RtUveFVIya:gNa[mantictYc bMput. edIm,Ikat;bnßyeRKaHfñak;qøgPñak;garbgáeraKtamry³karpþl;Qam/ GaC£aFrsuxPaBsaFarN³RtUv ³

- CMrujeKalneya)ayeRCIsykGñkpþl;QammansuxPaBl¥ nig CMrujesva pþlQ ; amedaysμR½ Kcitþ nig CaeTogTat;. karpþlQ ; amedaybg;R)ak;kas RtUveCosvag ehIyeKminTamTar[GñkCMgWrkmitþPkþi b¤ bgb¥ÚnedIm,ICafñÚr sMrab;karbBa©ÚlQam. - Qamrbs;Gñkpþl; RtUvEtBinitürkPñak;garbgáeraKsMxan;²EpñkKøInik dUcCa vIrusrlakeføImRbePT B nig C/ HIV1+2 ¬Pñak;garbgá[ekItCaCMgW eGds¾¦ nig eraKsVay. enARbeTsmYycMnYn GacfacaM)ac;RtUvBinitürk Pñak;garbgáeraKepSg²eTot dUcCaRKuncaj; cMeBaHtMbn;NaEdlvaman. - RtUvFana[)anfa bNþaesvaCatipþl;QamrYmTaMgmnÞIrBiesaFn_ nig esva epSg²eTotpþl;edaymCÄmNÐlpþl;Qam dUcCa karsþúk nig karbBa¢Ún plitplQamRtUveqøIytbeTAnwgniyamGnþrCati. EhSNUTIbRkumQam (Blood group genotype) suvtßiPaBQamk¾Cab;Tak;TgCamYyktþaepSg² dUcCa RtUvFana[)anfaGñkCMgW RtUv)aneFVIetsþsMrab;RbB½n§RkumQamsMxan;² tamEdlGaceFVIeTArYc. ]TahrN_ GñkCMgWRtUveFVIetsþrkRkumQamrbs;BYkeK ehIyRtUvdwgfa erhSús (Rhesus = Rh) vIC¢man b¤ GviC¢man rYmCamYyRbB½n§RkumQamepSg²eTot dUcCa Kell, Kidd nig Duffy . eBlcab;epþImbBa©ÚlQamPøam/ vaGaclM)ak kñúgkarkMNt;RbePTRkumQamTaMgGs;Edlman. dUecñHvasMxan;RtUveFVIetsþrk ehSNUTIbeKalikaQamRkhm munbBa©ÚlQamelIkdMbUg. ykl¥GñkCMgWRtUv eFVIetsþrkvtþmanGgÁbdibkçfμI² cMeBaHeKalikaQamRkhmmunerogral;kar bBa©ÚlQam. karpÁÚrpÁg (Matching) [)aneRcIntamEdlGaceFVIeTA)annUv RbB½n§RkumQamGñkCMgW nig Gñkpþl; KW CameFüa)ayénkarkat;bnßyRbtikmμ EdlmanCamYyRkumQamminsIuKña. eKalikaQamRkhmmanRbB½n§RkumQam 26 rYmmanGg;TIEhSn 600 xus²Kña. eBlEdleyIgminGacpÁÚrpÁg [)an TaMgGs;eT/ ya:gehacNas;k¾RtUvxitxMpÁÚrpÁgRbB½n§RkumQamEdlman jwkjyCageKEdr dUcCa ABO, RbB½n§ Rhesus nig Kell. 31

etIQamGVIRtUvbBa©Úl QamEdlRbmUl)anBIGñkeFVIGMeNay eKehAfa QamTaMgmUl (whole blood) EdleRkABImaneKalikaQamRkhm vak¾manpøasμa/ eKalikaQam>s nig pøaEkt ¬sUmemIlvKÁsþIBIQam¦. RbmaN 450 m>l énQam ¬= 1 Ékta¦ EdlbUmBIGñkbric©aKQammñak;². eBlNapøaEkt/ eKalikaQam>s nig ekasikaepSg²eTotRtUv)andkecj/ enAsl; RbmaN 250 m>l eTotCaeKalikaQamRkhmeKehAfa mYyÉktaéneKalikaQamRkhm Rckkñúgfg;TuksMrab;bBa©Úl. vtßúravepSg²eTotmandUcCa sarFatuTb; kMNkQam (Anticoagulant) nig sarFatucBa©wmRKab;Qam k¾RtUv)anbEnßm cUlkñúgfg;QamEdr ehIybrimaNvaGaRs½yelIviFIykQammkerobcM. GñkCMgWtaLasesmIukMritF¶n; mankarxVHeKalikaQamRkhm. dUcenHGñkCMgW EdlRtUvkarbBa©ÚlQam ykl¥KYrEtTTYlEteKalikaQamRkhmsuT§ehIy KμanpÞúksarFatuepSgrbs;QamTaMgmUleLIy dUcCa )aøsμa/ eKalikaQam>s nig pøaEkt. RbsinebIGñkCMgWTTYlQamTaMgmUl vamaneRKaHfñak;edayehtufa RbB½n§srésQamnwgTTYlpÞúkelIscMNuH ehIyvivDÆn_eGaymanplvi)ak epSg² dUcCa exSayebHdUg elIsCatiTwkkñúgtMbn;RTUg ¬ehImsYt¦. karyk eKalikaQam>s nig pøaEktecjBIQamTaMgmUl k¾CYybnßyeRKaHfñak;énpl EdleyIgmincg;)an dUcCaekþAxøÜnGMLúgeBl nigeRkaykarbBa©ÚlQam. eTaHCasBaØaTaMgenHGacBüa)alk¾eday eKRtUvEtxitxMbeBa¢ósnUvplvi)ak TaMgenH edaypþl;EtsmasFatuénQamNaEdlGñkCMgWRtUvkar. etIeKTTYleKalikaQamRkhmdak;fg;dUcemþc eKalikaQamRkhm RtUvEjkBIQamTaMgmUlenAÉmnÞIrBiesaFn_ tamry³ dMeNIrénkarbgVil (Centrifugation) EdlGacEjkecj)an pøasμa nig eKalikaQam>s BIQamTaMgmUldl;Cag 70°. kñúgdMeNIrénkarbgVilQam/ eKalikaQamRkhm Ejkdac;BIeKedayenA)at Épøasμa/ eKalikaQam>s nig 32

pøaEktenAxagelI eFVI[gayRsYldkykecj. sUluysüúgmYycMnYnGac bEnßmeTAelIeKalikaQamRkhm dUcCa sarFatuTb;kMNkQamEdlkarBar ekasikakMu[s¥itCab;Kña/ sarFatuciBa©wm b¤ sarFatubEnßmEdlGacBnüaCIvit nig EfrkSaKuNPaBeKalikaQamRkhm dUcCa SAGM (saline, adenine, glucose, mannitol), PAGGS-M (phosphate, adenine, glucose,

nig ADSL (adenine, dextrose, saline-mannitol) suT§swgCasUluysüúgbEnßmEdl eKeRbI. CPD (citrate, phosphate, dextrosel), CPDA-1 (citrate, phosphate, dextrose, adenine) nig CP2D (citrate, phosphate, double dextrose) suT§swgBYkRbqaMgkMNkQam. enAGWr:ubeKeRbIbBa©ÚlKñanUv CPD/SAG-M edIm,IrkSaCIvitrbs;eKalika QamRkhm dl; 42 - 48 éf¶/ ÉenAshrdæGaemrikvij eKeRbIbBa©ÚlKña CPDA-1/AS-3 b¤ eRbI CP2D/AS-3. GñkCMgWtaLasesmuI RtUvTTYlkarbBa©ÚlQamedayeRbIeKalikaQamRkhm suT§dak;fg; Cakarl¥QamEdlrkSaTukmin[elIsBI 7 éf¶. sUm,IenAkEnøgNa EdlmaneRbIsarFatuciBa©wmeKalikaQamRkhmdUcerobrab;xagelIk¾eday/ eKminRtUvbBa©ÚlQam[GñkCMgWnUvQamEdlrkSaTukelIsBI 2 s)aþh_eLIy BIeRBaHBMuTan;mankarsikSaKYr[eCOCak;NamYyEdlGHGagfa eKalika QamRkhmEdlsþúkkñúgry³eBlyUr vapþl;RbeyaCn_kñúgkarBüa)alCMgW taLasesmuIkMritF¶n;)anl¥dUceKalikaQamRkhmEdlsþúkkñúgry³eBlxøI eLIy. viFIepSg²eTotEdlGaceFVI[RbesIrnUvKuNPaB nig suvtßiPaBQam rYmman ³ i. lagsMGateKalikaQamRkhmEdldak;kñúgfg; ³ vtþmanRbUetGIunkñúgQambBa©ÚleGayGñkCMgW Gacbgá[manRbtikmμkñúgxøÜn GñkCMgWIBIkMritmFümeTAxøaMgkøa)an. eTaHbIdMeNIrénkarbgVilQamdUc erobrab;xagelI)andkecjCag 70° énpøasμa nig RbUetGIunBIQam guanosine, saline-mannitol), AS-3 (Nutricel system)

TaMgmUlk¾eday k¾KuNPaBéneKalikaQamRkhmdak;fg; GacnwgeFVI[ RbesIreLIg)aneRcInEfmeTot edaykarlagvakñúgTwkesrU:méRb (Saline solution) edIm,Idk[)anGtib,rmanUvpøasμa nig RbUetGIun. CaTUeTA/ cMeBaH GñkCMgWPaKeRcIn minTamTar[eFVIkarlageKalikaQamRkhmenaHeT. karlageKalikaQamRkhmeKRtUveRbIeBlrvag 24 ema:g. ii. sMGateKalikaQam>s BIfg;eKalikaQamRkhm ³ mKÁúeTsn_GnþrCati)anENnaMfa cMnYneKalikaQam>s RtUv[ticCag 1 x 10 kñúgmYyÉktaéneKalikaQamRkhm edIm,I[Rbtikmμbgáeday eKalikaQam>s sßitkñúgkMritGb,rma. RbtikmμTaMgenHEdlEtgEtbgá[ manekþAxøÜn eKeCOfaedaysarEteKalikaQam>s beBa©jnUvsarFatuKImI eQμaHfa exm:UKIn (Chemokines). elIsBIenHeTot karykeKalika Qam>secj mann½yfa ykPñak;garbgáCMgWEdlmankñúgeKalikaQams ecj dUcCa )ak;etrI Yersinia Enterocolitica, CMV, EBV, B19 nig HAV ¬sUmemIlenATMB½rBakübMRbYj¦ EdlCaPñak;garbgáCMgWedaymin)ansMGat enAmCÄmNÐlpþl;Qam ehIykñúglkçxN½ÐxøH vanwgbgá[ekIteraKF¶n;F¶r. viFIsMGateKalikaQams (Leucoreduction) man ³ ¬k¦ kareRcaH (Filtration) ³ eKalikaQam>s nig Pñak;garbgáCMgWEdlman Cab;CamYyvaGacRtUv)andkecjBIQamrbs;Gñkbric©aKya:gman RbsiT§iPaBbMputedayeRbItMrgBiessEdlP¢ab;nwgfg;Qam. mKÁúeTsn_ GnþrCati)anENnaM[eFVIcMeraHQameRkayBIRbmUlQam)an rvag 4-8 ema:g edaycenøaHeBlenHeKsþúkvakñúgTUrTwkkksin. karBnüakñúgkareFVI cMeraHenH GacTuk»kas[katalIkrkñúgeKalikaQam>s (Phagocytes) eFVIkarkMeTc)ak;etrI (Phagocytosis) dUcCa )ak;etrI Yersinia Enterocolitica EdlvaGaccUlkñúgeKalikaQam>s ehIyEbkkUnenAkñúg enaH ¬emIlrUbenATl;mux¦. cMeraHGaceFVI)anenAmCÄmNÐlpþl;Qam b¤ enAERKrbs;GñkCMgWkñúgeBlbBa©ÚlQam. eTaHbIkareFVIcMeraHenAERKrbs; 6

GñkCMgWGaceFVI)anedaymanRbsiT§iPaBk¾eday EtvaGaclM)aknwgFana )annUvkarRtYtBinitüKuNPaB. kñúgb:unμanqñaMcugeRkayenH kareFVIcMeraHeKalikaQam>s (Leucofiltration) kan;Etmansar³sMxan;bEnßmeTot CaBiesssMrab;GñkCMgW Ebtta taLasesmuI v½yekμgEdl)aneFVI b¤ manEpnkarRtUveFVIkarbNþúHxYrq¥wg (Bone marrow transplatation). enHk¾edaysareKalikaQams manP¢ab;mkCamYy nUvvIrusmYyehAfa sIutUem:háaLÚvIrus (Cytomegalovirus) EdlGacbgáeraK F¶n;F¶r b¤ rhUtdl;søab; cMeBaHGñkCMgWEdlmanRbB½n§karBarexSay (Immune supressed) dUckrNIGñkCMgWbNþúHxYrq¥wg CaedIm. eBlEdlmCÄmNÐlpþl;QamBMumanlT§PaBGnuvtþkarEjkpøasμa b¤ lag b¤ cMeraHQamtamviFIdUcerobrab;xagelI)an/ QamTaMgmUlEdlbUm)anBIGñkpþl; RtUvsþúkTukkñúgTUrTwkkkenAsItuNðPaB 4oC ry³eBl 24 - 48 ema:g/ Tuk[ eKalikaQamRkhmrgenA)atfg;Qam ehIypøasμa/ eKalikaQam>s/ pøaEkt nig FatuepSg²eTot EdlTaMgGs;enHeKehAfa RsTab;BN’etñat vaGENþt mkEpñkxagelIEdleKGacykvaecj)angaybMput edayeRbI]bkrN_mnÞIr BiesaFn_samBaØ. eTaHya:gNak¾eday/ viFIEbbenHmanRbsiT§iPaBticCag 1000 dg eRbobeFobnigkareRcaHQam. RbsiT§iPaBénbec©keTsepSg²kñúgkarcMraj;eKalikaQam>secj RsTab;BN’etñatEdlykecjBIQam eRkayBITukeGayrgedayxøÜnÉg kñúgTUrTwkkk/ karenHQamenAsl;maneKalikaQamsticCag 1,2 x 10 /Ékta. eKalikaQamRkhmEdlEjk)anedaykarbgVilQam nig karlagkñúg TwkesrU:méRb ¬Saline solution¦/ vamaneKalikaQams ticCag 1 x 10 /Ékta. eKalikaQamRkhmEdl)anBIcMeraHeFVIenAÉERKGñkCMgW/ vamaneKalika

blood filter

phagocyte filled with bacteria

Qam>s ticCag 5 x 10 /Ékta. eKalikaQamRkhm Edl)anBIcMeraHeFVIenAÉmnÞIrBiesaFn_mCÄmNÐl pþl;Qam/ vamaneKalikaQam>s ticCag 5 x 10 /Ékta. eKalikaQamRkhmEdleRcaHmunykmksþúkTuk/ vamaneKalikaQam>s ticCag 1 x 10 /Ékta. CakarRbesIr/ RKb;GñkCMgWtaLasesmIukMritF¶n;TaMgGs;RtUvTTYlkarbBa©Úl QamEdlCaeKalikaQamRkhmsuT§ Edl)aneFVIcMeraHmunykmksþúkTuk/ )anlagsMGat/ )aneFVIkarpÁÚrpÁgPaBRtUvKñaya:geBjelj ehIymanbEnßm sUluysüúg RbqaMgnwgkMNkQam nig sarFatuciBa©wmRKab;Qam. ya:gNa k¾eday eBlNaKμanlT§PaBeFVIeTA)an eKRtUvBicarNadl;karerobcMQam BiesssMrab;GñkCMgW EdlTamTarnUvlkçN³ dUcCa ³ 1> eKalikaQamRkhm Edl)anlagsMGat (Washed RBCs) RtUverobcMsMrab;GñkCMgWEdlxVH RbUetGIunBiesskñúgQam ehAfa IgA nig sMrab;GñkCMgWEdl]sSah_man RbtikmμGalELhSIúF¶n;F¶r (Severe allergic reactions) eBlBüa)aledaykarbBa©ÚlQam. GñkCMgWxøHKμan kgVH IgA eT EtmanekþAxøÜndEdl²eBlbBa©ÚlQamcMeraH krNIenHk¾ TamTar[lageKalikaQamRkhmmuneBlbBa©ÚlEdr. 2> eKalikaQamRkhmRtCak;kk (Frozen b¤ cryo-preserved RBCs) eRbIcMeBaHGñkCMgWEdlmanGg;TIEhSnkMrelIeKalikaQamRkhm eRBaH QamBYkeKBi)akeFVIkarsakl,grkPaBRtUvKña (Cross-match) CamYy QamGMeNay[)anc,as;las;. enAGWr:ub nig shrdæGaemrik/ eKalika QamRkhmRtCak;kkEdl)anBIRkumQamkMr eKrkSavakñúgmCÄmNÐl evC¢saRsþBiess edayrkSakMrit -60 C kñúgsUluysüúg Glycerol 40% EdlCalkçx½NÐmYyGac[eKsþúkva Tuk)anrhUt 10qñaM. 3> eKalikaQamRkhm)aj;edaykaMrsIμ (RBCs treated with radiation 6

eRbIcMeBaHGñkCMgWEdlTTYlkarbNþúHxYrq¥wg b¤ GñkCMgWeRtomTTYlkarbNþúH xYrq¥wg edIm,IeCosvagekItPaBF¶n;F¶rmYyehAfa CMgWTMnas;bnSaMsrIragÁ (Graft versus host disease) ¬GanenAxageRkay¦. 4> eKalikaQamRkhmEdl)andkeKalikaQam>secj (Leucoreduced RBCs) eRbIcMeBaHGñkCMgWEdl]sSah_manRbtikmμ CamYyeKalikaQam>s eRkaykarbBa©ÚlQam. QamEbbenH k¾Tukpþl; [pgEdrdl;GñkCMgWv½yekμg eTIbnwgeFVIeraKvinicä½y ehIymanKMeragRtUv eFVIkarbNþúHxYrq¥wg edIm,IecosvagkarbgáeraKeday sIutUem:háaLÚvIrus ¬Cytomegalovirus = CMV¦/ k¾dUcCaTukpþl;[GñkCMgW Edl)anbNþúH xYrq¥wgrYcehIy EtRtUvkarbBa©ÚlQam edIm,IeCosvagkarbgáeraKCafμIeday sIutUem:háaLÚvIrus. kMNt;rbbbBa©ÚlQam KNnatMrUvkarbrimaNQam brimaNQamEdlGñkCMgWRtUvkar nig kMritbBa©ÚlQamGaRs½yeTAelIGayu GñkCMgW nig sPaBKøInik/ eTAelIsUluysüúgbEnßm edIm,IrkSaeKalika QamRkhm/ eTAelIeGm:atURKIt (Hematocrite) rbs;QamGñkpþl; nigeTAelI kMritcg;)anrbs; eGm:UkøÚb‘Ín. PaKeRcInénGñkCMnajEpñkCMgWtaLasesmuI )aneGayGnusasn_fa karbBa©Úl QamRtUveFVIedaylkçN³mFüm min[elIs/ CaFmμta GñkCMgWTTYleKalika QamRkhmxab;kMrit 10 - 15 ml kñúgTMgn;mYyKILÚRkam/ bBa©Úlkñúg ry³eBl 3 - 4 ema:g ¬Ep¥kelImUldæan eGm:atURKIt 75° éneKalika QamRkhmrbs;Gñkpþl;Qam¦ ¬kMritbBa©ÚlQam¦ erogral; 2 eTA 5 s)aþh_ ¬KMlatbBa©ÚlQam¦. GñkCMgWminkat;GeNþIk RtUvkareKalika QamRkhmsuT§RbmaN 180 ml¼KRk¼qñaM/ ÉGñkCMgWkat;GeNþIkvij RtUvkar RbEhl 133 ml¼KRk¼qñaM. 37

krNIGkñ CMgmW anbBaðaebHdUg b¤ krNIbBa©lÚ Qamcab;epIþmeBlkMrit eGm:UkøÚb‘Ín (Hb) TabCag 5 Rkam¼d>l (g/dl) eBlenaHeK bBa©ÚlQamkñúgbrimaNticedaykMrityWt CagFmμta/ ]TahrN_ 2 - 5 ml¼KRk¼ em:ag. eKmantarag nig RkahiVk ¬emIlrUb 3a, 3b¦ edIm,ICYydl;buKÁlikeBTükñúgkar KNnabrimaNQamBitR)akdEdlGñk CMgWRtUvkar. rbbbBa©ÚlQam KYrmaneKalbMNgrkSa kMrit eGm:UkøÚb‘Ín rbs;GñkCMgWeGay enAcenøaH 9 - 10/5 Rkam¼d>l muneFVIkarBa©ÚlQam ehIyminRtUveGay elIs 15 Rkam¼d>l eRkaykarbBa©ÚlQam. eRkamkarkMritEbbenH GñkCMgW nwg)anTTYl ³ rIkxYrq¥wgkñúgkMritGb,brima lUtlas;FMFat;Fmμta nig famBlragÁkayekIneLIg BMumankarrIkGeNþIk b¤ BnüaeBl)anyUrmineGayrIkGeNþIk kat;bnßykMhab;Qam Edlvagaybgáplvi)akeGaysÞHsrésQam edaybNþúMRKab;Qam/ QWk,al nig QamRcmuH karelIsCatiEdkenAkñúgsrIragÁ)anfycuH KMlatbBa©ÚlQam GacecaTbBaðadl;ktþa epSg²Edr rYmman kargar b¤ karsikSarbs; GñkCMgW nig cMgaypøÚvEdlGñkCMgWRtUveFVIdMeNIr eTAmCÄmNÐlbBa©ÚlQam. Kit[Evgq¶ay eTAkarkMNt;eBlevlabBa©lÚ QamRtUverobcM [s½kþismnwgEbbbTrs;enArbs;GñkCMgWk¾dUc CaeqøIytbtamtMrUvTamTarEpñkevC¢saRsþ .

Amount of donor blood, depending on haematocrit, required to raise the patient’s haemoglobin by 1 g/dl

Guidelines for choosing how much blood to transfuse

vaytMélRbsiT§iPaBénrbbbBa©ÚlQam RbsiT§iPaBénkmμviFIbBa©ÚlQam eKEtgvas;EvgtamGRtaFøak;cuHkMrit eGm:UkøÚb‘Ín (Hb) EdlminelIs 1 Rkam¼d>l¼s)aþh_ cMeBaHGñkCMgWkat; GeNþIkecj nig 1/5 Rkam¼d>l¼s)aþh_ cMeBaHGñkCMgWmin)ankat;GeNþIk. ebIeKeXIjfakMrit eGm:UkøÚb‘Ín Føak;cuHkñúgGRtaelOn/ eKGacRsavRCavnUv buBVehtudUcteTA ³ manbegáItGgÁbdibkçRbqaMgnwgeKalikaQamRkhmEdlbBa©Úl (Alloimmunisation) ¬sUmGan RbtikmμTak;TgnwgkarbBa©ÚlQam¦. GeNþIkrIkFM (Hypersplenism) nig¼b¤ eføImrIkFM (Hepatomegaly). ]TahrN_ krNIGñkCMgWRtUvkareKalikaQamRkhmeRcInCag 200 ml¼KRk¼qñaM/ RtUvEtRsavRCavrklT§PaBrIkFMénGeNþIk. KuNPaBQamminsUvl¥ )anesckþIfa eKalikaQamRkhmmanCIvit xøI ehIybMeBjmuxgarminsUvmanRbsiT§iPaB. mankarhUrQam ¬]TahrN_ enAeBaHevon¦. karbMpøajeKalikaQamRkhmmankarekIneLIg edaysarkar eRbIfñaM ¬]TahrN_ Ribavirin¦. karbMpøajeKalikaQamRkhmmankarekIneLIg edaysarkar bgáeraK ¬]TahrN_ CMgWRKuncaj;¦. ykl¥eKRtUvvas;kMrit eGm:UkøÚb‘Ín mun nig eRkaykarbBa©ÚlQam edIm,IeFVIkar vaytMélrbbkarBüa)al. ebIsineFVImin)aneT eKKYrEtvas;kMrit eGm:UkøÚb‘Ín tamlT§PaBeFVI)anmYydgkñúgmYys)aþh_/ ral; 15éf¶mþg b¤ eBlNaGñkCMgW TTYlkarbBa©ÚlQam.

RbtikmμTak;TgnwgkarbBa©ÚlQam karbBa©ÚlQamGacbgá[manRbtikmμ EdleyIgmincg;)an b¤ plvi)akcMeBaH GñkCMgW eKehAfa RbtikmμénkarbBa©ÚlQam. RbtikmμénkarbBa©ÚlQamCa RBwtþikarN_eFVITukçEdlekItkñúgxøÜnGñkCMgWGMLúgeBl b¤eRkaykarbBa©ÚlQam. RbmaN 4° én karbBa©ÚlQam ¬ehIyebIQammanKuNPaBminl¥/ vaGac maneRcInCagenH¦ bgáeGaymanTMrg;xøHnUvRbtikmμ b¤ RbtiskmμEdleyIgmin cg;)an. karbBa©lÚ QamCakarbBa©lÚ nUvsarFatuxageRkA ¬QamGñkpþl¦; cUlkñúg xøÜnGñkCMgW. dUcenHvaCakarsmehtupl EdlRtUvEtrMBwgTukfamanRbtikmμ ekItecjBIRbB½n§karBarragÁkayrbs;GñkCMgW dUcKñanwgRbtikmμEdleKrMBwgTuk eBlbNþúHsrIragÁ b¤ bNþúHxYrq¥wgEdr. tamBiteTA ral;nitiviFIevC¢saRsþman plRbeyaCn_ k¾dUcCamaneRKaHfñak;xøaMgkøacMeBaHGñkCMgW EdlbuKÁlikeBTü RtUveFVIkarvaytMéledayykcitþTukdak;. RbtikmμEdlGacekItmancMeBaHGñkCMgW EdlTTYlkarbBa©ÚlQam EckCaBIr RbePT ³ k> RbtikmμénkarbBa©ÚlQamedayTak;TgnwgRbB½n§karBarragÁkay CakrNIRbB½n§karBarragÁkayrbs;GñkCMgWeqøIytbeTAnwgQam EdlbBa©ÚleTA. x> RbtikmμénkarbBa©ÚlQamedayminTak;TgnwgRbB½n§karBarragÁkay CaRbtikmμEdlminEmnekItecjBIRbB½n§karBarragÁkayrbs;GñkCMgW . RbtikmμTaMgBIrRbePTenH GacekIteLIgGMLúgeBl b¤ eRkaykarbBa©ÚlQam ¬RbtikmμRsYcRsav¦ ekIteLIgbnÞab;BIbBa©ÚlQam)an 2 b¤ 3 ml/ GMLúg eBlbBa©Úl b¤ eRkaykarbBa©ÚlQam)anbBa©b; b¤ RbtikmμmansPaByWt ekIteLIgeRcInéf¶ b¤ eRcIns)aþh_eRkaykarbBa©ÚlQam b¤ k¾yUrCagenH. taragxageRkamenH ¬1/ 2/ 3¦bBa¢ak;BIRbtikmμRbePT ¬k¦ nig ¬x¦ Edl GacekItmaneLIg ehIynigPaBjwkjb;rbs;va ¬tarag 3c, 3d, 3e¦. 40

¬kñúgEpñkenHmankareRbIR)as;Bakü bec©keTsevC¢saRsþeRcIn EdlnwgmankarlM)aksMrab;GñkGan fñak;mFümEdlcaM)ac;RtUvemIlniymn½yBaküenAkñúgsTÞanuRkm/ sgÆwmfakarCUnniymn½yBakü TaMgenHeFVI[GñkCMgWnigmatabitamanCMenOcitþxøaMgeLIgkñúgkareFVITMnak;TMngCamYybuKÁlikeBTü¦ 3c

taragelx 1 ³ RbtikmμénkarbBa©ÚlQamedayTak;TgnwgRbB½n§karBarragÁkay

Acute

Frequency

Delayed

Frequency

¬RsYcRsav¦

¬PaBjwkjab;¦

¬BnüaeBl¦

¬PaBjwkjab;¦

Hemolytic (intravascular)

1¼25>000

Alloimmune

1¼100

1¼50>000

Hemolytic (extravascular)

1¼2>500

[EbkeKalikaQamRkhm ¬kñúgsrésQam¦] Anaphylactic

[EbkRKab;QamRkhm ¬eRkAsrésQam¦] Febrile non-hemolytic

1¼100

Platelet refractoriness

1¼10

1¼100

Graft vs Host Disease (GVHD)

rare

¬TMnas;bnSaMsrIragÁ¦

¬kMr¦

¬ekþAxøÜn edayKμanEbkeKalikaQamRkhm¦ Allergy (Urticarial)

Transfusion Related Acute Lung

1¼1>000

Injury (TRALI)

¬rbYssYtRsYcRsavbNþalmkBIkarbBa©ÚlQam¦

taragelx 2 ³ RbtikmμénkarbBa©ÚlQamedayminTak;TgnwgRbB½n§karBarragkay

Acute

Frequency

Delayed

Frequency

¬RsYcRsav¦

¬PaBjwkjab;¦

¬BnüaeBl¦

¬PaBjwkjab;¦

Metabolic iron overload

Long term

¬elIsCatiEdkkñúgemtabUlIs¦

¬ry³eBlyUr¦

Hemolytic (in vitro) [EbkeKalikaQamRkhm ¬kñúmnÞIrBiesaFn_¦] Metabolic :

Infectious :

¬Tak;TgnwgkarbgáeraK¦ Coagulopathy

¬CMgWvibtþikMNkQam¦ ¬sItuNðPaBcycuH¦ Citrate toxicity ¬BulCatisuIRtat¦ Hypocalcaemia ¬elIskMritCatikal;süÚm¦ Hyperkalaemia ¬elIskMritCatib:UtasüÚm¦ Hypothermia

kMr kMr kMr kMr kMr

1. Bacterial

¬)ak;etrI¦

Gram negative Gram positive 2. Viral*

¬vIrus¦

HBV HCV HIV 1&2 HTLV 1&2 CMV E-B B19 3. Prions :Creutzfeld Jakob 4. Parasitic** ¬]> CMgWRKuncaj;¦

Embolic

¬sÞHsrésQamedayvtßúGVImYy¦

Circulatory overload

1¼15>000 1¼2>000 1¼15>000 1¼60>000 1¼100>000 1¼700>000 1¼650>000 7¼100 1¼200 kMr ?

kMr

kMr 1¼10>000

¬elIscMNuHQamkñúgRbB½n§crnþQam¦

PaBjwkjyénkarcMlgvIrus ERbRbYlxøaMgeTAtambNþaRbeTsnana GaRs½yelIKuNPaBén suxPaBsaFarN³ ehIynigesvabBa©ÚlQam nig eRbva:Lg;énPñak;garbgáCMgWTaMgenHtamtMbn;. PaBjwkjyEdlbgðajkñúgtaragenH KWyktamr)aykarN_TUeTA enAGWr:ubkñúgb:unμanqñaM cugeRkayenH. ** PaBjwjyénkarcMlg)a:ra:sIut vamanCaTUeTAenAbNþaRbeTskMBugGPivDÆn_. taragelx 3 bgðajBIbuBVehtuénRbtikmμmYycMnYn Tak;TgnwgkarbBa©ÚlQam/ dMNak;kalEdl RbtikmμekItmanjwkjab;bMput nig eraKsBaØaP¢ab;CamYy. *

taragelx 3 ³ buBVehtu/ eBlkekItRbtikmμ nig eraKsBaØaRbtikmμEdl Tak;TgnwgkarbBa©ÚlQam Type of reaction

¬RbePTRbtikmμ¦ EbkeKalikaQamRkhmPøam²

Timing

¬eBlkekIt¦ bnÞab;BIbBa©ÚlQam)an 2 - 3 ml

Cause

¬buBVehtu¦ RkumQam ABO minRtUvKña

Anaphylactic

“

kgVH

Air embolism

“

xül;cUlkñúgRbB½n§srésQam

IgA

BIkMeNIt

Symptoms

¬eraKsBaØa¦ ht;/ ENnRTUg/ ekþA/ jak;/ QWcegáH/ sMBaFQamFøak;cuH/ Shock/exSaytMrgenam RkhmEs,k/ knÞalRtGak rmas;/ht;/ k¥Üt/raK/ QWRTUg/ sMBaFQameLIg/ )at;sμartI/ Shock k¥k/ ht;/ QWRTUg nig Shock

¬dMuxül;kñúgsésQamsYt¦ Sepsis

¬qøgemeraK)ak;etrIeTAkñúgQam¦ elIscMNuHkñúgRbB½n§Qam TRALI (Transfusion Related

enAcugbBa©b; b¤ eRkay karbBa©ÚlQam)ancb; “

“

Acute Lung Injury)

¬rbYssYtRsYcRsavbNþalmk BIkarbBa©ÚlQam¦ FNHTR (Febrile Non-

“

Haemolytic Transfusion Reaction)

¬ekþAxøÜn EtminmanEbkeKalika QamRkhm¦

Allergic (Urticaria)

¬knÞalRtGak¦

“

)ak;etrIqøgtamQamEdlbBa©Úl karbBa©ÚlQamkñúgel,Ón elOneBk RbtikmμrvagGgÁbdibkç eKalikaQams énQambBa©Úl nig eKalikaQam>s rbs;GñkCMgW RbtikmμrvageKalikaQam>s énQam bBa©Úl nig GgÁbdibkç eKalikaQams rbs;GñkCMgW. RbtikmμxøHeKeCOfa bNþalmkBI sarFatu cytokine pliteday eKalikaQamskñúgeBlEdl QamrkSaTukmunykmkeRbI. BNalmkBIsarFatuTas; (Allergens) kñúgQambBa©Úl RbtikmμCamYyGgÁbdibkç rbs;GñkCMgW b¤ GgÁbdibkç énQambBa©Úl RbtikmμCamYy srFatuTas;rbs;GñkCMgW

ekþA/ jak;/ k¥Üt/ raK/ sMBaFQamFøak;cuH/ exSaytMrgenam/ Shock/ DIC ht;/ esøksøaMg/ sMBaFQam)aj;ecj BIebHdUg ekIneLIg ht;/ esøksøaMg/ k¥k/ sMBaFQamFøak;cuH

sItuNðPaBGñkCMgWekIneLIj 1oC b¤ elIs edayKμankarBnül;NamYy)an.

knÞalRtGak/ mancMNucRkhm tUc²elI Es,k/ ehImedaydMu²

taragelx 3 ³ buBVehtu/ eBlkekItRbtikmμ nig eraKsBaØaRbtikmμ EdlTak;TgnwgkarbBa©ÚlQam ¬bnþ¦

Type of reaction

Timing

¬RbePTRbtikmμ¦

¬eBlkekIt¦ 3 - 30éf¶

GVHD (Graft vs Host Disease)

¬TMnas;bnSaMsrIragÁ¦

Alloimmune reaction

¬RbtikmμedaymanbegáItGgÁ bdibkçRbqaMgnwgeKalika

Haemolytic delayed anamnestic response

¬EbkeKalikaQamRkhm eRkayBIkareqøIytbeLIgvij¦

Cause

¬buBVehtu¦ Antigens HLA rbs;GñkCMgWeTA eFVI[skmμ T-Lymphocyte énQambBa©Úl CaBiess cMeBaH ³ [i] GñkCMgWEdlmanRbB½n§ karBar cuHexSay [ii] TarkkñúgépÞTTYlkar bBa©ÚlQam [iii] TarkekItmkTTYl karpøas;bþÚrQam [iv] GñkTTYl QamBIsac;jati . eRcInéf¶ b¤ s)aþh_eRkay GñkCMgWbegáItGgÁbdibkçRbqaMgnwg karbBa©ÚlQam eKalikaQamRkhmxøH²EdlbBa©Úl bNþaleGayEbkRKab;QamRkhm EdlbBa©ÚleTA.

3 - 7 éf¶

Symptoms

¬eraKsBaØa¦ ekþA/ rlakeBaHevon/ raKsuT§EtTwk/ rlakEs,k/ Es,keLIgRkhm nig karfycuHRKb;eKalikaQam ¬Pansicytopenia¦.

ekþAxøÜnbnþicbnÞÜc/ jak;/ Es,keLIgelOg/ Hb fycuH minGacBnül;)an ¬eRKaHfñak;Qam RkhmEdlbBa©Úl¦ ekItEbbenH Gacfy)anedayqab;eFVIkar bBa©ÚlQam[)anmunGayu)an 3 qñaM¦.

dUcxagelIEdr EtGgÁbdibkçminGac dwg)anedaykareFVIetsþmnÞIBiesaFn_ munkarbBa©ÚlQam ehIyRbB½n§ kaBar ragÁkayplitnUvbrimaNd¾eRcInén GgÁbdibkçeBlbBa©ÚlQam.

karBüa)alRbtikmμbBa©ÚlQam buernimitþ ¬eCaKvasna¦ énRbtikmμbBa©ÚlQamGaRs½yelIPaBF¶n;F¶rénRbtikmμekIteLIg. teTAenHbgðajnUvrebobBüa)alRbtikmμmYycMnYn ³ 44

plvi)akF¶n;F¶r ³ RbtikmμbBa©ÚlQam eFVI[EbkRKab;QamRsYcRsav ¬Acute Haemolytic Transfusion Reaction¦/ RbtikmμbNþaleGay Shock ¬Anaphylactic¦/ qøgemeraK)ak;etrIeTAkñgú Qam ¬Sepsis¦ nig dMxu ül;sHÞ kñgú sésQam ¬Air embolism¦ ³ eKRtUvbBaÄb;karbBa©ÚlQam. eKGacbBa©ÚlCatiTwktam srésQam ehIyeRbIfñaMmYycMnYnedIm,IBüa)al b¤ karBarsßanPaBmYycMnYn kMu[ekIteLIg dUcCa CMgWkMNkQamkñúgsrésQamRKb;TIkEnøg ¬Dissemination Intravascular Coagulopathy = DIC¦/ exSaytMrg enam nig Shock . RbB½n§srésQamelIsbnÞúk GacBüa)aledayGukSIEhSn ¬O ¦nig fñaMbBa©úHTwkenam. rbYssYtRsYcRsavbNþalmkBIkarbBa©ÚlQam ¬Transfusion Related Acute Lung Injuey = TRALI¦ GaceFVI[FUres,Iyvij)an edayeFVIkar KaMRTpøÚvdegðIm. \T§iBlénEbkeKalikaQamRkhmeRkayBIkareqøIytbeLIgvij nig RbtikmμedaymanbegáItGgÁbdibkçRbqaMgnwgeKalikaQamRkhm EdlbBa©Úl Gackat;bnßy)anedayeRbI Corticosteroids . TMnas;bnSaMsrIragÁ ¬GVHD¦ TamTarnUvkarBüa)alKaMRTd¾smRsb . karqøgvIrus RtUvBüa)altamRbePTvIrusEdlqøg . plvi)akminF¶n;F¶r ³ ekþAxøÜn EtminmanEbkeKalikaQamRkhm eKRKan;EteRbIfñaMbBa©úHkMedA. knÞalRtGak/ cMNucRkhmtUc²elIEs,kehIyrmas; Gackat;bnßy )anedayfñaM Antihistamines.

karBarkMu[ekItmanRbtikmμbBa©ÚlQam esvabBa©ÚlQam nig EpñkCab;Bak;B½n§nwgkarbBa©ÚlQam RtUvGnuvtþn_ eKalneya)aybBa©ÚlQam[)anhμt;ct; edayFanafa nitiviFIénkarbBa©Úl Qamd¾l¥RtUv)aneKGnuvtþþn_ ehIysuxPaB nig suvtßiPaBGñkCMgWRtUv)anFana. buKÁliksuxaPi)alRtUvRbkan;x¢ab;nUvsþg;da nig BiFIsaEdl)anbegáIteLIg. karkMNt;GtþsBaØaNGñkCMgWxus ¬dUcCaxusRkumQam¦ mkTl;eBlenH vaCakMhusEdlmaneRcInbMputEdlbNþal[søab;enAkñúgkarbBa©ÚlQam. 1> sMNakRbePTQam nig karBiesaFn_rkPaBsIuKñaénQam ¬Compatibility testing¦ RtUvEtkMNt;GtþsBaØaNeGayc,as;las;. eQμaHeBj nig éf¶ExqñaMkMeNItGñkCMgW RtUvsresrbBa¢ak;elIsøakbMBg;dak;Qam munnwg bUmQam ehIyTinñn½ybMeBjelIRkdassñamsMrab;karbBa©ÚlQam ¬Transfusion Application Form¦ RtUvEtBinitü. 2> mRnþIsuxaPi)al RtUvEtepÞogpÞat;fa ral;]bkrN_sMrab;bBa©ÚlQam RtUv)an kMBugeRbIeTAtamkarENnaMrbs;plitkrén]bkrN_enaH. 3> munnwgfg;QamNamYyP¢ab;nwg]bkrN_bBa©ÚlQam mRnþIsuxaPi)alRtUv eFVIkarRtYtBinitüpÞal;nwgEPñknUvfg;QamrkemIlPsþútagénkarcMlgeraK ]TahrN_karERbRbYlBN’QameTACaRsKaMRkem:A/ mankMNk b¤ k¾Ebk eKalikaQam ehIyepÞógpÞat;emIlfa QamBitCaminTan;hYs kalkMNt;eRbI . 4> PaBsIuKñarvagQamGñkCMgW nig Qamkñúgfg;RtUvEtepÞógpÞat; edayBinitü elIlixitbBa¢ak;RkumQamGñkCMgW nig RkumQamEdlbgðajelIsøakCab; nwgfg;Qam. 5> GtþsBaØaNlMGiténQamkñúgfg;Edl)anbBa©ÚlRtUvcuHenAkñúgÉksar GñkCMgW edIm,IfaebImankarcaM)ac; eKGacnwgdwgGñkeFVIGMeNayQam. 46

6> eKRtUvBinitüemIlGñkCMgW eBjGMLúgeBlkMBugbBa©ÚlQam CaBiess dMNak;kaldMbUgEdlRbtikmμbBa©ÚlQamPaKeRcInGacnwgekIteLIg. 7>> edIm,IeCosvagkMu[b:HBal;dl;RbsiT§iPaBKøInik ehIymansuvtßiPaB nig manPaBgayRsYlkñúgkarbBa©Úl/ smasPaBQam ¬Blood components¦ RtUvbBa©ÚlkñúgefrevlakMNt; tamesckþIENnaM. 8> QamRtCak;bBa©ÚlelOnGacmaneRKaHfñak;. fg;QamkkeKRtUvEtkan;va edayRbugRby½tñbMput eRBaHQamkñúgfg;GacRsYy ehIyGacgayeRbH EbkenAsItuNðPaBTab. 9> RbsiT§iPaBénkarbBa©ÚlQam RtUvkMNt;edaykarkt;RtanUvtYelx eKalikaQammun nig eRkaykarbBa©ÚlQam . 10> RbtikmμNamYyEdleKsegáteXIj eKkt;RtaTukedayykcitþTukdak; ehIyraykarN_;. ral;plvi)akF¶n;F¶rTaMgGs; EdlekItmaneLIg RtUvEt esIubGegát ¬RtUvbUmQameRkaykarbBa©ÚlehIy rYcbBa¢ÚnvaCamYy plitplQamEdlminTan;)aneRbI ehIynigrbs;rbrbBa©ÚlQameTA mCÄmNÐlpþl;Qam edIm,IRsavRCavrkPaBminsIuKña ¬Serological Incompatibility¦ nig eFVIBiesaFn_bNþúH)ak;etrI. 11> kñúgkrNIRbtikmμbBa©ÚlQamekItmandEdl² RtUvEteFVIkaresIubGegát rkvtþmanGgÁbdibkçmineTogTat; ¬Irregular antibodies¦ eRkABIRbB½n§ ABO nig Rh. eBlEdl FNHTR ekItmanmþgehIymþgeTot eKRtUv eRbIQamEdlmaneKalikaQamstic. 12> sMxan;eKRtUvcgcaMfa plvi)akmYycMnYnekIteLIgmanlkçN³yWt dUcCa karcab;epþImCMgWEdlqøgtamkarbBa©ÚlQam. krNIGñkpþl;Qam eKrk eXIjfavIrusERbCaGviC¢manvij k¾GñkCMgWEdl)anTTYlQamGñkTaMgenaH RtUvEtTTYlkartamdanEpñkevC¢saRsþ . 47

mCÄmNÐlpþl;QamRtUvEtmanRbB½n§Fanara:b;rgKuNPaB EdlFananUvKuNPaB plitplQamEdleRbIR)as;. RbB½n§EbbenHRtUvFana[)anfa ³ 1> eKRtUvGnuvtþeTAtameKalkarN_ Rbtibtiþplitpll¥ ¬Good Manufacturing Practice¦ kñúgkareRCIserIsGñkpþl;Qam/ karbUmyk Qam/ plitQam/ eFVIetsþmnÞIrBiesaFn_/ sþúk nig Eckcayplitpl Qam. 2> RtUvkMNt;[)anc,as;nUvkic©kar nig karTTYlxusRtUvrbs;buKÁlikBak;B½n§ nwgskmμPaBTaMgenH. 3> RtUveRbIR)as;sMPar³[)anRtwmRtUv. 4> RtUv[manRbB½n§svNkmμépÞkñúg edIm,IgacvaytMél[)aneTogTat;nUvkar Gnuvtþn_ nig RbsiT§iPaBénkarRtYtBinitüelIKuNPaB. 5> kMhusqÁgRtUvraykarN_[)aneBjelj nig eronsURtemeron bTBiesaFn_. mnÞIeBTüCaeRcInenAGWr:ub nig enAshrdæGaemrik manKN³kmμaFikarbBa©Úl QamEdlrYmman tMNagénesvabBa©ÚlQam nig GgÁPaBKøIniksMxan;²Edl manskmμPaBbBa©ÚlQamxøaMgkøa. KN³kmμaFikarEbbenH GacelIkkMBs; EfmeTotnUvRbsiT§iPaBénkarGnuvtþn_karbBa©ÚlQameday ³ 1> eFVIkMNt;eKalneya)aybBa©ÚlQam eTAtamskmμPaBKøInikkñúgtMbn;. 2> eFVIkarvaytMél[)aneTogTat; elIkarGnuvtþn_karbBa©ÚlQam. 3> eFVIviPaKRBwtþikarN_minl¥ EdlCab;Bak;B½n§CamYykarbBa©ÚlQam ehIycat; viFankarN_EktMrUvCacaM)ac;.

CMBUkTI 4 elIsbnÞúkCatiEdk nig karkMcat;CatiEdkecj 4a

dUc)anBnül;kñúgEpñkxagedImrYcmkehIy/ taLasesmIubgá[CatiEdkkkkuj kñúgragÁkay. manpøÚvBIrEdlGñkCMgWtaLasesmIuRsUbCatiEdk KWBI cMNIGahar nig edaykarbBa©ÚlQam. RbsinebIkarelIsCatiEdk enHmin)ankMcat;ecjeT/ vaGacbgá[mankarxUcxatdl;srIragÁ sMxan;² dUcCa eføIm nig ebHdUg. GñkCMgWtaLasesmIu RtUveRbIfñaM BiessehAfa XILaT½r ¬Chelators¦ EdleTAcMraj;CatiEdkecj BIragÁkay. EdkenAkñúgsarBagÁkayGñkmansuxPaBl¥ mnusSeBjv½ymansuxPaBl¥ FmμtamanpÞkú CatiEdkRbmaN 4 Rkam kñúgenaH 3 Rkam eRbIR)as;sMrab;plit eGm:UkøÚb‘Ín (Hb) kñúgeKalika QamRkhm. dUcmanBnül;xagedIm eBleKalikaQamRkhm cas;/ søab;eTAvijehIy eGm:UkøÚb‘Ín rbs;vaRtUvEbkCa FatupSMpÁMú KW EGm nig køÚb‘Ín ¬Haem nig Globin¦. EdkEdlbeBa©jBI EGm RtUv)andwkCBa¢Únedaym:UelKulRbUetGIunmü:agehAfa Rtg;EsV‘rIn ¬Transferrin¦ edIm,IykeTAeRbIeLIgvijsMrab;plit EGm rbs;eKalikaQam Rkhmfμ²I eTot. sarFatuKmI EI dlbegátI RbUetGInu købÚ nÍ‘ eQμaHfa GamIuNUGasIud ¬Amino acid¦ RtUv)aneRbIeLIgvijedIm,IplitkøÚb‘Ín fμI²eTot. dMeNIrkarTaMg enHmann½yfa kñúgmnusSeBjv½ysuxPaBFmμtamñak; sarBagÁkayeRbIEdkeLIg vijesÞIrEtTaMgGs; edaybnSl;tictYcbMputsMrab;beBa©jecal. Caerogral;éf¶ EdkEt 1 mRk b:ueNÑaH RtUvbeBa©jecal PaKeRcIntamry³ Twkenam/ lamk/ tamEs,k ehIyebIRsþItamry³QamrdUv. EdkEdl)at;bg;enaH RtUvCMnYsmk vijedayRsUbtameBaHevonBIcMNIGahar ¬emIlrUb 4a¦. 49

EdkkñúgCMgWtaLasesmIu kñúgCMgWtaLasesmIukMritmFüm nig kMritF¶n; Edlmin)anbBa©ÚlQam sarBagÁ kayxitxMTUTat;cMeBaHPaBkgVHeKalikaQamRkhmF¶n;F¶r edaykarRsUbyk CatiEdkya:geRcInEfmeTotelIsBIFmμtaBIeBaHevon ¬2 - 5 Rkam¼qñaM eRbob eFobeTAnwg 0/0015 Rkam¼qñaM cMeBaHmnusSmansuxPaBl¥¦ edIm,IykeTA pliteKalikaQamRkhmEfmeTot. brimaNEdkRsUbbEnßmvaGaRs½yeTAelIPaBF¶n;F¶rrbs;kgVHeKalikaQam Rkhm. kgVHeKalikaQamRkhmkan;EtF¶n;F¶r xYrq¥wgk¾kan;EtrIkFM edayva xitxMeFVIya:gNapliteKalikaQamRkhm[)aneRcIn eBlenaHtMrUvkar CatiEdkk¾kan;EtFMeFg. ktþaepSg²k¾cUlrYmcMENkkñúgkarkMNt;brimaN EdkRsUbBIeBaHevonEdr. ]TahrN_ vItamInes CYybegáInkarRsUbCatiEdk rIÉ TwkEt nig FBaØCatimYycMnYnbnßykarRsUbCatiEdk. eTaHbIya:gNak¾eday pøÚvd¾sMxan;bMputkñúgkarbnßybrimaNEdkRsUbBIeBaHevon KWRtUvFanafa GñkCMgW mankMrit eGm:UkøÚb‘Ín l¥. dUecñHsMxan;KWfa GñkCMgWRtUvkarbBa©ÚlQam[)an eTogTat; edayrkSakMrit eGm:UkøÚb‘Ín [enAelI 9 Rkam¼d>l ¬vas;muneBl bBa©ÚlQam¦. GñkCMgWEdlbBa©ÚlQammin)anRKb; GacRsUbCatiEdkbEnßm BIeBaHevon 1 - 5 mRk¼éf¶ ¬b¤RbEhl 0/4 - 2 Rkam¼qñaM¦. eTaHbIya:gNak¾eday RbPBd¾sMxan;énkarelIsbnÞúkCatiEdkcMeBaHGñkCMgW TTYlkarbBa©ÚlQam KW tamry³QamEdlbBa©ÚlenHÉg. tamBiteTAbrimaN CatiEdkEdlTTYltamry³karbBa©ÚlQammankMritFMeFgCagCatiEdkEdl )anmkBIcMNIGahartamry³eBaHevon. dUcenHvaCakarsMxan;EdlfaGñkCMgW TTYlkarbBa©ÚlQamCaRbcaMRtUveRbIfñaMkMcat;CatiEdk KWCafñaMEdleTAP¢ab;nwg CatiEdk ehIybeBa©jvaecaleRkARbB½n§sarBagÁkay. eKalikaQamRkhm 1 ml manCatiEdk 1/16 mRk. mYyÉktaQam CamFümmanQameKalikaQamRkhmRbEhl 250 ml RtUvmanCatiEdk = 50

250 x 1/16 b¤enAcenøaH 200 - 290 mRk. CatiEdkEdlbeBa©jBI eKalikaQamRkhmTaMgenHEdlxUcxat KWCaRbPBd¾sMxan;énCatiEdkEdl kkpþúMenAkñúgsarBagÁkayénGñkCMgWEdlTTYlkarbBa©ÚlQameBjmYyCivit. ]TahrN_ GñkCMgWEdlTTYlQam 30 Ékta ¬30fg;¦ kñúgmYyqñaM nwgman CatiEdkelIsRbEhl 6 Rkam kñúgmYyqñaM ¬ 200 x 30 = 6000 mRk = 6 Rkam¦ b¤ RbEhl 16 - 16 mRk¼éf¶ . sarBagÁkayBMuGacmanlT§PaB beBa©jCatiEdkEdlelIskñúgbrimaNd¾eRcIndUcenHeT dUecñHvanwgkkBUn kñúgCalika nig srIragÁepSg². RbsinebICatiEdkenH minRtUv)anyk ecjedaymanGnþraKmn_BIfñaMeBTüeT vaGacCaeRKaHfñak;d¾xøaMg edaybgá Caplvi)akF¶n;F¶rbMputkñúgCMgW Ebtta taLasesmIukMritF¶n;. eraKsBaØaKøInikénkarelIsCatiEdk CaTUeTAekIteLIgenAGayuRbEhl 10qñaM eTaHbIeKeXIjmanPsþútagBI\T§iBlBulCatiEdkxøHcMeBaHeføImelIkumar GayuticCagenHk¾eday. rbYsenAnwgeføImbgáedayelIsCatiEdk KW sPaBcak;es£Hedaysrésevov ¬Fibrosis¦ edayvacab;epþImkñúgGMLúg 2qñaMénkarcab;epþImbBa©ÚlQam. rbYsF¶n;F¶r KW RkineføIm ¬Cirrhosis¦ GacekIteLIgmun Gayu 10qñaM RbsinebIKμankarBüa)albM)at;karelIsCati EdkeT CaBiesscMeBaHGñkCMgWmanrlakeføImRbePT eb nig¼b¤ RbePT es. CMgWebHdUg EdlCabuBVehtumYyjwkjab;CageK bNþal[søab;kñúgCMgW taLasesmIukMritF¶n; RtUveK)andwgfava ekIteLIgkMLúgeBl 10qñaM cab;taMgBIcab;epþImbBa©ÚlQam eTaH CaCMgWexSayebHdUgekItmanTal;EtkarbBa©ÚlQamry³eBl 15qñaM b¤ eRcInCagenHk¾eday. karelIsCatiEdk k¾CamUlehtusMxan;bMputpgEdr naMeGayyWtya:vdl;kar cas;TMuEpñkpøÚvePTcMeBaHGñkCMgWtaLasesmIu TaMgePTRbus nig ePTRsI edayva b:HBal;cMnYnRbEhlCaBak;kNþal. elIsBIenHeTAeTot karelIsbnÞúk CatiEdk Gacbgá[mankarlM)akcMeBaHRsþIkñúgkarmanépÞeBaH ¬manRbEhl

Increase of transfusional iron as needs for blood transfusions increase with age

(From Cohen and Schwartz Chelation therepy in chronic iron overload, 977, symposia specialists, Inc., Box 610397, Miami Florida)

Ferritin molecule

25° énkrNI¦ ehIyCamUlehtujwkjykñúgkarvivtþn_eGayekItCMgWTwkenam Ep¥meTAGnaKt. kñúgry³eBlyUreTA elIsCatiEdkbgá[manplvi)akelIq¥wg nig xUcxatdl; srIragÁsMxan;²epSgeTotdUcCa RkeBj Thyroid nig Parathyroid. dUecñH ehIyGñkCMgWRtUvTTYlkarBüa)albM)at;karelIsCatiEdk EdlnwgkkpþMúkñúg ragÁkay ehIyb:HBal;F¶n;F¶relIKuNPaB nig GayuCIvitrbs;GñkCMgW. etIkarelIsCatiEdkbMpøajragÁkayya:gdUcemþc eBlEdlCatiEdkkkpþúMkñúgragÁkay eTaHbNaþalmkBICMgWtaLasesmIu xøÜnvapÞal; b¤ mkBIkarbBa©ÚlQam b¤ k¾TaMgBIrkrNI/ RbUetGIunsMxan;EdldwknaM EdkkñúgQam KW Transferrin mansPaBEq¥tnwgCatiEdk ¬Saturated¦. edayKμan Transferrin NamYyenATMenr EdkesrIEdlKμanP¢ab;nwgRbUetGIun eFVIdMeNIrenAkñúgQamEdlCaeRKaHfñak;xøaMgcMeBaHsarBagÁkay. Edkk¾kkpþúM pgEdrkñúgCalikaedayP¢ab;nwgm:UelKulRbUetGIunsMrab;sþúk ehAfa Ferritin ¬emIlrUb 4c¦ nig Haemosiderin. EdkEdlsþúkkñúgRbUetGIunTaMgenH vaBMusUv eRKaHfñak;dUcEdksßitkñúgPaBCaesrIeT. eTaHya:gNak¾eday sarBagÁkay eyIgeFVIkarbMEbk Ferritin nig Haemosiderin Cab;rhUt EdlbnÞab;mkeFVI karbeBa©jEdkmYycMnYnsßitkñúgPaBesrI. CatiEdkGacnwgRtUv)anbeBa©jBI RbUetGIunsþúkEdr eBlNaGñkCMgWFøak;xøÜnQW. CatiEdkEdlenAsl;Edlmin)anP¢ab;nwg Transferrin edaysarKμan Transferrin sMrab;P¢ab; KWCaEdkEdlKμanPaBnwgnr )anesckþIfavaGacgay nwgTTYlyk b¤ pþac;ecalbnÞúkGviC¢manEdleKehAfa eGLicRtug ¬Electron¦. eBlEdkekIn eGLicRtug mYy/ vabþÚrBImanbnÞúkviC¢manbI ¬CaRbePTEdkEdleKsÁal;fa 3+ b¤ ferric iron¦ eTACaEdkmanbnÞúkviC¢manBIr ¬CaRbePTEdkEdleKsÁal;fa 2+ b¤ ferrous iron¦. eBlEdkpøas;BIsPaB 2+ eTA 3+ vabegáItnUvsarFatueRKaHfñak;mYy ehAfa ra:DIkal;esrI ¬Free

¦ EdlGacbgáeGayxUcxatFMeFgcMeBaHCalikasarBagÁkay. dMeNIrEdleKsÁal;c,as; edaybegáItCara:DIkal;esrI eKehAfa Rbtikmμ Fenton EdlCaRbtikmμKImImYy EdlsMraydUcxageRkamenH KW karbegáItCa ra:DIkal;GIuRduksIul ¬HO¦ ³ radicals

O2 + Fe3+ O2 + Fe2+ H2O2 + Fe2+ OH- + HO- + Fe3+

Desferrioxamine (DFO)

b¤ Desferal

edaysarBagÁkayeyIgBMumanmeFüa)ayRbsiT§iPaBNaedIm,IkMcat;Edkecj viFIEtmYyKt;edIm,IkMcat;CatiEdkelIsecj KW eRbIfñaM ehAfa XILaT½rEdk ¬Gñkcab;ykEdkecj¦ edaybegáItCasmasFatuCamYyEdk EdlGacbeBa©jecalBIsarBagÁkayeyIgtamry³Twkenam nig lamk . Desferrioxamine (DFO) KWCafñaMkMcat;CatiEdkTImYy EdlRtUv )aneKbegáIteLIgdMbUgenATsvtSn_qñaM 1960 ehIyRtUv)an beBa©jenAelITIpSarenAedImTsvtSn_qñaM 1970 sMrab;karBüa)alCMgW taLasesmIukMritF¶n;. DFO ¬emIlrUb 4d¦ eFVIkartamviFIBIirEbb ³ TI1dMeNIrRbRBwtþieTAmanPaBsnSwm² kñúgenaH DFO)anP¢ab;eTAnwgFatuEdkbegáIt )anCasarFatumYyehAfa Ferrioxamine ¬emIlrUb 4e¦ EdlbnÞab;mk)an beBa©jBIsarBagÁkay. tYnaTITI2rbs; DFO KWRtUvbnßysPaBBulrbs;Edk kñúgsarBagÁkayedaycab;CamYyra:DIkal;esrI. dMeNIrRbRBwtþiEbbenHman sPaBelOn. DFO )ancab;ykEdkBIRbPBBIrsMxan;kñúgsarBagÁkay. RbPBTI1 KWkar xUcxaténeKalikaQamRkhm RbPBenHmanrhUtdl; 70° énEdk Edl cab;ykeday DFO ehIybeBa©jeTAeRkAxøÜntamry³Twkenam. RbPBTI2 énEdk Edl DFO cab;yk KWEdkmkBIeføIm EdlCasrIragÁsþúkEdkFMbMputkñúg

Desferrioxamine B, the active substance of Desferal

Ferrioxamine B

sarBagÁkayeyIg. EdkEdlsþúkTukkñúgeføImRtUv)anbeBa©j kalNasarFatu BIr ¬Ferritin nig Haemosiderin¦ EbkkñúgekasikaeføIm ¬Hepatocyte¦. bnÞab;mk DFO kñúgekasikaeføIm P¢ab;CamYyEdkenaH muneBlRtUvbeBa©jeTA eRkAsarBagÁkaytamry³lamk. DFO mincab;ykEdkEdl)anP¢ab;CamYy Transferrin rYcehIyeT. m:UelKul DFO nImYy²P¢ab;CamYyGatUmEdkmYy edIm,IbegáIt)an Ferrioxamine . enH)anesckþIfa RbsinebI DFO manRbsiT§iPaB 100° enaH 1 mRk DFO nwgbeBa©jecalEdk)an 93 mRk. eTaHbIya:gNa vaminGacBitdUecñaH)aneT/ kMritRbsiT§iPaBBwgEp¥kelIkMritfñaM nig pøÚvEdleKeRbIfñaM/ brimaNsþúkénEdk/ kMritvItamIn es (Vitamin C) enAkñúgxøÜn nig kMritEdlGñkCMgWGnuvtþtamevC¢bBa¢aeRbIfñaMkMcat;CatiEdk. tYnaTIrbs;vItamInes ¬Vitamin C¦ vItamInes KWCaPñak;garkat;bnßymYy KwfavaGacCYy[EdkbþÚrBI Fe3+ eTAEdk Fe2+ . enHvasMxan;Nas;eRBaHEdk Fe2+ GaceFVIdMeNIr)an gayRsYlkñúgsarBagÁkayCagEdk Fe3+ ehIyEdk Fe2+ k¾CaTMrg;mYy EdlgayP¢ab;nwgXILaT½r dUcCa DFO CaedIm. edayehtuenHehIyEdk Fe2+EdlCaTMrg;Edk Ferrous rEmgeKehAfa EdkEdlGackMcat;ecj)an rIÉEdk Fe3+ EdlCaTMrg;Edk Ferric KWCaEdkKμanclna eKeXIjmanenA kEnøgsþúkkñúgsarBagÁkay maneføImCaedIm edayvaP¢ab;CamYyRbUetGIunsþúk dUcCa Ferritin nig Haemosiderin. viFImYyedIm,IbegáInEdkbeBa©jeday RbUetGIunTaMgenH KWkareRbI vItamInes bEnßmCamYyfñaM DFO. GñkCMnajpþl;dMbUnμanfa GñkCMgWtaLasesmIKYrEtelbfñaMvItamInes erogral;éf¶ ykl¥kñúgeBlEtmYyCamYyfñaM DFO. eTaHbIya:gNak¾eday CaTUeTAeKENnaMfa GñkCMgWcab;epþImelbfñaMvItamInesbEnßm EteBlNaeK)an TTYl DFO BIr b¤ bI s)aþh_ehIy. 54

kMritvItamInes eRbIRbcaMéf¶ EdleKENnaMenaHKW 50 mRk sMrab;ekμgGayutic Cag 10qñaM ehIy 100 mRk sMrab;ekμgFMCag 10qñaM. ehIykMriteRbIenH minRtUvelIs 200 mRk¼éf¶eT. GñkCMgWEdlmanrbbcMNIGahareTogTat; jaMuRkUc b¤ TwkRkUcRss; GacmincaM)ac;elbvItamInesbEnßmk¾)an. ]TahrN_ RkUcFMmYyEpømanpÞúkvItamInes 75 mRk ÉTwkRkUcRc)ac;Rss; 100 ml manpÞúkvItamInes 50 mRk. minxusBIfañ BM üa)al nig fñabM EnßmepSg²Edr/ kMrti eRbIRtUvéllkedayBiPakSa CamYyevC¢bNÐti . sMxan;Nas;RtUvcMNaMfa vItamIneseRcIneBkGacmanplBul elIGkñ CMgW edayvaeFV[I EdkCaeRcInmanclna. dUecñH vItamInesbEnßmGacman pleRKaHfñak; CaBiesscMeBaHGñkCMgEW dlmin)anTTYlfñaM DFO edayEdkman clnaeLIgedaysarvItamInes CaEdkesrIehIyvabgáeRKaHfñak;dl;Calika. etIeKcab;epþImBüa)alkMcat;CatiEdkeBlNa GñkCMgWtaLasesmIukMritF¶n; RtUvcab;epþImBüa)aleday DFO EteBlNaeK )anTTYlkarbBa©ÚlQamya:geTogTat;. DFO minsMrab;edaHRsaybBaða TaMgLayEdlbNþalmkBIkarbBa©ÚlQamminRKb; dUcCa kgVHRKab;Qam Rkhm/ karERbRbYlq¥wg/ rIkGeNþIk nig rIkeføImeLIy. eKminRtUvpþl; DFO dl;GñkCMgWEdlmin)anbBa©ÚlQam b¤ bBa©ÚlQambnþicbnþÜc luHRtamanGayu elIs 10qñaM b¤ manPsþútagénkarelIsCatiEdk. CaRkitüRkmTYeTA GñkCMgWRtUvcab;epþImBüa)aledayfñaMkMcat;CatiEdkeBlNa eK)anbBa©ÚlQam 10 - 20dg b¤eBlkMrit Ferritin eLIgelIs 1000 μg/l. reboblayfñaM/ karrkSaTuk nig kareRbIR)as; Desferrioxamine Gs;ry³eBlCag 30qñaM énbTBiesaFn_KøIniksMrab;RKUeBTü nig GñkviTüasaRsþ edIm,I)ankarRBmeRBogKñaelImeFüa)ayl¥bMputkñúgkareRbI DFO edIm,IkMcat; CatiEdkBIsarBagÁkay[manRbsiT§iPaB ehIyeCosvag)annUvplBulNamYy bNþalmkBIkareRbIfñaMeRcInhYseBk. 55

edaysarm:UelKul DFO manTMhMFM/ sarBagÁkayRsUbyk DFO )anbnþic bnþÜctamry³eBaHevon. dUecñHpøÚvl¥bMputkñúgkareRbI DFO KWcak;bBa©Últam eRkamEs,k b¤ cak;bBa©ÚltamsrésEv:n. TnÞwmnwgenHEdr eday DFO beBa©jeTAvijBIQammanlkçN³elOn [Bak;kNþalCIvit ¬Half-life¦ rbs;va 10 - 15naTI ] fñaMRtUvEtbBa©ÚlyWt²kñúgry³eBlmYyedIm,ITTYl)annUv kMhab;efrmYyenAkñúgQamkñúgry³eBlEvgtamEdlGaceFVIeTA)an. eKeRbI DFO tamry³sñb;BiessmYy Edlcak;bBa©ÚlfñaMcUleRkamEs,kyWt² ry³eBl 8 - 12ema:g ya:gehacNas; 6éf¶ kñúgmYys)aþh_. kMriteRbIBit R)akdsMrab;GñkCMgWnImYy² KWKNnaelImUldæanénGayu bnÞúkCatiEdkkñúg sarBagÁkay nig sPaBKøInik. CaTUeTAkMriteRbICamFümsMrab;kUnekμgmin[ elIs 20 - 40 mRk¼KRk énTMgn;xøÜn edaysarkMriteRbIx<s; GacBnüWtkarrIk FMFat;rbs;kumar. kMriteRbICasþg;da sMrab;mnusSeBjv½y KW 30 - 50 mRk¼KRk énTMgn;xøÜn. reboblayfñaM DFO DFO Caem:SAs¶ÜtBN’s manCaÉkta 500 mRk b¤ 2 Rkam. ÉktanImYy² manP¢ab;mkCamYydbEkvtUcmYymanemS:ABN’s EdleKRtUvlay[eTACa l,ay 10° muneRbI. ]TahrN_edIm,I)anl,ay 10° énfñaM DFO BIdbfñaM cMNuH 500 mRk eKRtUvbEnßmTwklay 5 m>l. TwklayeKGacrkTij)anBI »sfsßannana. bnÞab;mkeKTuk[emS:Avarlaykñúgry³eBl 2 - 3naTI. sMxan;eKminRkLúkdbfñaMeT edIm,ICYy[fñaMrlayeKGacRKan;EtbgVilRbemol dbfñaMkñúg)atéd b¤ elIépÞturabesμI. eBlemS:AfñaMrlayGs;ehIy eKyksIuraMg bUmehIydak;cUlkñúgsñb;cak;edIredayfμBil sñb;sgßt;elIsIuraMgyWt² rujfñaMcUl eTAkñúgsarBagÁkaykñúgry³eBlkMNt;mYy KWcenøaHBI 8 - 12 ema:g. sIuraMg nwgcak;Gs;fñaMeTAtamcMnYnema:gEdleyIgkMNt;. sñb;mYycMnYnEdleKeRbI jwkjab;CageKbMput mansMelg[sBaØaRbsinsIuraMgsÞH b¤ Gs;fñaM. 56

karrkSafñaM DFO l,ayfñaM DFO EdleK)anlayrYc GacsþúkkúñgbnÞb;mansItuNðPaB 23ºC kñúgry³eBlCaGtibrima 24ema:g. ebIGakasFatuekþA caM)ac;RtUvrkSavaenA kEnøgRtCak; edayTukvakñúgTUrTwkkkenAsItuNðPaB 4ºC ebIeKminTan;eRbIva PøameT. l,ayfñaMEdlTukkñúgTUrTwkkk GacTuk)anrhUt 5éf¶. eTaHbI ya:gNak¾eday ebIl,ayfñaMenaHkøayCal¥k; nig manBN’minl¥ eKRtUve)aHva ecalPøam. ykl¥bMput muneRbIeKRtUvykl,ayfñaM DFO mkeRkATUrTwkkk BIrema:g edImIi[vamankMedAesμIkMedAsarBagÁkay. sMrab;karcak;eRkamEs,k nig tamsrés eKGaclayTukmuneday»sfkarI kñúglkçxN½ÐmYys¥atKμan emeraK ¬Sterile condition¦ ehIy DFO layrYcGacrkSaTuk)anrhUtdl; BIrs)aþh_enAsItuNðPaB 4ºC b:uEnþ DFO layTukmunedayGñkCMgWxøÜnÉg ehIy minEmnCa»sfkarI l,ayfñaMminGacTuk[elIsBI 1 - 2 éf¶)aneT edayehtu favaGacqøgemeraK. karcak;l,ayqøgemeraK Gacbgá[manRbtikmμ. karcak; DFOeRkamEs,k edIm,Icak; DFO eRkamEs,k sIuraMgEdlpÞúkfñaM DFO RtUv[smlμmdak;cUl kñúgsñb;)an ehIybnÞab;mkPÞab;cUlkñúgbMBg;)aøsÞicesþIg EdltMrg;eTAmÞúl ya:gtUcmYy/ mÞúlenHehIyEdlcak;cUleTAeRkamEs,k. fñaM DFO RtUvcak; bBa©ÚleTAkñúgcenøaHEs,k nig sac;dMu b¤ k¾xøaj;EdlsßitenAeRkamEs,k. CaeRcInqñaMcugeRkayenH sñb;sMrab;cak;fñaMkan;EttUceTA²/ RsalCag nig s¶at; CagRbePTsñb;mun. enH)aneFVI[sñb;sMrab;cak;fñaMenH gayRsYleRbIkñúg lkç½Ns¶b;s¶at;kñúgeBléf¶ Gaclak;eRkamsMelokbMBak;)an. eTaHbIya:gNa k¾eday GñkCMgWCaeRcIncUlcitþeRbIsñb;cak;fñaMenHkñúgeBlyb; edIm,IkMu[rMxandl; skmμPaBkargareBléf¶rbs;eK. kareRbIfñaM DFO CaeTogTat; CacMENkd¾sMxan;bMputkñúgkarEfrkSaGñk CMgWtaLasesmIu[mansuxPaBl¥. b:uEnþkarBüa)alkMcat;CatiEdkeday DFO 57

CakarlM)ak KWfavaeFVI[QW/ eRbIR)as;eBlevlaeRcIn ehIylM)aksMrbsMrYl CIvitRbcaMéf¶. dUecñHsMxan;RKb;²rUbEdlcUlrYmEfTaMGñkCMgW rYmman evC¢bNÐit/ Kilanubdæak-yika/ «Bukmþay sMxan;bMputenaH KwGñkCMgWxøÜnÉgEtmþg rYmKñark meFüa)ayeFVIya:gNa[karBüa)alkan;Etgay tamEtGaceFVIeTA)an. CaBiesskumarFM² GacvaytMélx<s;cMeBaHkarxitxMeFVI[karBüa)alman lkçN³s¶at;es¶omeRcIneLIg. eKRtUvEtxitxMpgEdr edIm,Ibnßy[dl; Gb,brimanUvRbtikmμkEnøgcak; b¤ plvi)akepSg²eTotEdlCab;Tak;TgnwgfñaM DFO ¬GanenAxageRkay¦. meFüa)ayepSg²eTotkñúgkarcak;bBa©ÚlfñaM DFO eRkABIkareRbIfñaMtampøÚveRkamEs,k dUcerobrab;xagelI enAmanpøÚvmYycMnYn eTotEdleKGaceRbIfñaM)an EtpøÚvnImYy²manTaMgKuNsm,tþi nig KuNvibtþi. karcak;bBa©ÚltamsésQambnþCab; 24 em:ag viFIenHKWeRbI DFO cak;cUltamsrésQamEvn. bec©keTsenHGacseRgÁaH CIvitGñkCMgWEdlTTYlrgkarelIsCatiEdkF¶n;F¶r ehIymanplvi)akcMeBaH ebHdUgmkCamYy edaybnßykarBulmkBICatiEdkkñúgsarBagÁkayeday kMcat;CatiEdkecj)aneRcInCag nig elOnCagviFIepSg² kñúgeBleKkMBugcak; bBa©ÚlfñaMEtmþg. eTaHya:gNak¾eday karcak;bBa©ÚltamsrésCab;GacnaM mkCamYynUveRKaHfñak;F¶n;F¶rCagEdr CaBiesskarbgáeraK nig kMNkQam bgáedayExSbBa©ÚlQam Edlpþl;»kas[emeraKeRCotcUltamsrésEvn)an ¬sUmGanEpñkxageRkamsþIBIkareRbI DFO bBa©ÚltamsrésEvn Cab;rhUt¦ . dUecñHeKRKan;EteRbIviFIenHkñúgkrNIGñkCMgWbgðajnUv ³ [i] elIsCatiEdkmanlkçN³F¶n;F¶r/ )anesckþIfa kMrit Ferritin ekInCab;elIs BI 2500 mIRkURkam¼l ¬μg/l¦ nig¼b¤ kMhab;CatiEdkkñúgeføIm elIsBI 15 mIRkURkam¼l TMgn;eføImsuT§ EdlkMNt;edaykarRcwbsac;eføImBinitü. 58

plvi)akelIebHdUg bNþalmkBIelIsCatiEdk. [iii] GñkCMgWCaRsþImanKMeragfanwgmanKP’ ehIymankMrit Ferritin kñúgesr:Umx<s; nig¼b¤ kMhab;CatiEdkkñúgeføImx<s; ¬LIC = Liver Iron Concentration¦. [iv] karcak;bBa©ÚltamsrésEvnCab; GacRtUveFVIEdrcMeBaHGñkCMgW EdlcaM)ac; RtUvkarkMcat;ecjCatiEdk edayminKitBIkMrit Ferritin b¤ kMrit LIC . ]TahrN_ munkarbnSaMxYrqw¥wg b¤ GñkEdlmanrlakeføImRbePT es ¬C¦ skmμ ehIyrauMér:. karbBa©ÚltamsrésEvnCab;énfñaM DFO KWRtUv[kMrit 50 mRk¼KRk¼éf¶/ [ bnþCab;Etmþg 7 éf¶kñúgmYys)aþh_ tammeFüa)ayénRbB½n§bBa©ÚlfñaMBiess mYYyeKehAfa kaetETrP¢ab;eTAsésEvnFM ¬In-dwelling Catheter¦ . kaetETr RbePTenHmantMéléfø ehIyGñkCMgWEdleRbIvaTamTarnUvkartam danBIRKUeBTüedayykcitþTukdak;eGay)anditdl;. karbgáeraKF¶n;F¶r KWCa plvi)akjwkjab;bMputEdlekItmanCamYyviFIkMcat;CatiEdkEbbenH ehIyCYn kalmankMNkQameTotpg ¬Thrombosis¦. fñaMBiess ¬fñaMRbqaMgnwg kMNkQam¦ RtUv)aneKcak;bBa©ÚledIm,IkarBarkMu[mankMNkQam ehIy Rby½tñRbEygrkSaEs,kCMuvij kaetETr eGays¥atedIm,IkarBarkarbgáeraK. GñkCMgWRtUvEtrkSaEs,keGays¥at ehIymkBieRKaHCamYyRKUeBTüPøam ebIsin maneraKsBaØa jak; b¤ ekþA b¤ manTTYlGarmμN_faQW b¤ RkhmenAelIEs,k. karcak;bBa©ÚlfñaM DFO tamsésQam 8 - 12 em:ag pÞúyBIkarcak;bBa©ÚltamsrésQamCab;ry³eBl 24ema:g dUc)anBnül; xagelI/ karcak;bBa©Úl DFO tamsrésQam ry³eBl 8 - 12 ema:g CaCMerIsmYyepSgeTot kalNakarcak;eRkamEs,kmanbBaðaecaTF¶n;F¶r b¤ bBaðaecaTenAnwgkEnøgcak;. kMriteRbI nig ry³eBleRbIdUcKñanwgkarcak;bBa©Úl eRkamEs,kEdr KW 40 - 50 mRk¼KRk¼éf¶ ry³eBl 8 - 12 ema:g eRcInCag 5éf¶ kñúgmYys)aþh_. eTaHya:gNak¾eday viFIenHvaBMuman [ii]

RbsiT§iPaB dUckarcak;bBa©ÚltamsrésQamry³eBl 24ema:g Cab;enaHeT ebIkrNIelIsCatiEdkvaF¶n;F¶r ehIymanplvi)akcMeBaHebHdUgpgEdr. vamansarsMxan;Nas;edaycaMfa kareRbItamsrésQamnUvfñaM DFO RtUvman karRbugRby½tñ ehIyeRbIEtkrNIcaM)ac;. eRbIry³eBlEvg viFIenHGacbgá eRKaHfñak;dl;srésQamEvnEdlmansar³sMxan;sMrab;karcak;bBa©ÚlQam ehIymaneRKaHfñak;bgáeraKkMritx<s;. karcak;bBa©ÚlfñaM DFO tamsésQamGMLúgeBlbBa©ÚlQam enAmnÞIreBTümYycMnYn/ DFO RtUvcak;bBa©ÚltamsrésQamdMNaleBl CamYykarbBa©ÚlQam. eTaHya:gNak¾eday eKminRtUvykfñaM DFO eTAlaycUlpÞal;EtmþgkñúgQamEdlkMBugbBa©ÚleGayGñkCMgWenaHeT eRBaHva GaccMlgemeraKdl;Qam b¤ bgá[manRbtikmμ. CaTUeTA eKminRtUvbEnßm sarFatuNamYycUlkñúgQambBa©Úl[GñkCMgWeT elIkElgEt)anbgðajtam viTüasaRsþfa vaminbgá[maneRKaHfñak;NamYyenaHeT. edIm,IbBa©Úl DFO tamsrésQamGMLúgeBlbBa©ÚlQam/ brimaNfñaM DFO EdlRtUveRbI RtUvykmklayCamYyesr:UméRbFmμta ¬Normal saline¦. m¢úlrbs;fg;esr:UmEdlmanpÞúkfñaM DFO RtUvedatbBa©ÚlCaEbb “Y” CamYy fg;Qam ehIyRtUvbBa©Úl[GñkCMgWkñúgry³eBlRbEhl 4 ema:g. eKGaceRbI sñb;GMLúgeBlbBa©ÚlenHk¾)an RbsinebIeKcg;. CYnkal eBlevlaRtUvkar edIm,IbBa©ÚlfñaM DFO mYykMriteRbI[manRbsiT§iPaB manry³eBlyUrCag ry³eBleRbIsMrab;bBa©ÚlQam. sMxan;RtUvkMNt;cMNaMfa DFO EdleRbIEtkñúgGMLúgeBlbBa©ÚlQam/ )anesckþIfa 2 - 4 s)aþh_bBa©ÚlfñaM DFO mþg/ vamanRbsiT§iPaBtictYc Nas;. ya:gNak¾eday enHCaCMerIsEtmYyKt; kñúgkrNIfñaM DFO mankar xVHxat. müa:gvijeTot mnÞIreBTümYycMnYneRbIfñaM DFO bBa©Últamsrés QamGMLúgeBlbBa©ÚlQam[GñkCMgW CakarbEnßmeTAelIkarBüa)alRbcaMéf¶ 60

ya:geTogTat; kñúgkic©RbwgERbgedIm,IeFVI[)anRbesIrnUvRbsiT§iPaBTUeTAénkar Büa)alkMcat;CatiEdk. karcak;bBa©ÚlfñaM DFO tamsac;dMu eBlcab;epþImeRbIfñaM DFO dMbUgedIm,Ibüa)alCMgWtaLasesmIu Kwcak;vabBa©Úl tamsac;dMu. eTaHya:gNak¾eday eK)anrkeXIjfa viFIEbbenHBMusUvman RbsiT§iPaBdUckarcak;bBa©ÚleRkamEs,k b¤ tamsrésQameLIy. karcak;tamsac;dMu eKenAEteRbIkñúgkarNImYycMnYn ]TahrN_enARbeTsEdlfñaM DFO mantMéléføeBk ehIymancMnYntictYc b¤ krNIKμansñb;sMrab;cak;. manPsþútagkan;EteRcInbBa¢ak;fa GñkCMgW)anTTYlfñaM DFO xøH eTaHCatam viFINak¾eday k¾RbesIrCagkarmin)anTTYlfñaMesaH. dUecñHebIKμanCMerIseTenaH eKGaccak;fñaM DFO tamcak;dMuk¾)an. bec©keTsmYyeTot KW eKlayfñaM 500 mRk CamYyTwklayfñaM 5 ml ehIycak;sac;dMuéd b¤ sac;dMueCIg. kMriteRbIfñaM enHeKEckCaBIr ehIyeRbI 2dgkñúgmYyéf¶ enHCaviFImYyEdlGñkRsavRCav mYycMnYn)anbgðajnaeBlfμI²enHfa vamanRbsiT§iPaB. vaytMélRbsiT§iPaBénkarBüa)aledayfñaM DFO meFüa)aymYyénkarvaytMélRbsiT§iPaBénrbbkarBüa)alGñkCMgW KWkarvay tMéleTAelIbrimaNCatiEdkEdlsþúkkñúgeføIm nig kñúgCalikaepSg²eTot énsarBagÁkay. eføImmanlT§PaBGacpÞúkCatiEdk)aneRcIn )anCag 70° énCatiEdkEdlsþúkTukkñúgsarBagÁkay KWRbEhl 20 Rkam. CatiEdkEdl elIskñúgxøÜn vasþúkTukenAeføIm/ ebIeføImeBj KWvasþúkTukenACalikaepSg² eTot. EdkEdlsþúk KWP¢ab;CamYyRbUetGIun Ferritin nig Haemosiderin dUcKña nwgEdkEdlenAkñúgQamEdrEdlvaP¢ab;CamYyRbUetGIun Transferrin. man Ferritin tictYcdac;ecjBIeføImcUlkñúgcrnþQam karvas;kMrit Ferritin kñúg Qam KW eRbIedIm,IkMNt;bnÞúkCatiEdk. 61

kMritFmμtarbs; Ferritin kñúgQam ¬b¤ Serum ferritin¦ mandl; 250 mIRkURkam¼l ¬μg/l¦ sMrab;burs nig enAcenøaH 10 - 120 mIRkURkam¼l sMrab;RsþI. 1 mIRkURkam ¬1μg¦ én Ferritin kñúgQamcat;faesμInwgEdk 8 mIRkURkam sþúkenAkñúgxøÜn. GñkCMgWtaLasesmIukMritF¶n; EdlmankMritCatiEdl x<s;eRcInenAkñúgxøÜn eKrMBwgfamankMrit Ferritin x<s;ya:gsMebImenAkñúgQam. eTaHbIkMrit Ferritin GacCaGñkeGaykarN_mYyKYr[Tukcitþ)anénEdksþúkTuk kñúgeføIm EtvaminGac[eyIgTsSn_Tay)anc,as;las;BIbnÞúkCatiEdkenAkñúg srIragÁepSg²eTot dUcCaebHdUg b¤ bnÞúkCatiEdkkñúgsarBagÁkayTaMgmUl )aneLIy. elIsBIenH ktþaepSg²eTot dUcCa karrlak/ karbgáreraK edayvIrus b¤ )ak;etrI/ CMgWeføImraMér:/ CMgWrlaksnøak; nig kgVHvItamInes Gac b:HBal;elIkMrit Ferritin kñúgQam edaybBa¢ak;faCatiEdkmankMritx<s;Cag b¤ TabCag sPaBBitCak;Esþg. ya:gNak¾eday kMrit Ferritin kñúgQamRtUvcat;TukfaCakMNt;sMKal;Cak;Esþg bMputénplvi)akEdlGacekItmaneLIgedayCab;Tak;TgCatiEdk. ]TahrN_ karsikSamYycMnYn)anbgðajfa krNIkMrit Ferritin enAeRkam 2500 mIRkURkam¼l ¬μg/l¦ Cab;rhUtCaeRcInqñaM/ eRKaHfñak;cMeBaHGñkCMgW eGayman plvi)akelIebHdUgmantictYc. dUecñH eKalbMNgKW RtUvrkSa Ferritin [enA cenøaH 1000 - 2000 mIRkURkam¼l ehIyRtUvBinitükMrit Ferritin ya:gehac Nas; 3Exmþg kñúgeKalbMNg[dwgfa etIkarBüa)alkMcat;CatiEdkrbs; GñkCMgWRtUvEfmfy b¤ ya:gNa. vasMxan;pgEdr RtUvRtYtBinitülT§PaBBul edayfñaM DFO EdlGacekItmaneBleRbIvakñúgkMritx<s; nig eBlEdlkMrit Ferritin Føak;eRkam 1000 mIRkURkam¼l. eRbIkMrit Ferritin edIm,IkMNt;kMriteRbI DFO eKGaceRbIkMrit Ferritin edIm,IkMNt;kMriteRbIsmRsbrbs;fñaM DFO sMrab; GñkCMgW edayeRbIsmIkadUcxageRkam EdleKehAfa snÞsSn_Büa)al 62

. kMriteRbImFümRbcaMéf¶ ¬mRk¼KRk = mg/kg¦*

“Therapeutic Index” (TI)

TI =

kMrit Ferritin kñúgQam ¬m>Rk¼l = mg/l¦ eKaledA KW RtUvrkSatMél TI [enAeRkam 0/025 RKb;eBlevla. * kMriteRbImFümRbcaMéf¶én DFO KW KNnaedayKuN kMriteRbI EdleRbIkñúg karBüa)alnImYy² CamYynwgcMnYnsrubkMriteRbIkñúgmYys)aþh_ Ecknwg 7 cMnYnéf¶éns)aþh_. kñúgb:unμanqñaMcugeRkayenH eK)anbegáItetsþepSg²eTotmYycMnYnEdlGac vas;EvgEdkkñúgsarBagÁkayRbkbedayPaBKYrTukcitþ)an. CaerOy²eK GnuvtþetsþTaMgenH CamYykarvas;Evg Ferritin kñúgQam edIm,Ipþl;rUbPaBBit R)akdEfmeToténkarpÞúkCatiEdkkñúgragÁkay CaBiess eføIm nig ebHdUg . kMhab;EdkkñúgeføIm (Liver Iron Concentration = LIC) karvas;kMhab;CatiEdkkñúgeføIm Tak;Tgnwg karRcwbykCalikaeføIm ¬Liver biopsy¦ehIyvas;brimaNEdkkñúgsac;eføImEdlRcwbenaH. karRcwbykCalikaeføIm KWeFVIeLIgedays‘km¢úlBiesstameBaHcUleTAeføIm CaTUeTAeKeRbIfñaMs<wkRtg;kEnøgcak; ehIyeKcUlcitþeRbI eGkU ¬Ultrasound¦ CYynaMpøÚvykCalikaeføImmYyEpñktUc. karvaytMélbnÞúkCatiEdk)anRtwmRtUv mYyEpñkBwgEp¥kelIbrimaN ¬ya:gehacNas; 1 mRk TMgn;eføIms¶Üt¦ nig KuNPaB ¬)anesckþIfa KμanRkineføIm b¤ Kμan PaBcak;es£Hedaysrésevov¦ énCalikaeføImEdlykmkBinitü dUecñHsMxan; KWPaBsÞat;CMnajrbs;buKÁlik eBTüEdlGnuvtþkargarenH. eTaHCakarRcwbykCalikaeføIm manGRtapl vi)akTab eBlEdleyIgeFVIenAmnÞIreBTüman eGkU CYytMrg;Tisk¾eday k¾RtUv TamTarnUvkaryl;RBmBIGñkCMgW nig¼b¤ «BukmþayGñkCMgW¼GaNaBüa)alEdr. 63

SQUID (Liver iron measurement system)

karRsavRCav)anbBa¢ak;fa krNIkMritCatiEdkkñúgeføImrkSa[enAeRkam 7 mRk¼Rkam énsac;eføIms¶Üt)anCab;lab;enaH eRKaHfñak;énbBaðaecaT EpñkebHdUg b¤ eføIm mantictYc. ebIkMritelIs 15 mRk¼Rkam énsac;eføIms¶Üt enaHvaGacmaneRKaHfñak;x<s;énkarsøab;ebHdUg. eTaHbIya:gNa karRsavRCav enHkMBugbnþedIm,IeGaydwgfa etIkMritkMhab;EdkkñúgeføImdUcxagelIenH GacbBa¢ak;KYr[Tukcitþ)anGMBIeRKaHfñak;xøaMg b¤ tic cMeBaHebHdUg b¤ y:agNa. dUckñúgkrNIén Ferritin kñúgQamEdr bNþaGñkviTüasaRsþ)anecaTsYrfa etIbec©keTsenHqøúHbBa©aMgya:gRtwmRtUvnUvbnÞúkCatiEdksrubkñúgsarBagÁkay ehIyBiessCagenHeTotCatiEdkenAebHdUg b¤ ya:gNa. karRsavRCavfμI)an bgðajfa karvas;EvgKYr[TukcitþbMputénCatiEdkkñúgxøÜn KWTTYl)aneday KuNkMhab;EdkkñúgeføIm CamYyktþamYy KW 10/6 mRk¼KRk énTMgn;xøÜn. karRcwbykCalikaeføImGacpþl;Bt’manpgEdr cMeBaHr)ayCatiEdk rvagBYk ekasikaeføImdUcCa Hepatocytes nig ekasika Kuffer edaybBa¢ak;)anEdrBI karrlak b¤ karxUcxatcMeBaHeføIm dUcCaRkineføIm b¤ PaBcak;es£Heday srésevov. viFIfμI²EdleFVImineGayQWcab; kñúgkarvaytMél[)nRtwmRtUvnUvbnÞúkCatiEdk RtUv)aneKbegáItpgEdr dUcCa]bkrN_ SQUID (Superconducting Quantum Interface Device) EdleRbIKuNsm,tþiGmma:ej:Ticrbs;Edk ¬Paramagnetic properties¦ edIm,Ivas;kMhab;rbs;vakñúgsarBagÁkay. vaeRbI Ednma:ej:TicmYyEdlmankMlaMgRbEhlEdkqk;EdleKeRbIkñúgTUrTwkkk. munnwgeRbI]bkrN_enH GñkCMgWRtUvedkeGayRsYlelIERKry³eBl 2 - 3 naTI ehIyeKeRbI eGkU CYykMNt;TItaMgrbs;eføIm bnÞab;mkeKrujERKGñkCMgWcUleTA cMBIeRkam]bkrN_ SQUID. Epñkrbs;ma:sIun SQUID Edlb:HdgxøÜnGñkCMgW KWCa)aLúgmYyeBjedayTwkekþA]NÑ² EdleKdak;BIelIeBaHEpñkxagelIeday snSwm². kñúgeBlEdlma:sIunkMBugvas;EvgCatiEdkkñúgeføIm GñkCMgWRtUvGt; degðImry³eBl 2 - 3 dg/ kñúgmþg² 2 - 3 vinaTI. GñkCMgWGayueRkam 6qñaM

GacRtUvkareRbIfñaMguyedkedIm,I[vaenAes¶óm. GñkCMgWTMgn;ticCag 10 KRk b¤ GñkCMgWFat;duHeBaH eKKitfamin)anpll¥ sMrab;etsþenHeT. CaGkusl bec©keTsenHmantMéléfø TamTarnUvbec©kviTüax<s; ehIybc©úb,nñenHman eRbIEtenAmCÄmNÐlR)aMEtb:ueNÑaHenAGWr:ub nig enAGaemrikxageCIg. eTaHya:gNak¾eday cMeBaHGñkCMgWBIbNþaRbeTsnana ebIRKUeBTüBüa)alKitfa caM)ac; eKGaceFVIkarNat;TukmunedIm,I)anBinitüedayma:sIun SQUID enHEdr. viFIepSgmYyeTotedIm,Ivas;bnÞúkCatiEdkkñúgeføIm EdlkMBugEtRsavRCav enAeLIy KWeRbI MRI (Magnetic Resonance Imaging) EdlBwgEpñkpgEdr eTAelIKuNsm,tþiGmma:ej:Ticrbs;Edk . kMhab;EdkkñúgeføImdwg)aneday viFIenH ¬MRI, T2*¦ bgðajnUvTMnak;TMngsmehtupl nig Tukcitþ)an CamYy kMhab;CatiEdk)anBIkarviPaKKImIcMeBaHsac;eføImEdlRcwb. KuNRbeyaCn_d¾ manskþanuBlénbec©kviTüaenH KWfa MRI maneRbIeRcIn TUeTACag. eK)an bgðajfa MRI Ca]bkrN_ mansar³sMxan;kñúgkarvaytMélbnÞúkCatiEdkkñúg ebHdUgpgEdr. CatiEdkkñúgTwkenam viFIepSgmYyeTotEdlmanCayUrmkehIykñúgkarvaytMélbnÞúkCatiEdk KWvas; CatiEdkkñúgTwkenamkñúg 24ema:g edIm,IvaytMélRbsiT§iPaBénkarBüa)al kMcat;CatiEdkedayfñaM DFO. ya:gNak¾eday xøwmsarEpñkKøInikrbs;etsþenH vamankMNt; eRBaHvaERbRbYlBImYyéf¶eTAmYyéf¶ kñúgkarbeBa©jCatiEdkeTA kñúgTwkenamrbs;fñaM DFO. plvi)akcMeBaHkareRbIfñaM DFO ehIyvabgðajeLIgEbbNa RbtikmμEs,kenAkEnøgcak;fñaM RbtikmμenAkEnøgcak;jwkjab;CageKbMput KW rma:s;/ Rkhm/ ehIm/ e)a:g/ dMe)A/ QW nig karrMxanTUeTA. cMnucKnøwHdUcxageRkamGacCYybnßyRbtikmμ dUcxagelI)an ³ 65

eCosvagkars‘km¢úlEk,rsrésQam b¤ srésRbsaTsMxan;² edIm,Ibnßy CaGtibrimanUveRKaHénkarxUcxat nig¼b¤ karhUrQam. [ii] RtYtBinitüemIlfaetIfñaM DFO rlaykñúgbrimaNTwkRKb;RKan;Edr b¤ eT ¬Twklay 5 ml sMrab;fñaM DFO 500 mRk¦. ebIcaM)ac; eKbEnßmTwk layedIm,I[varavEfmeTot. [iii] RtUvbþÚrTIkEnøgcak;. eBaHrEmgCaTIkEnøgcak;d¾l¥bMput. GñkCMgWxøHcUlcitþ cak;edIméd b¤ ePøA. eTaHya:gNak¾eday RbtikmμenAkEnøgcak;vaekIteLIg mYyry³eBl eTaHTIkEnøgNak¾eday sMxan;RtUvdUrkEnøgcak;epSg²Kña. kareRbI DFO yUr²eTA vaGacbgá[mandMue)a:genACMuvijkEnøgcak;. karenH eKGackarBar)an edaybgVilCMuTIkEnøgcak; b¤k¾/ kñúgkrNIxøH minTaMgGs; eT/ edaybMeBjnUvsarFatuKImI Hyluronidase bnþiccUlkñúgbMBg;cak; )aøsÞic mundMeLIgsñb;cak;fñaM. [iv] eKGacbnßykarQWcab;edaylabERkmfñaMs<wkdUcCa Emla rvag 30 60naTI muncab;epþImkarBüa)aleday DFO. ehImGacbnßyehImeday s¥MtwkekþA]Nð²eRkayBIcak;fñaM DFO . sMrab;kareLIgRkhm/ rma:s;/ ehIm b¤ dMe)A/ evC¢bNÐitxøH eKeRbIERkm Heparin b¤ Fusicor. [v] el,Óncak;bBa©ÚlfñaMk¾RtUvRtYtBinitüpgEdr edaysarfakarehIm GacekIt maneLIgeBleKcak; DFO elOneBk. [vi] evC¢bNÐitGaceFVIkarsMerccitþpgEdr eGayBYk Antihistamine dl;GñkCMgW muncak;fñaM DFO b¤ kñúgkrNIF¶n;F¶r eKGacbEnßm Hydrocortisone 5 10 mRk eTAkñúgl,ayfñaM DFO. [vii] mansñb;sMrab;cak;fñaMbBa©ÚleRcInRbePTenATIpSar ehIyGñkCMgWGaceRCIs ykRbePTNamYymkeRbIsmnwgesckþIR)afñarbs;eK. sñb;cak;fñaMman )aLúg KWCaRbePTfμICageKbMput. vatUcCag/ RsalCag ehIys¶at;Cag RbePTcas;. vaeFVIeGaycMeNjeBlevlapgEdr ehIyGacfasmrmü [i]

CageKsMrab;GñkCMgW eRBaHl,ay DFO RtUv)anlayTukmuneday»sfkarI kñúglkçxN½ÐmYys¥atKμanemeraK. edaysarKuNsm,tþiTaMgGs;enHehIy sñb;cak;fñaMEbbenHGacCYyeFVI[RbesIreLIgnUvkarGuvtþn_rbs;GñkCMgW cMeBaHkarBüa)alkMcat;CatiEdk. eTaHya:gNak¾eday/ tMéléfø)aneFVI[ kareRbIR)as;sñb;cak;fñaMEbbenH minmanPaBTUlMTUlayeLIy. [viii]GñkCMgWGacsakl,gm¢úlepSg² edayBikSaBIkarEfm b¤ fynUvRbePT xus²KñaCamYyevC¢bNÐit b¤ Kilanub,dæak-yika nig GñkCMgWepSgeTot. manGñkCMgWCaeRcInniymm¢últUc ehIyRsalehAfa m¢úlemGMe)Aelx 25 b¤ tUcCagenH cak;eTAelIépÞEs,kenAmMu 45 GgSa. GñkCMgWepSgeTot cUlcitþm¢últUcEbbCa m¢úledatrUbPaBCab;CBa¢aMg ¬Thumbtack¦ EdleKcak;EkgeTAnwgEs,k ehIybiTCab;nwgEs,kedaybg;s¥itBiess . RbsinebIviFIdUc)aneBalxagelI BMu)anCYyGñkCMgWBIRbtikmμTak;TgfñaM DFO eT eKRtUvBicarNakarBüa)aledayfñaM DFO edaybBa©ÚltamsrésQam b¤ fñaMkMcat;CatiEdkepSgeTot. RbtikmμF¶n;F¶rCamYyfñaM DFO RbtikmμF¶n;F¶rCamYyfñaM DFO CakarkMr. eraKsBaØarYmman karTTYlGarmμN_ TUeTAminRsYlxøÜn/ hwugRtecok/ vilmux/ eLIgRkhmeBjxøÜn b¤ ehIm nig Bi)akdkdegðIm CYnkalrYmCamYyekþAxøÜn/ Qwsac;dMu. eKRtUvbBaÄb;fñaM DFO ebImaneraKsBaØadUcxagelIekIteLIg. RbtikmμTUeTATaMgGs;enH manninñakarekIteLIgPøam² cMeBaHGñkCMgWEdleTIb cab;epþImBüa)aleday DFO. cMENkÉGñkCMgWEdlTTYlkarBüa)aleTogTat; nwgsMKal;eXIjeraKsBaØaekIteLIgbnþicmþg². kñúgkrNINak¾eday kar Büa)alcMeBaHRbtikmμF¶n;F¶r CaviFImYyEdleKehAfa bnßyPaBqab;TTYl Rbtikmμ ¬Desensitisation¦ EdlkñúgenaHGñkCMgWRtUveKcak; DFO kñúgbrimaN tictYclayCamYy Hydrocortisone rYcbegáIn DFO bnþicmþg²rhUtdl; 67

brimaNeRcIn. karBüa)alenHRtUvGnuvtþeRkamkarRtYtBinitüBIRKUeBTü ya:gditdl; CaerOy² eKeFVImþgehIymþgeTot rhUtdl;)at;eraKsBaØa ehIy rEmgTTYl)aneCaKC½y. ya:gNak¾eday RbsinebIkarBüa)alenHmin)an eCaKC½y GñkCMgWKYrEtQb;eRbIfñaM DFO ehIyBicarNakareRbIfñaMkMcat;CatiEdk epSgeTot dUcCa Deferiprone ¬sUmGanenAEpñksþIBIkareRbI Deferiprone¦. plvi)akcMeBaHkMriteRbIfñaM DFO minRtwmRtUv plvi)akxageRkamenH sMxan;bNþalmkBIkareRbIfñaM DFO kñúgkMritx<s;elIGñk CMgWkumar nig GñkCMgWEdlmankMrit Ferritin kñúgQamTab ¬)anesckþIfaman elIsCatiEdkbnþicbnþÜc¦. bBaðaRtecok ¬BulcMeBaHRtecok¦ rYmmansMelgeralT×kñúgRtecok/ fycuHPaKxøHénkarsþab;B¤ CaBiess enAeBleRhVkg;x<s;. bBaðaEPñk ¬BulcMeBaHEPñk¦ rYmmanxVak;man;/ RBilEPñk/ kMritKMehIj fycuH ¬Visual acuity¦/ fycuHKMehIjBN’/ eLIg)ayEPñk/ nig karrMxan énEPñkepSg²eTot. CaTUeTAfñaM DFO bgá[manbBaðaRtecok nig EPñk kalNaeKeRbIvakñúgkMritx<s; kalNam:UelKul DFO eFVIdMeNIrkñúgQamedayKμan)anP¢ab;nwgEdk. ya:gNa k¾eday GñkCMgWTwkenamEp¥m b¤ GñkCMgWkMBugBüa)aledayfñaMrMgab;GarmμN_ ¬Psychotrope¦ GacmaneRKaHeGayekItbBaðaEbbenH eTaHCaeKTTYlkMritfñaM DFO RtwmRtUvk¾eday eRBaHfasßanPaBTaMgenHeFVI[brimaNeRcInénfñaM DFO eTAdl;RbB½n§srésRbsaTkNþal. GñkCMgWEdlekItbBaða Rtecok nig EPñk CaTUeTAeKENnaM[Qb;eRbI DFO mYyry³ sin ehIycab;epþmI karBüa)alvij kñgú kMrti eRbITabeBlplvi)ak )anFUres,Iy b¤ )at;eTAvij. RbsinebI)antamdanBIedImTI plvi)akEbbenHGacRKb;RKg)an ehIynwgFUrRsalmkvij. dUcenHsMxan;RtUvRtYtBinti üGñkCMgTW TYlfñaM DFO [)aneTogTat;

Difference parts of the brain have different jobs to do

kñúgenaHmankarBinitüevC¢saRsþ[)aneBjelj rYmCamYykarvas;lT§PaB sþab;lWrbs;Rtecok ¬Audimetric test¦ nig lT§PaBemIleXIjrbs;EPñk ¬Ophtalmologic test¦. karrIkFMFat;yWt nig karERbRbYlq¥wg ³ kMriteRbIfñaM DFO kMritx<s;elI GñkCMgWmankMrit Ferritin Tab GacBnüakarrIkFMFat;. ktþaeRKaHfñak;rYm man GayuekμgeBlcab;epþImBüa)al ¬ticCag 3 qñaM¦ ehIykMriteRbI DFO x<s;CagkarENnaMeGayeRbI ¬> 35 mg/kg cMeBaHkumartUc²¦. karbnßy kMriteRbImkvij GaceFVIeGaykarrIkFMFat;vilmkFmμtavij dUcmunkar Büa)aledayfñaM DFO. \T§Bi lfñaM DFO eTAelIkarrIkFMFat; rYmmandgxønÜ b¤ édxømI ni smamaRtKña rYmCa mYykarxUcxatq¥wg b¤ snøak;EdleKeFVIeraKvinicä½y)anedaykarftviTüúsaRsþ . ktþaepSg²eTot dUcCabnÞúkCatiEdkk¾Gacb:HBal;dl;karrIkFMFat; kñúgCMgW taLasesmIu Edr. ya:gNak¾eday vagayRsYlnwgdwg)anfaetIkaryWtya:v kñúgkarrIkFMFat; bNþalmkBIfñaM DFO eRbIkñúgkMritx<s; b¤ ya:gNa. ebIbNþal mkBIfñaM DFO GñkCMgWnwgmineqøIytbnwgkarBüa)aleday G½rm:UnlUtlas; ¬Growth Hormone¦ eT. GñkCMgWKYrTTYlkarRtYtBinitü[)aneTogTat; sMrab;karERbRbYldUecñH eRBaHfavaminFUres,IymkvijeTebIvaekItehIy. karbgáeraKedayemeraK Yersinia enterocolitica ³ karbgáeday )ak;etrI Yersinia enterocolitica k¾eXIjmanCaTUeTACamYykareRbIfñaM DFOEdr. ral;GtisuxumR)aNmanCIvitTaMgGs; RtUvkarFatuEdkedIm,IrIk FMFat; kñúgenaHman )ak;etrI/ )a:ra:sIut nig Pñak;garbgáCMgWepSgeTot. GtisuxumR)aNPaKeRcInmanrcnasm<½n§BiessEdlpþl;lT§PaB[vaTTYl ykEdk)an. ya:gNak¾eday Yersinia vasßitkñúgRkum)ak;etrImYyEdlBMu manmeFüa)aypÞal;xøÜnedIm,ITTYl)anEdk dUeqñHvaBwgEp¥kelI receptor rbs; ferrioxamine EdlCasmasFatuekIteLIgeRkayBIEdkP¢ab;CamYy fñaM DFO. DFO CaPñak;gardwkCBa¢ÚnFmμCatimYyrbs;Edk EdleKehAfa

¬mkBIBaküRkik “Sidero” )ann½yfa Edk nig “Phoro” )ann½yfa dwkCBa¢Ún¦ dUcenHCalT§pl DFO KaMRT[mankarrIkFMFat; én)ak;etrI Yersinia edaypþl;[)ak;etrIenH nUvCatiEdk. karbgáeraK eday Yersinia GacmanlkçN³F¶n;F¶rxøaMgcMeBaHGñkCMgWelIsCatiEdk ehIyeRKaHfñak;enHkan;EtFMeFg eBleKkMBugEteRbIfñaM DFO. dUecñHsMxan; eKRtUveFVIeraKvinicä½ykarbgáeraKeday Yersinia [)anelOn ehIy Büa)alvaedayfñaMpSH[)ansmRsb ¬sUmGanenACMBUksþIBIkarbgáeraK¦. eraKsBaØaénkarbgáeraKrYmman QweBaH/ raK/ QWsnøak;/ ekþAxøÜn/ QWbMBg;k. ebImanekIteraKsBaØadUcenH eKRtUvbBaÄb;fñaM DFO rhUtdl; )at;eraKsBaØa ehIyvKÁeBjénkareRbIfñaMpSH)anbBa©b;. mankarbgáeraK F¶n;F¶r Edlbgáeday)ak;etrIepSg²eTot mandUcCa Klebsiella b¤ BYkpSit ¬Mucormycosis¦ GacBak;B½n§EdrnwgkareRbIfñaM DFO b:uEnþkarbgáeraK TaMgenH vaminsUvjwkjab;dUcRbePT Yersinia eT. eKRtUvBinitüGñkCMgW edayykcitþTukdak; ehIyRtUvrMlwkGñkCMgW[)anCaRbcaMfa RtUveTACYb BikSaCamYyRKUeBTü kñúgkrNImanekþAxøÜnminGacBnül;)an. GacfaebI caM)ac; RtUvbBaÄb;fñaM DFO sin rhUtdl;eBleraKvinicä½yNamYy )aneKrk eXIjc,as;. plvi)akd¾kMrcMeBaHfñaM DFO eKminRtUvcak; DFO eday)aj;eGaylOncUlkñúgxøÜneT eRBaHvaGacbgá[ eLIgRkhmelIEs,k/ cuHsm<aFQam/ ebHdUgedIrjab; ehIymanrhUtdl; snøb; ¬Shock¦. karxUcxatdl;tMrgenam nig bBaðasYtF¶n;F¶r ¬Bi)akdkdegðIm¦ RtUv)aneKraykarN_fa ekItmaneBlNacak;bBa©Últam srésQam edaykMriteRbIx<s; 10 mRk¼KRk¼ema:g b¤ eRcInCagenH. Siderophore

karmanKP’ karsikSaCapøÚvkarBMuTan;)anbBa¢ak;BIsuvtßiPaBénfñaM DFO GMLúgeBlman épÞeBaHenAeLIy. bc©úb,nñenH dMbUnμand¾l¥cMeBaHGñkCMgWmanépÞeBaH EdlRtUv karkMcat;CatiEdk KWRtUveCosvageRbIfñaM DFO GMLúgeBl 3Ex dMbUgénkar manKP’. GñkCMgWmanépÞeBaH ehIymanelIsCatiEdkxøaMgkøa b¤ manbBaða ebHdUgF¶n;F¶r/ RtUveRbI DFO kñúgkMritTab 20 - 30 mRk¼KRk¼éf¶ enARtImascugeRkayénKP’ ehIyKμanKuNvibtþiGVIeLIy. eKGaceRbI DFO bnþeTotTnÞwmKñanwgkarbMe)AedaH. karsMrbtamcMeBaHkarBüa)aledayfñaM DFO plGaRkk; nig karQWcab;bNþalmkBIkareRbIfñaM DFO mann½ysMxan;fa eKRtUvEtpþl;karKaMRTelIkTwkcitþdl;GñkCMgW edIm,I[Kat;Rbkan;x¢ab;kar Büa)al. evC¢bNÐit/ Kilanub,dæak-yika nig GñkmanGaCIBepSgeTot dUcCa GñkcitþsaRsþ/ mRnþIsgÁmkic© k¾dUcCa«Bukmþay RtUvpþl;dl;GñkCMgWRKb;v½y CaRbcaMnUvkþIsgÇwm nig elIkTwkcitþeGaybnþkarBüa)aledayfñaM DFO BImYy éf¶eTAmYyéf¶. TnÞwmnwgenHEdr eKminRtUvePøcnUvqnÞHd¾FMeFg edIm,Ies;enA teTAeTot ehIyrs;edayPaBRbesIr EdlGñkCMgWmñak;²RtUvBRgwgxøÜneLIg edIm,ICMnH[)annUvkMritTamTarEbbenH. karBüa)alfñaM DFO mankarlM)ak/ eRbIeBlevla/ Qwcab; nig mantMéléfø ehIyRtUvRbkan;x¢ab;Gs;mYyCIvit. eTaHbIvaCakarBüa)alCYyseRgÁaHCIvit k¾eday/ DFO BMu)anpþl;[GñkCMgWnUvGarmμN_fa )anTTYlplRbeyaCn_Pøam² dUceRkaykarbBa©ÚlQamenaHeT. pÞúyeTAvijKuNRbeyaCn_énkarkMcat; CatiEdkvaGacbgðaj[eXIjkñúgry³eBlyUr kñúgkarkarBar b¤ Büa)alpl vi)akF¶n;F¶rCaeRcInbNþalmkBIelIsbnÞúkCatiEdk. edaysarEtplRbeyaCn_ karBüa)aledayfñaM DFO BMubgðaj[eXIjPøam manGñkCMgWCaeRcInCYbbBaða elIsCatiEdk sUm,IEtenARbeTsGPivDÆn_sMbUrfñaM DFO k¾eday enHedaysar EtGñkCMgWKitfaBi)akGnuvtþn_tamkarBüa)al. eBlEdlGñkCMgWQandl;v½y

L1 molecule

Cag 10qñaM nig eBjv½y rEmgFujRTan;cMeBaHkarBüa)al eRBaHvaGacrMxan dl;skmμPaBRbcaMéf¶/ GaCIB/ kic©karpÞal;xøÜn nig / kic©karsgÁm. dUecñH eBlEdlenARbeTskMBugGPivDÆn_ GRtaGñkCMgWmanCIvitmankMritTab CaTUeTAbNþalmkBIxVHxatfñaM nig sñb;sMrab;cak;fñaM/ enARbeTsGPivDÆn_vij bBaðaTMngbNþalkmBIxVHPaBRbkan;x¢ab;tamkarBüa)aleTAvij. eRkABIcMNuc sMxan;EdlRtUvpþl;fñaM nig sñb;sMrab;cak;fñaM EdlGñkCMgWRtUvkarsMrab; karBüa)al b¤ k¾RtUvFana[)anfaGñkCMgWRtUvkan;x¢ab;eTAtamkarBüa)al/ GñkCMgWenARtUvkarCYyeRCamERCg nig elIkTwkcitþBIsmaKmCatiCMgW taLasesmIu/ buKÁlikeBTü/ «Bukmþay nig GñkCMgWepSgeTot. TIF ¬shB½n§GnþrCati CMgWtaLasesmIu¦ k¾CaRbPBmYysMxan;pþl;karKaMRT edayCYybBa©úHbBa©ÚlrdæaPi)al[CYydl;karBüa)al nig kmμviFIkarBar. fñaMkMcat;CatiEdk epSg²eTot RbsiT§iPaBrbs;fñaM Deferiprone (L1, Ferriprox®, Kelfer®) edIm,IkMNt;RbsiT§iPaB nig karBarKuNvibtþibNþalmkBIeRbIfñaM Deferiprone (L1) eKRtUvGnuvtþCMhandUcteTA ³ [i] eKRtUvvas;kMrit Ferritin kñúgQamerogral; 3Ex mþg. [ii] eKRtUvrab;RKab;QamTaMgGs;erogral;s)aþh_ edaytamdancMnYneKalika Qam>s. [iii] erogral; 3 Ex mþg eKyksMNakTwkenamkñúg 24 ema:g edIm,IkMNt;bnÞúk CatiEdkkñúgTwkenam. karkMNt;bnÞúkCatiEdkenHmanlkçN³sMxan;Biess kalNaeKeRbIfñaM L1 eRBaHbrimaNCatiEdkesÞIrEtTaMgGs;beBa©jtam Twkenam xusBIfñaM DFO. [iv] karviPaKCIvKImI ¬Biochemistry¦ GMBImuxgareføImRtUvEteFVIerogral;Ex.

KYrvas;kMritCatisgá½sI erogral; 3 - 6Ex. RKUeBTüBüa)al CaGñkdwgBIsPaBrbs;GñkCMgWc,as;las; KW CaGñksMerceTAelI cMnYndg nig lMdab;lMedayetsþtamdan. ebIGaceFVI)aneKRcwbsac;eføImmk Binitü ¬k¦/ eRbI]bkrN_ SQUID ¬x¦/ eRbI MRI ¬K¦ Gacpþl;Bt’man c,as;las;BIkMritbnÞúkCatiEdkkñúgeføIm nig CalikaebHdUg. GMLúg BIrbIqñaMcugeRkayenH Tinñn½ysMxan;²mYycMnYnmaneRcIneLIg²Tak;Tgnwg RbsiTi§PaB nig suvtßiPaBfñaM Deferiprone . RKUeBTüBüa)alCMgWtaLasesmIu CaeRcIn )aneFVIkarviPaKTinñn½yTaMgGs;EdlmanTak;TgnwgRbsiT§iPaB RBmTaMg PaBgayRsYltamdaneBleKeRbIfñaMenH. eK)aneFVIkarvaytMélfa cab;taMgBIkarsakl,gKøInikelIkdMbUgbMputmk cMnYn GñkCMgWsrubCag 15000 nak; )anTTYlfñaM Deferiprone enACag 50 RbeTs TUTaMgBiPBelak mYyPaKFMsuT§EtCaGñkCMgWtaLasesmIu elIsbnÞúkCatiEdk. bc©úb,nñenHfñaM Deferiprone )anRtUvcuHbBa¢IkarenACag 50 RbeTsTUTaMg BiPBelak. b:uEnþenAminTan;mankaryl;RsbBI rdæ)alcMNIGahar nig »sf ¬FDA¦ enAeLIyeT. rhUtdl;Ex mIna qñaM 2007 eK)ancuHfñaM Deferiprone kñúgGtßbTviTüasaRsþ 495 c,ab; kñúgkarsakl,gKøInikcMnYn 60 ¬13 eFIVedaymineRCIserIs¦ nig cuHkñúgGtßbTepÞógpÞat;EpñkviTüasaRsþcMnYn 78 c,ab;. plrMxanrbs;fñaM L1 eKsMKal;eXIjfa GñkCMgWmYycMnYntUcRtUvbBaÄb;kareRbIfñaM Deferiprone bNþalmkBIkarBul. plb:HBal;sMxan;bMputrbs;fñaM L1 KWkarfycuHeKalika Qam>s CaBiessKW Neutrophils EdledIrtYnaTIsMxan; kñúgkarkarBarsarBagÁkayTb;Tl;nwgkarbgáeraK. karfycuHlμmén Neutrophils ¬500 - 1500¼mm3¦ eKehAfa [v]

ÉkarfycuHxøaMg ¬< 500¼mm3¦ eKehAfa Agranulocytosis. eyagtamkarsikSamYycMnYn EdlvaytMélcMeBaHkareRbIR)as; L1, ]sSah_eXIj Neutropenia Cag/ rIÉ Agranulocytosis ekItmankñúgkrNI tictYc. ya:gNak¾eday krNITaMgBIrenHGacvilRtLb;mkFmμtavij ebIGñk CMgWQb;eRbI L1. dUecñHvasMxan;Nas;cMeBaHGñkCMgWeRbIfñaM L1 RtUvBinitücMnYn eKalikaQam>s ya:gehacNas;erogral; 7 eTA 10 éf¶ mþg. eKRtUvBinitü GñkCMgW[)anjwkjy ERkgmanbgðajeraKsBaØaénkarbgáeraKNamYy. sBaØadMbUg KWekþAxøÜn/ QWbMBg;k b¤ sBaØaepSgNamYyeToténkarbgáeraK/ GñkCMgWRtUvQb;eRbI Deferiprone Pøam ehIyBinitüQamlMGit nig pþl;kar Büa)al[)ansmRsb. krNIPaKeRcIncMnYneKalikaQam>s vilRtLb;mk Fmμtavij ehIy Deferiprone Gaccab;epþImvijmþgeTot)an. eTaHya:gNa k¾eday eKcab;eRbI L1 vij Tal;EteKalikaQams esμI b¤ elIsBI 3000¼mm3/ cMnYn Neutrophils esμI b¤ elIsBI 1000¼mm3 hIycMnYn pøaEkt esμI b¤ elIsBI 100 000¼mm3. edaymanKuNvibtþiEbbenH eKRtUvBicarNaedayykcitþTukdak; munnwgeRbIfñaM L1 cMeBaHGñkCMgWtaLasesmIukMritF¶n; ehIymanCMgWrlakeføIm B nig¼b¤ C EdlsuT§EtTamTarkarBüa)aleday Interferon pgEdr. plrMxanepSg²eTotrbs; Deferiprone [i] GñkCMgWmYycMnYnEdleRbI L1 TTYlnUvkarQWcukcab; nig rlakenAtam snøak; dUcCak,alCgÁg; keCIg Ekgéd RteKok nig¼b¤ cegáH. BYkeKGac mansPaBrwgxøÜn nig Bi)akeFVIclnaepSg². eTaHbIeKBMuTan;yl;)anc,as; BIbuBVehtubgá[manplrMxanenHk¾eday EteKKitfavaGacmkBIkarrlak ¬Inflammation¦ EdlGacbgáedayBYkCatiEdkEdlfñaM L1 RbmUlBIkEnøg epSg²mkpþúMenAsnøak;. karbnßykMriteRbI b¤ bBaÄb;fñaM L1 plrMxanenH nwg)at;eTAvij. karQWcab;snøak; Gacbn§ÚbnßyedayeRbIfñaMbM)at;karQW cab; ¬Analgesic¦. ebIkarbnßykMriteRbIfñaM L1 nig¼b¤ eRbIfñaMbM)at;kar Neutropenia,

QWcab;enAEtmin)anFUres,Iy eKRtUvbBaÄb;eRbIfñaM L1 Etmþg. mü:ageTot ebIkarQWsnøak;ehIymanehImeTot CaFmμtaeKtMrUv[GñkCMgWQb;eRbIfñaM Edr. eKGaccab;epþImeRbI L1 eLIgvijmþgeTot edaykMriteRbITabCagmun ehIytamdaneGayditdl; eRBaHvagaynwgbgáeGayQW nig ehImsnøak; ekItmanmþgeTotEdr. [ii] bBaðaRkBHeBaHevon dUcCa ceg¥ar/ vilmux/ k¥Üt/ raK nig QWeBaH ekItman elICMgWxøHEdleRbI L1. eraKsBaØaTaMgenHrEmgminsUvxøaMgkøa ehIymin TamTarkarBüa)aleT. fñaM ¬bM)at;CatiGasIutkñúgRkBH/ bM)at;k¥Ütceg¥ar¦ CaTUeTAbnßyeraKsBaØaTaMgenH. elIsBIenHeTot elbfñaM L1 CamYycMNI GaharGacCYybnßyk¥Ütceg¥arEdr. [iii] GñkCMgWeRbI Deferiprone GacnaMeGaykgVHCatixniC nig sgá½sI ¬Zinc¦ edaysarfñaMenHvaGacP¢ab;nwgelah³FatuepSg²eTot eRkABIelah³Cati Edk dUcCa sgá½sI EdlCasarFatuEdlsarBagÁkayeyIgRtUvkarsMrab; dMeNIrkarmYycMnYn dUcCakarrIklUtlas;ragÁkay nig Es,k. karfycuHkMritsgá½sI eKsMKal;eXIjmanelIGñkCMgWxøH BiessGñkmanCMgW TwkenamEp¥m. bBaðaTaMgenHeKGacEksMrYlmkvijedaypþl;sgá½sIbEnßm. vaCakarcaM)ac;pgEdr edayFanafakar)at;bg;elah³FatuepSg²eTot edaysarkareRbIfñaM L1 RtUv)aneKpþl;b®gÁb;mkvij. TisedAénkarkMcat; CatiEdkkñúgCMgWtaLasesmIukMritF¶n; KWbeBa©jecalEtCatiEdkEdlelIs bgá[Bul ehIymaneRKaHfñak;Etb:ueNÑaH ehIyrkSaeGay)anBYkelah³ FatusMxan;²epSgeTotkñúgsarBagÁkay. [iv] karBuleføIm ³ karsikSaenAmCÄmNÐlCaeRcInbgðajfamankarekIneLIg kMritGg;sIumrbs;eføIm dUcCa ALT CaTUeTAmanlkçN³beNþaHGasnñ ehIy ekItjwkjyelIGñkCMgWrlakeføImRbePT C. lT§pletsþmuxgareføIm ERbRbYlcuHeLIg TamTar[GñkCMgWmYycMnYntUcRtUvEtQb;eRbI L1. man ÉksarmYy)anbgðajfa sPaBcak;es£Hedaysrésevov ¬Fibrosis¦ 75

Shuttle effect

enAeføImGacvivtþn_eTAmuxelOneBleRbI L1 CagkareRbIfñaM DFO ehIyeK min)anepÞógpÞat;karGegátTaMgenH enAkñúgkarsikSamYycMnYneTot nig karvaytMélkñúgry³eBleRkaymkenaHeT. eTaHCaya:gNak¾eday krNI GñkCm¶WmanCMgWrlakeføImRbePT C skmμ eKGacsegáteXIjmankarekIn eLIgsPaBcak;es£HedaysrésevovenAeføIm enAdMNak;kaleRkaymk eTot . kareRbIfñaMkMcat;CatiEdkedaypÁúMcUlKña kñúgbNþaqñaMcugeRkayenH mCÄmNÐlevC¢saRsþmYycMnYn)aneRbIrbbBüa)al pÁÜbKña KW Desferrioxamine nig Deferiprone edIm,I[)anlT§pll¥. eKal KMnitenHKWfa fñaMkMcat;CatiEdkTaMgBIrGaccab;ykCatiEdkBIRbPBepSg²Kña. kñúgrebobdUcenHskmμPaBfñaMmYyRtUvbMeBjbEnßmedayfñaMmYyeTot. fñaMmYy CaGñkdwkCBa¢ÚnCatiEdkBIekasikaeTAkñúgQam EdlenATIenaHfñaMTI 2 )anTTYl nigP¢ab;ehIybeBa©jmkeRkAsarBagÁkay. ynþkarEbbenHeRbob)annwg {plekItBIRtl;tm,aj ¬Shuttle effect¦}. viFIsaRsþénkarBüa)aleday eRbIfñaMpÁÜbenH kMBugsßitkñúgkarsikSaenAeLIy. Etya:gNak¾eday lT§plén kareRbIfñaMpÁÜbbgðajfa GñkCMgWelIsbnÞúkCatiEdkF¶n;F¶r b¤ elIsbnÞúkCatiEdk enAebHdUg ¬edayEp¥kelIlT§pl MRI T2*¦ kareRbIfñaMpÁÜbKñaBitCaman RbsiT§iPaB. lT§plenaHbgðajfa mankarFUres,Iymuxgarrbs;ebHdUg ¬muxgarftebHdUgxageqVg Ep¥ktam T2* nig karEk)aneLIgvijsPaBcgVak; ebHdUgedIrmineTog¦. dUecñH kareRbIfñaMpÁÜbKña k¾pþl;plRbeyaCn_dl;karrs;ranmanCIvit rbs;GñkCMgWpgEdr CaplmYymankarepÞógpÞat; edaykarsikSaelIkarrs;ranmanCIvitrbs;Gñk CMgWEdleK)ane)aHBum<pSay.

fñaMfμI Deferasirox CaRbePTRKab;elbtammat; bc©úb,nñenH fñaMkMcat;CatiEdkepSgeTotmYycMnYnkMBugsßitkñúgkMNak;kal RsavRCav BiessfñaMkMcat;CatiEdkeRbIelbtammat;)an. manfñaMmYyb:ueNÑaHén bNþafñaMTaMgenH)anQancUldMNak;kalsakl,gKøInIk KWfñaM ICL670 ehA müa:geTotfa Deferasirox ¬b¤maneQμaHBaNiC¢kmμfa Exjade®¦. Rkumh‘un plitfñaMenH KWRkumh‘unEdlplitfñaM Desferrioxamine. eRkayBI)anbBa©b;karsakl,gKøInik mkdl;eBlenHeKkMNt;)annUvsuvtßiPaB RbsiT§iPaB nig kMritfñaMsmRsbrbs;mnusS. enAkñúgkmμviFId¾FMbMputénkarsak l,gKøInIk cMeBaH Deferasirox EdleFVIeLIgelIGñkCMgW KWmanGñkCMgW)ancUlrYm dl;eTACag 1000 nak;. mkdl;eBlenH manbTBiesaFn_ 5 qñaMehIybnÞab; BIkaryl;RBmCapøÚvkarmk. manCag 70 RbeTskñúgBiPBelakehIy Edl)anpþl;karyl;RsbcMeBaHfñaM Deferasirox dUcCa ³ shrd§Gaemrik ¬FDA¦ RbeTssVIs GWru:b kaNada GURsþalI RbeTsGara:b;rYm GIuRsaEGl eRbsIul nig GahSg;TIn enAbNþa 50 RbeTs fñaM Deferasirox )andak;[eRbIR)as;dl;GñkCMgW eday minkMrittwgrwgdl;karecjevC¢bBa¢NaeLIy. mkdl;RtImasTImYyénqñaM 2007 fñaMenHman[eRbIR)as;enAbNþaRbeTsepSgeTotCaeRcIn.

ICL670A molecule

mkdl;eBlenH eKvaytMélfa enATUTaMgBiPBelakmanGñkCMgWRbmaNCa 15000 nak; TTYlkarBüa)aledayfñaM Deferasirox . Deferasirox eRbIsMrab;Büa)alelIsbnÞúkCatiEdkraMér:bNþalmkBI ³ k> GñkCMgW Ebtta taLasesmIu cab;BIGayu 2 qñaMeLIgeTA ehIyTTYlkar bBa©ÚlQameRcIndg. x> GñkCMgWTTYlrgsPaBesøksøaMgepSg² nig GñkCMgW Ebtta taLasesmIu EdlbBa©ÚlQamjwkjab; b¤ eBlNakarBüa)alkMcat;CatiEdkedayfñaM Deferrioxamine minGaceFVIeTAekIt b¤ k¾eKKitfaenAminRKb;Tan;RKan;. karsMerccitþcab;epþImeRbI Deferasirox eRscEtenAelIkarvaytMélrbs;RKU eBTüBüa)al. kñúgkMrit fñaM 20-30 mRk¼KRk¼éf¶ Deferasirox kMcat;Cati EdkecjBIeføImRbhak;RbEhlnwgkareRbIfñaM Desferrioxamine kñúgkMrit 3540 mRk¼KRk¼éf¶. bc©úb,nñenHBMumankareRbIfñaM Deferasirox pÁÜbCamYykMcat; CatiEdkEpSgeToteT eRBaHKμankarsikSapþl;Bt’mansMrab;kareRbIfñaMpÁÜbdUcenH eT. Deferasirox manlT§PaBpþl;karkarBar)an 24 ema:g edayelbEtmþgkñúg mYyéf¶erogral;éf¶. sUmkMNt;cMNaMfa muncab;epþImkarBüa)alkMcat;CatiEdk edayfñaM Deferasirox eKRtUvkMNt;kMrit Ferritin kñúgQammun. elIsBIenH muxgareføIm nig tMrgenamk¾dUcCamuxgarRtecok nig EPñk RtUvEtBinitüCamun sin. kñúgGMLúgBüa)alk¾RtUvBinitütamdandUcKñaenHEdr. enHKWeyageTAtam karENnaMsMrab;kareRbIR)as;fñaMenHBIRkumh‘unplitfñaM nig eTAtamkarENnaM rbs;evC¢bNÐit. pøÚveRbIR)as;fñaM Deferasirox manTMrg;CafñaMRKab;EdlrlaykñúgTwk b¤ TwkRkUcRc)ac; b¤ TwkEpø e)a:m. fñaMenHelbmunBisarGarharya:gehacNas; 30 naTI eBlvarlay 78

Gs;PøamkñúgTwk. eKminelbfñaMenHkñúgsPaBminrlay b¤ CaemSAeLIy. kñúgmYyéf¶eKeRbIEtmþgkñúgkMrit 20-30 mRk¼KRk. etI Deferasirox dMeNIrkardUcemþc Deferasirox eTAP¢ab;nwgGatUmEdkkñúgTMrg; Fe3+ . dUecñHGatUmmYyrbs; Fe3+ RtUvP¢ab;nwgmU:elKul Deferasirox BIr. bNþMú ¬Complex¦ EdlekIt ecjBIkarP¢ab;m:UelKulEdk nig mU:elKul Deferasirox enHRtUvbeBa©jecal tamlamk. edaysarvaeFVIemtabUlIsyWtkñúgeføIm fñaMenHvasßitkñúgQam ry³eBlCaeRcInema:g ¬Bak;kNþalCIvitrbs;vamanBI 9-11 ema:g¦. dUecñH kMritfñaMelbmYydgkñúgéf¶GacmanRbsiT§iPaBry³eBleBj 24 ema:g. RbsiT§PaBrbs;fñaM eK)anbgðajfa kMritfñaM 20 mRk¼KRk GaceFVI[kMrit Ferritin kñúgQamenA efrRbmaN 2000 μg/l rIÉkMritfñaM 30 mRk¼KRk GacbnßykMrit Ferritin cuHdl; 1000 μg/l kñúgGMLúgry³eBlmYyqñaMénkareRbIfñaMCab;rhUt. karRcwb yksac;eføImmkBinitübgðajfa fñaM)ankMcat;CatiEdkecjBIeføImBiesseBleRbI kMrit 30 mRk¼KRk. müa:geTot Tak;TinnwglT§PaBrbs;fñaMkñúgkarkMcat;Cati EdkecjBIebHdUg karBiesaFn_edaybNþúHekasika bgðajfa fñaMenHcUleTAkñúg ekasika ehIycab;ykCatiEdkecjBIekasika. ya:gNak¾eday mkTl;nwg eBlenH karBiesaFn_EpñkKøInikBMuTan;)anbBa¢ak;[)aneBjeljnUvkarrkeXIj TaMgenHenAeLIyeT. eKrMBwgdl;karsikSaTUlMTUlay nig Evgq¶ayCagenHt eTAeTot. plrMxanrbs;fñaM Deferasirox kñúgs)aþh_dMbUgénkareRbIfñaM eKGacCYbRbTHeraKsBaØaraKruHeFVITukçeBaH. eraK sBaØaenHqab;)at;eTAvij ehIyCakarkMrEdlevC¢bNÐitcaM)ac;RtUvbnßykMritfñaM. GaceFVIeGayekþAxøÜn nig pþasaymYyry³eBl. GñkCMgWRbEhl 10 ° Gac 79

CYbsPaBknÞÜlrmas;elIEs,k EdlGacraldaleBjxøÜn b¤ enA)atéd b¤ )at eCIg mYyPaKFMekItmanGakar³enHkñúgGMLúgeBl 15 éf¶ dMbUgénkareRbIfñaM. eRkaymkrEmg)at;eTAvijbnþicmþg²edayBMucaM)ac;bBaÄb;fñaM b¤ bnßykMrit fñaMeT. eRkABIenH man 1° eKsegáteXIjkMrit Creatinine ekIneLIg ¬Creatinine CaRbUetGIunmYyEdlmanTMnak;TMng ya:gsñiteTAnwgmuxgarrbs;tMrg;enam¦. PaKeRcInkrNIEbbenH vamanTMnak;TngnwgkMritfñaMeRbI. müa:geTot manGñkCMgW mYycMnYntUcbu:eNÑaH EdlkMrit Creatinine ekItx<s;hYskMritFmμta. PaKeRcIn kMrit Creatinine vilRtLb;mkkMritFmμtavij eBleKbnßykMritfñaM. )an esckþIfa GñkCMgWPaKeRcInEdleKrkeXIjsPaBEbbenH minKYrekItkarRBYy )armÖeT EtCakarbBa¢ak;fa vaRtUvtamdanemIlmuxgartMrgenam[)anjwkjab; KW[)anral;Ex. müa:geTot eKminRtUvpþl;fñaMenHcMeBaHGñkCMgWEdlexSaytMrg enam nig¼b¤ kMBugQaneTArkkarexSaytMrgenam. eKkMreXIjPaBminl¥énGg;sIumrbs;eføIm ¬kMeNIt Transaminase¦. dUcKña enHEdr ebIeføImmandMeNIrkarminRsYleTAehIy RKUeBTüBüa)alKWCaGñksMerc faKYr b¤ minKYreRbIfñaMenH ehIydMeNIrkarGg;sIumrbs;eføImRtUvBinitü[)an ral;Ex. PaBvibløasekItenARtecok nig EPñkk¾eKeXIjelIGñkCMgWmYycMnYn tUcEdr. ya:gNak¾eday dUcKñanwgkareRbIfñaM Desferrioxamine Edr eKRtUv BinitüRtecok nig EPñk[)anral;qñaM. bMrameRbIR)as; ¬Contra-indications¦ kareRbIfñaMepSgeTotkñúgeBlCamYyKñanwgfñaM Deferasirox fñaMmYycMnYn dUcCa Rifampicin b¤ Phenobarbitone b¤ Phenytoin GaceFVI[kMhab;fñaM Deferasirox fycuHkñúgQam. enHeFVI[RbsiT§iPaBfñaMfycuHEdr. ebIcaM)ac; RtUveRbIfñaMdUcxagelI KWRtUvmankarENnaMBIRKUeBTüBüa)al. kareRbIfñaMvItamInesdMNalKñanwgfñaM Deferasirox BMuTan;mankarsikSaenA 80

eLIy. eTaHya:gNa BMuTan;eXIjmanplb:HBal;NaeTeBleKeRbIvItamInes 200 mRk CalkçN³mUldæanRbcaMéf¶. müa:geTot eKRtUveCosvagkarelbdMNalKñanUvfñaMbM)at;CatiGasIudmanpÞúk GaluymJÚ:m ¬Alumimium¦ dUcCa Alumimium hydroxide ¬Maalox nig¼b¤ Gaviscon¦ eRBaHBMumankarsikSaBIkarpÁÜbEbbenHeLIy. ebIcaM)ac;RtUvEteRbIfñaMenH enaHeK ENnaM[eRbIvaeRcInema:geRkayfñaM Deferasirox. karmanépÞeBaH nig kareRbIfñaM Deferasirox edaykgVHTinñn½y/ eKhammin[eRbIfñaM Deferasiox kñúgGMLúgeBlmanKP’ eLIy elIkElgEtRKUeBTüBüa)alyl;facaM)ac;sMrab;GayuCIvit.

CMBUkTI 5 bBaðaevC¢saRsþbNþalmkBItaLasesmuI nig karBüa)al karBüa)altaLasesmuIkMritF¶n; mankarvivtþn_y:agxøaMgkñúgry³eBlBIrTsvtS cugeRkayenH EdleFVI[GRtars;ranmanCivitekIneLIgkMritx<s; Rbkbeday KuNPaBCIvitGñkCMgWmanPaBRbesIrxøaMgkøa CaBiessGñkCMgWenARbeTselak xaglic. ya:gNak¾eday GñkCMgWtaLasesmuIkMritF¶n;GacTTYlrgnUvbBaða evC¢saRsþmYycMnYn kñúgenaHxøHbNþalmkBICMgWpÞal; ÉmYycMnYneTotbNþal mkBIkarBüa)almanlkçN³minRtwmRtUv b¤ min)anditdl; rYmmankarbBa©Úl Qammin)anRtwmRtUv xVHsuvtßiPaBQam nig xVHlT§PaBeRbIfñaM DFO. GeNþIkrIkFM GñkCMgWtaLasesmuIkMritF¶n;PaKeRcInmanbBaðaecaTelIGeNþIk ¬Spleen¦ EdlCasrIragÁmYyTn;BN’RsKaM TMhMb:unkNþab;éd sßitenAeqVgéd xageRkam sac;dMusnÞHRTUg nig eRkamq¥wgCMnI ¬emIlrUb 5a¦. GeNþIkFmμtamanpÞúk eKalikaQamRkhm 20 - 30 ml EtGñkCMgWEdlmankgVHRKab;Qam RkhmmFüm nig F¶n;F¶r ry³eBlyUrEdlbNþalmkBIkarbBa©ÚlQam minRtwmRtUv GeNþIkGacpÞúkQamrhUtdl; mYylIRt b¤ eRcInCagenH. enHbNþalmkBIGeNþIkplitRKab;QambEnßmkñúgdMeNIrmYyeKehAfa Extra-medullary erythropoiesis KWCakarplitRKab;Qam enAeRkATItaMgFmμtarbs;vakñúgxYrq¥wg kñúgkic©RbwgERbgCYysarBagÁkay edIm,ICMnHPaBkgVHRKab;QamRkhm. GeNþIkbMeBjmuxgarsMxan;²mYycMnYn kñúgenaHrYmmankarkarBarsarBagÁkay Tl;nwgkarbgáeraK edaykareRcaHQamkMcat;mIRkub/ )ak;etrI nig )a:ra:sIut EdlebotebonsarBagÁkay ¬emIlrUb 5a¦. GeNþIkk¾CaGñkTTYlxusRtUv 82

pgEdr kñúgkarkMeTcecaleKalikaQamRkhmv½ycMNas;dl;Gayu edaykar bMEbkvaedIm,Iyk køÚb‘Ín nig Edk BI eGm:UkøÚb‘Ín rbs;va edIm,IeRbIeLIgvij pliteKalikafμI. cMeBaHGñkCMgWtaLasesmuIkMritF¶n; dMeNIrykCatiEdk nig køÚb‘Ín edIm,IpliteLIgvijenH minRbRBwtþeTAdUcFmμtaeT. pÞúyeTAvijEdk)an kkpþúMkñúgGeNþIk b¤ EdkRtUvbeBa©jeTAkñúgcrnþQam ehIybBa¢ÚnRtLb;eTA GeNþIkvij. elIsBIenHeTAeTot eKalikaQamRkhmPaKeRcIn EdlGñkCMgW taLasesmuIplit)an manrUbragÁxusFmμta ehIybNþal[vas¥itCab;kñúg GeNþIk. CalT§pl GeNþIkrIkFMeTA² CYnkalk¾eFVI[eBaHGñkCMgWrIkFMtamenaH Edr. edIm,IxitxMeqøIytbtamtMrUvkarEdlekIneLIg GeNþIkritEtskmμxøaMgeLIg eFVIeGayGeNþIkrIkFM. kñúgdMeNIrenHpgEdr k¾mankarbMpøajeKalikaQam RkhmFmμtaEdl)anmkBIkarbBa©ÚlQam CaehtueFVI[GñkCMgWRtUvkarQam ecHEtekIneLIgral;eBlbBa©Úlmþg² b:uEnþkarbBa©ÚlBMuGacman\T§iBleTAelI PaBkgVHRKab;QamRkhmeT. GeNþIkskmμxøaMg k¾bMpøajsmasFatuepSg² eTotrbs;QamEdr dUcCa eKalikaQams nig paøEkt. eKminGacEkkunsPaBGeNþIkrIkFM)aneT. dUecñHebIeKdwgfa GeNþIksßitkñúg sPaBmYybgáeRKaHfñak;dl;sarBagÁkaydUceBalxagelI eKRtUvEtkat;vaecj eKehAfa Splenectomy Etkarkat;GeNþIkecjminGacBüa)altaLasesmuI )aneT vaRKan;EtedaHRsaybBaðamYycMnYnb:ueNÑaH EdlbgáedaysarGeNþIk rIkFM. karsMerccitþkat;GeNþIkecj RtUveFVIeRkayBI)anBicarNaedayykcitþ Tukdak;eTAelIktþaevC¢saRsþmYycMnYnrYmmancMNucsMxan;dUcxageRkam ³ [i] GeNþIkrIkFM )anesckþIfamanRbEvgbeNþayEvgCag 6 sm ehIybgá[ mankarrMxan. [ii] GñkCMgWecHEtRtUvkarbBa©ÚlQamekIneLIg eTaHKμanbBaðaecaTEpñkevC¢ saRsþepSgeTotk¾eday )anesckþIfa eBlNabrimaNtMrUvkarQamekIn

phagocyte filled with bacteria

a neutrophil that detected the bacteria nearby

1/5dg b¤ elIsBI 200 - 220 m>l¼KRk¼qñaM éneKalikaQamRkhm edIm,IrkSa[)ankMrit eGm:UkøÚb‘Ín mFüm. [iii] GñkCMgWRtUvmanGayuelIsBI 5qñaM . dUc)anCMrabehIy GeNþIkedIrtYsMxan; kñúgkarkarBarsarBagÁkayTl;nwgkarbgáeraK. dUcenHkarkat;GeNþIkecj vabegáIneRKaHfñak;karbgáeraKF¶n;F¶r. elIkumarGayuticCag 5qñaM eRKaHfñak; mankMritx<s;xøaMg eRBaHRbB½n§karBarrbs;vaenAminTan;maMTaMenAeLIy. \LúvenHeKcat;fa karkat;GeNþIkecj CadMeNIrEpñkvHkat;suT§saF BMuTak;Tg nwgeRKaHfñak;FMdMuEdleKeXIjenAGtItkaleT. eKk¾)anbegáItpgEdrnUv bec©keTskat;GeNþIkEtmYycMENk ¬minTaMgmUl¦ b¤ bec©keTs Embolisation kñúgkarRbwgERbgrkSamuxgarkarBarsarBagÁkayrbs;GeNþIk . karGeNþIkecj nig karbgáeraK karRBYy)armÖcMbgbMput eRkayBIkat;GeNþIkecj KWeRKaHfñak;énkarbgáeraK F¶n;F¶r. man)ak;etrICaeRcInecaTCabBaðFMeFgcMeBaHGñkCMgWkat;GeNþIkecj edaybgáeraKF¶n;F¶rGac[)at;bg;Civit. )ak;etrIEbbenH jwkjybMput KWBYk emeraK Streptococcus nig Meningococcus. eRKaHfñak;mankMritx<s;Nas; RbsinGñkCMgWmanGayuticCag 5qñaM ehIykan;Et x<s;Nas;eTot ebIGayuticCag 2qñaM. eRKaHfñak;enAEtx<s;eBjmYyCIvit eRkayBIkat;GeNþIk Biess 1 - 4qñaM eRkayBIvHkat;. manbec©keTs 3 ya:g edIm,IkarBar b¤ bnßyCaGtibrimanUveRKaHfñak;bgáeraK cMeBaHGñkCMgWkat;GeNþIkecj ³ [1] Immunoprophylaxis : kareFVI[manPaBsaMuedaycak;fñaMbgáarBYk Pneumococcus, Haemophilus influenza nig Meningococcus. karcak;fñaMbgáar FmμtaeKeFVIenARbmaNCa 2s)þah_ munkarvHkat;ehIy mþgeTotenAeRkaykarvHkat; eyageTAtameKalkarN_ENnaM. 84

fñaMpSH FmμtaeKeRbIfñaM Penicilline elbtammat; 125 mRk elbBIrdgkñúgmYyéf¶ cMeBaHekμgGayuticCag 2qñaM ehIy 250 mRk elbBIrdgkñúgmYyéf¶ cMeBaHekμgGayuelIs 2qñaM. ebIGñkCMgW minGaceRbI Penicilline )an eKGaceRbIfñaMpSHepSgeTot)an. y:agNa k¾eday ry³eBlénkareRbIERbRbYlBIkrNImYy eTAkrNImYy. ]TahrN_ RKUeBTüxøH[GñkCMgWkat;GeNþIkecj eRbIfñaMpSHGs;mYyCIvit ÉRKUeBTüxøH eTot[eRbIrhUtdl;GñkCMgWmanGayu 18qñaM ehIyGñkxøHeTot[eRbIEt ry³eBl 2qñaM eRkayBIkat;GeNþIkecj. eTaHCakrNINak¾eday GñkCMgW nwgTamTarnUvkarvaytMélCaeTogTat; edIm,Ifana[)anfa eRKaHfñak;RtUv )anrkSa[enAkMritGb,brma. [3] karGb;rMENnaM ³ Gb;rMENnaMGñkCMgW nig «BukmþayGñkCMgWGMBIeRKaHfñak; énkarbgáeraKNamYy ehIyelIkTwkcitþBYkeK[RbugRby½tñcMeBaHsBaØa NamYyénkarbgáeraKEdlGacekItmaneLIg ]TahrN_ ekþAxøÜn/ minRsYl kñúgxøÜn b¤ Qwsac;dMu TaMgenHmansar³sMxan;Nas;. GñkCMgW nig «Bukmþay GñkCMgW EdlmanKMerageFVIdMeNIrq¶ay RtUvyl;dwgpgEdr GMBICMgWqøgepSg² EdlmanenAtMbn;epSg²kñúgBiPBelak. [4] RtUvRbugRbyt½ñcMeBaHcMnYnpaøEktekIneLIg ³ karekIneLIgcMnYn paøEkt GacekItmaneRkayBIkat;GeNþIkecj )anesckþIfa ekInelIsBI 800 000¼mm3. sPaBenHGacRKb;RKg)anedayeRbI Aspirine 50 - 100 mRk¼éf¶ rhUtdl;paøEktRtLb;mkkMritFmμtavij. niyayCarYm/ CMhanl¥bMputKWRtUveCosvagbBaðaTak;TgnwgGeNþIk[)aneday qab;cab;eRbIQammansuvtßiPaBehIy)anviPaKRtwmRtUv eBleraKvinicä½ydwgfa CaCMgWtaLasesmuIkMritF¶n; edayrkSakMrit Hb [elIsBI 9 - 10 Rkam¼d>l karenH GaceFVI[GeNþIkminqab;rIkFM b¤ minrIkFMk¾man ehIyeCosvag)ankar kat;GeNþIkecj. elIsBIenHeTot karrkSaGeNþIk[enATMhMFmμta CYyFana dl;RbsiT§iPaBénkarbBa©ÚlQam. [2] Chemoprophylaxis :

plvi)akcMeBaHebHdUg nig RkeBjbeBa©jkñúg (Endocrine) GñkCMgWtaLasesmuIkMritF¶n; CaerOy²CYbbBaðaebHdUg/ eføIm nig RkeBj beBa©jkñúg/ TaMgGs;enHrEmgbgáedaybBaðaelIsbnÞúkCatiEdk. b:uEnþvamin dUcbBaðaebHdUgeT ¬GanenAEpñkxageRkam¦ GñkCMgWEdlTTYlQammin RKb;RKan; b¤ min)anTTYlkarbBa©ÚlQam kMrmanbBaðaeføIm nig RkeBjbeBa©j kñúgNas; mYycMENkedaysarsPaBTaMgenHekIteLIgcMeBaHGñkCMgW)anbBa©Úl QameRcIn EtTTYlfñaMkMcat;CatiEdktictYc ehIymYycMENkeTotedaysar GñkCMgWEdlbBa©ÚlQammin)anRKb;RKan;rs;enAmin)anyUr edIm,I[bBaða TaMgenaHekIt)an. plvi)akénkarelIsCatiEdk ebHdUg exSayftebHdUgeRkam TaMgsgxag ¬Biventricular failure¦ cgVak;ebHdUgedIrmineTogTat; ¬Arrhythmia¦ RkeBj Pituitary Hypogonadotrophic – Hypogonadism Bukq¥wg ¬Osteoporosis¦ RkeBjbeBa©jkñúg TwkenamEp¥m Hypothyroid hypoparathyroid

eføIm

sPaBcak;es£Hedaysrésevov RkineføIm/ CaBiess ebImanrlakeføImRbePT es skmμ

plvi)akcMeBaHebHdUg plvi)akcMeBaHebHdUg manCaTUeTAcMeBaHGñkCMgWbBa©ÚlQamminRKb;RKan; b¤ KμankarbBa©ÚlQam. GñkCMgWEbbenHmanPaBkgVHRKab;QamRkhmraMér: RBmCamYykarkkpþúMénCatiEdkenAebHdUgecHEteRcIneTA² TaMgenHeFVI[ebHdUg RbwgeFVIkarEfmeTot ehIyGaceFVI[exSayebHdUg. 86

mYyPaKFMénGñkCMgWbBa©ÚlQamminRKb;RKan; b¤ KμankarbBa©ÚlQam minGacQandl;GayuxÞg; 20qñaM)aneLIy edaysarCMgWebHdUgCa mUlehtucMbgbgá[søab;. GñkCMgW)anbBa©ÚlQamRKb;RKan; Etmin)anTTYlkarkMcat;CatiEdk manRbsiT§iPaB edaysarGñkCMgWmincg;eRbIfñaM DFO [eTogTat;tam karENnaM b¤ edaysarfñaM DFO mingayrk)an ¬b¤ mantMéléføeBk¦ GacCYbbBaðaebHdUg bNþalmkBIelIsbnÞúkCatiEdk. dUc)aneBal BIxagedImmkehIy karbBa©ÚlQam)annaMeTACamYynUvbrimaNCatiEdkbEnßm CaeRcIncUlkñúgsarBagÁkay ebImin)ankMcat;ecj vanwgeTAkkpþúMenARKb;srIragÁ TaMgGs;kñúgenaHmanebHdUg. CatiEdkelIskkpþúMenAebHdUgeRcIneTA² rMxandl; dMeNIrkarFmμtarbs;va eFVI[vamansmtßPaBtictYcTb;Tl;nwgkarbgáeraK b¤ CMgWepSgeTot. CalT§pl sac;dMuebHdUgGacexSay eFVI[vaRc)ac;QameTAkñúg sarBagÁkaymin)anxøaMg ¬emIlrUb 5c¦. GñkCMgWEdlbBa©ÚlQam)anRKb;RKan; Etmin)ankMcat;CatiEdkecj[)anRKb;RKan; CaTUeTAnwgsøab;edaysarbBaða ebHdUgenAGayuCag20qñaM. EpñkepSg²mYycMnYnrbs;ebHdUgRtUvb:HBal; edayCatiEdkEdlelIs dUcCa eRsamebHdUg/ sac;ebHdUg/ va:l;ebHdUg/ b¤ CalikabBa¢ÚncrnþGKÁisnIebHdUg. EpñknImYy²enH manmuxgarepSg²KñaEdlGacb:HBal;edaysarelIsCatiEdk edaybegáItCaeraKsBaØaepSg² rYmman cgVak;ebHdUgedIrmineTogTat; ¬Arhythmia¦/ karrlavijénsac;dMuebHdUgminRbRktI ¬Diastolic dysfunction¦/ karkRnþak;rujQamxusRbRktI ¬Systolic dysfunction¦/ karRcalsÞHénCatiTwkkñúgCalika ¬karRCabTwkcUleRsamsYt/ eRsamebHdUg/ eRsameBaH[CaTacTwk/ ehImGvyv³¦ nig sBaØaexSayebHdUgepSg²eTot. dUcKñaenHEdr CMgWebHdUgF¶n;F¶r bNþalmkBIelIsCatiEdk GacekItmaneLIg edayKμaneraKsBaØaNamYyk¾man. dUecñHsMxan;GñkCMgWRtUvBinitüsuxPaB [)an eTogTat;CamYyRKUeBTüCMnajCMgWebHdUgcab;taMgBIGayu 10qñaMeTA eBlEdl 87

A summary of the homeostatic control of the heart.

minTan;eFVITukçemø:H edIm,IrkeraKsBaØa[Tan;eBlevla dUcCa j½rRTUg/ xSl;K/ ht;/ QWcugdegðIm/ hat;R)aNqab;ht; b¤ k¾ehImenACMuvijkeCIg b¤ enAEpñk epSgeTotragÁkay. ebIkalNaeraKsBaØaEbbdUecñHekItehIy CMgWebHdUg Gacfa )anvivtþn_dl;dMNak;kalF¶n;F¶rrYceTAehIy. y:agNak¾eday eTaH muxgarebHdUg)anRtUvb:HBal;F¶n;F¶rk¾eday karBüa)alkMcat;CatiEdkxøaMgkøa GacCYysþaebHdUgmksPaBFmμtavij)an. tamdanmuxgarebHdUg GñkCMgWtaLasesmuI KYrRtUvBinitüvaytMéleBjeljEpñkebHdUg ya:gehac Nas;[)anmþgkñúgmYyqñaM - eRcIndgRbsinebImanbBaðaebHdUgehIy. etsþdUcxageRkamenH CaEpñkd¾sMxan;sMrab;karBinitüRbcaMqñaM ³ karBinitüragÁkayGñkCMgW nig Rbvtþi ¬kñúgenaHmankarerobrab;BIsBaØa NamYy¦ . ftkaMrsμIGiucenARTUg ¬Chest x-ray¦. eTaHbIbc©úb,nñmanbec©keTs pþl;Bt’manya:geRcInsMrab;GñkCMnajebHdUg k¾karftkaMrsμIGiucenA RTUgenAEtCa]bkrN_d¾mansar³sMxan; kñúgeBlEdlviFITMenIbepSg eTotBMumaneRbIR)as; edayvapþl;Bt’mansMxan;sþIBITMhMebHdUg/ sYt RBmTaMgkarRBman[dwgBIdMuplitRKab;QamRkhmenAeRkAxYrq¥wg EdlGacekItmaneLIg. ECG ¬vas;GKÁIsnIebHdUg¦ P¢ab;CamYylMhat;R)aN KWCaetsþmYy nwgbgðajGakar³cgVak;ebHdUgedIrmineTogTat; b¤ dMeNIrkarminl¥én ftebHdUgeRkam. ECG - ry³eBl 24ema:g. karP¢ab;]bkrN_tamdanBiess ¬Holter¦ Cab;xøÜnGñkCMgWry³eBl 24ema:g GaceGaydwg)anBIPaB minFmμtaNamYyéncgVak;ebHdUgEdl ECG FmμtaminGacdwg.

eGkUebHdUg. enHKWCa]bkrN_mYymansar³sMxan;Nas; KwvabBa¢ak;BITMhM ftebHdUg nig dMeNIrkarEpñknImYy²rbs;ebHdUg . MUGA scan . enHKWCakarBinitümYyeRbIsarFatu Radioisotope Edl pþl;Bt’manbEnßmsþIBIdMeNIrkarrbs;ebHdUg. karBinitüenHGaceFVIeLIg eBlGñkCMgWsMrak eTaHya:gNa Bt’manc,as;las;CagenHGacTTYl)an ebIeKBinitüeBlGñkCMgWeFVIlMhat;R)aN. edIm,IkarBar b¤ edIm,IRKb;RKgCMgWebHdUgelIGñkCMgWtaLasesmuIkMritF¶n; eKRtUv Rbkan;ykviFankarN_mYycMnYn ³ GñkCMgWEdlminTan;manplvi)akelIebHdUgenAeLIy RtUvpþl;Qam[)an RKb;RKan;edIm,IrkSakMrit eGm:UkøÚb‘Ín (Hb) [enARbhak;RbEhlkMritEdl eKENnaM KW 9/5 - 10 Rkam¼d>l (g/dl). GñkCMgWEdlmanplvi)akelIebHdUgehIy RtUvTTYlQam[)anRKb;RKan; edIm,IrkSakMrit eGm:UkøÚb‘Ín muneBlbBa©ÚlQam [enAkMrit 10 - 11 Rkam¼d>l edIm,IFananUvkarpþl;GuksIuEhSn[sac;dMuebHdUg)anl¥. kñúgeKalbMNgeCosvagbnÞúkF¶n;F¶relIebHdUg eKENnaMGñkCMgW[TTYl QamEdlmankMhab;eKalikaQamRkhmkñúgbrimaNtictYcEteRcIndg. krNImansPaBexSayebHdUg eKGacpþl;fñaMbBa©úHTwkenameBlbBa©Úl Qammþg² eRkamkarRtYtBinitürbs;RKUeBTü. GñkCMgWmanbBaðaebHdUg b¤ elIsbnÞúkCatiEdk RtUvGnuvtþtamkmμviFIkMcat; CatiEdkd¾xøaMgkøamYy/ kñúgenaHmanlT§PaBbBa©ÚlfñaM DFO bnþCab; 24ema:g ¬cak;eRkamEs,k b¤ bBa©ÚltamsrésedayeRbI kaetETrP¢ab;eTA sésEvnFM¦ kñúgkMriteRbI 50 - 60 mRk¼KRk¼éf¶. rbbBüa)alenH karBarnUvkarxUcxatNamYycMeBasac;dMuebHdUgenAeBlxagmux edaysar CatiEdkEdlelIs RBmTaMgkarBarsac;dMuebHdUgBIkarbMpøicbMpøajCaRbcaM rbs;ra:DIkal;esrI .

karsikSafIμ²)anbgðajfa kareRbI DFO nig L1 bBa©ÚlKñaGacbnßy)an ya:gelOnnUvbnÞúkCatiEdkenAebHdUg ehIyeFVI[RbesIrdl;dMeNIrrbs; ebHdUg. \LÚvenHeK)anrkeXIjfa karkMcat;CatiEdk[)anxøaMgkøa GaceFVI[)an FUres,IyCMgWebHdUgF¶n;F¶relIGñkCMgWtaLasesmuI. b:uEnþ karRtUvBüa)al kMcat;CatiEdkedIm,IkarBarCamun vaRbesIrCagcab;Büa)aleBlmanCMgW ebHdUgekIteLIgrYceTAehIy. karvaytMélkMhab;CatiEdkkñúgCalika ebHdUgmankarlM)ak. b:uEnþkarGPivDÆn_cugeRkay kñúgkareRbIbec©keTs MRI CameFüa)ayd¾l¥bMputsMrab;eFVIkarvas;EvgbnÞúkCatiEdkkñúgebHdUg. niyay[xøIeTA karBüa)alplvi)akelIebHdUgcMeBaHGñkCMgWtaLasesmuI kMritF¶n; BwgEp¥keTAelIkarBüa)al[eBjTMhwgkMcat;CatiEdkEdlkkpþúM kñúgCalika bEnßmeTAelIfñaMpøÚvkareRbICYyKaMRTsac;ebHdUgEdlexSay dUcCa ³ k> fñaMEdleFVI[RbesIrdl;skmμPaBRc)ac;Qamrbs;ebHdUg. fñaMTaMgenH sßitkñúgRkum EdlehAfa Angiotensin Converting Enzyme b¤ ACE inhibitors. x> fñaMbBa©úHTwkenam EdlCYysPaBht;cMeBaHGñkCMgWexSayebHdUg. K> fñaMEdlCYyEkkuncMeBaHcgVak;ebHdUgedIrmineTogTat; ¬Agent antiarrhythmique¦ . CMgWepSg²eTotdUcCa plvi)akcMeBaHRkeBjbeBa©jkñúg Hypothyroidism ehIynig Hypoparathyroidism b¤ k¾kgVHvItamInes GacbgáCMgWsac;dMu ebHdUgpgEdr EtCMgWTaMgenHGac)anFUres,Iyvij edaykarBüa)al mUlehtuedImrbs;va )anesckþIfa karBüa)alplvi)akénRkeBjbeBa©j kñúg b¤ Büa)alkgVHvItamInes.

plvi)akcMeBaHRkeBjbeBa©jkñúg RbB½n§RkeBjbeBa©jkñúg rYm manRkeBjCaeRcIn ³ RkeBj Pituitary, RkeBj Thyroid, Parathyroids, RkeBj Adrenals, nig ekasika Ebtta ¬β¦ rbs;lMEBg/ RBmTaMgBgsVasrbs;burs/ GUEvrrbs;RsþI. RkeBjTaMg Gs;enHTTYlxusRtUvkñúgkar plit nig beBa©jG½rm:Un/ BYk vagayTTYlrg\T§iBlBulénCatiEdkEdlelIskkpþúMkñúgekasikarbs;va Edl rMxandl;karplitG½rm:Un. dUecñH plvi)akeTAelIRbB½n§RkeBjbeBa©jkñúg CabBaðamanCaTUeTAcMeBaHGñkCMgtW aLasesmuI eTaHbIGkñ CMg)W ancab;epþmI Büa)al kMcat;CatiEdk)anRtwmRtUvBIedImTIénCIvti k¾eday. CMgRW keBjbeBa©jkñgú rYmman karrIkFMFat;manPaByWtya:v/ RkeBjv½y/ CMgTW kw enamEp¥m/ Hypothyroidism, Hypoparathyroidism ehIycMeBaHmnusSFMvij exSaymuxgarpøÚvePT. karrIkFMFat; (Growth) RbmaN 30 - 50° én GñkCMgWtaLasesmuIkMritF¶n; TTYlrgvibtþirIkFMFat; Edl bNþalmkBIktþamYycMnYn. kgVHRKab;QamRkhmraMér:/ rIkGeNþIk/ elIsbnÞúkCati Edk/ BulfñaM DFO, Hypothyroidism, PaBeBjv½y/ 91

Incidence (%) of endocrine complications in thalassaemia major

(from De Santis et al 1995 multicentre study on endocrine complications in thalassaemia major, Clin Endocrinol 42:581-86)

yWty:av/ Hypoparathyroidism, nig CMgWeføImraMér:/ TaMgGs;enHCa plGviC¢maneTAelIkarrIkFMFat; RBmTaMgkgVHG½rm:UnsMrab;rIkFMFat; b¤ PaBsauMeTAnwgskmμPaBrbs;G½rm:UnenaH/ PaBgaybMErbMrYlBn§ú/ kgVH cMNIGahar nig karkgVl;kñúgGarmμN_. enAbNþaRbeTsEdlGñkCMgWBMu)anTTYlkarBüa)alRtwmRtUv/ kgVH RKab;QamRkhmraMér: nig cMNIGaharminRKb;RKan; KWCabuBVehtu cMbg bNþal[Gak;xandl;karrIkFMFat; ÉbNþaRbeTsEdlGñkCMgW )anbBa©ÚlQamRKb;RKan;EtGnuvtþn_karBüa)alkMcat;CatiEdk min)anl¥/ karelIsbnÞúkCatiEdkCamUlehtusMxan;bgá[Gak;xandl;karrIk FMFat;. y:agNak¾edaycMeBaHGñkCMgWEdlTTYlkarBüa)alRtwmRtUv eday bBa©ÚlQam)anRKb;RKan; nig kMcat;CatiEdk)anl¥/ kMriteRbIx<s;rbs; DFO bgá[Buldl;q¥wg TIbMputeFVI[Gak;xandl;karrIkFMFat;. karBüa)aleGaymanRbsiT§iPaB cMeBaHbBaðarIkFMFat; BwgEp¥kelIkarvaytMél [)anc,as;las;BImUlehtubgárbs;va. dUecñHcab;taMgBIedImdMbUgénkumarPaB rhUtdl;v½yCMTg; eraKvinicä½yRtUvEteFVIeLIgedaykarBinitüKøInIkedayykcitþ Tukdak; nig eTogTat; RBmTaMgetsþmnÞIrBiesaFn_epSg². karvaytMélcMeBaH karbeBa©jG½rm:UnsMrab;rIkFMFat; nig skmμPaBEsþgecjrbs;va)anpþl;lT§pl pÞúyKñaeTAvij tMrUveGaykarBüa)aledayG½rm:UnenH cMeBaHEtGñkCMgWNaEdl bgðajfamankgVHG½rm:Un ehIyeqøIytbKYrCaTIeBjcitþeTAnwgkarBüa)al. PaBRkeBjv½y nig Hypogonadism enAeBlcab;epþImeBjv½y/ Hypothalamus EdlCaEpñkmYyénxYrk,alenA Ek,rRkeBj Pituitary cab;epþImbeBa©j G½rm:UnmYyeQμaHfa GonadotrophinReleasing Hormone (GnRH) EdleTAeP£acRkeBj Pituitary [plit nig beBa©j Gonadotrophins EdlmandUcCa ³ Follicle Stimulating Hormone (FSH) nig Luteinizing Hormone (LH). G½rm:UnTaMgenHeFVIskmμPaBeTAelI

RkeBjePT KWBgsVascMeBaHburs nig GUEvr cMeBaHRsþI edIm,I[varIkFMFat; ehIyplitbeBa©jG½rm:UnePT KW Testosterone cMeBaHekμgRbus/ Estradiol nig Progesterone cMeBaHekμgRsI. G½rm:n U ePTTaMgenHRtUv)andwknaMkgúñ sarBagÁkay RKb;RKgelIkarvivtþné_ nsrIragÁePTRbus nig ePTRsI RBmTaMgsmtßPaBbnþBCU . RkeBj Pituitary EdlCaGñkplit FSH nig LH qab;TTYl\T§iBlxøaMgNas;BI pleRKaHfñak;rbs;EdkesrI eFVIeGayfycuHlT§PaBrbs;RkeBjenHkñúgkar plitG½rm:Un. PaBRkeBjv½y nig Hypogonadism KWCaplvi)akeRcInCageK eTAelIRkeBj beBa©jkñúgEdlTak;TgnwgEdk enHebItamkarsikSaesÞIrEtTaMgGs;enAtam bNþaRbeTsmYycMnYn. PaBRkeBjv½yenH eKeGayniymn½yfaCaGvtþman énkarvivtþn_pøÚvePT dUcCa KμankarrIkFMénsudn;cMeBaHekμgRsIeBldl;Gayu 13 qñaM/ KμankarrIkFMénBgsVas cMeBaHekμgRbuseBldl;Gayu 14 qñaM. ebIKμansBaØaeBjv½yeT eBldl;Gayu 16 qñaM GñkCMgWRtUvvinicä½yfaman Hypogonadism KwekμgRbus BgsVas nig lwgÁenAEttUc ÉekμgRsIsudn;KμanvivtþehIyKμanrdUvdMbUg ¬Primary amenorrhoea¦. sPaBEbbenHrEmgbgáPaBkgVl;EpñkpøÚvcitþ ¬Psychological stress¦. RbsiT§iPaBBüa)alcMeBaHPaBRkeBjv½y GaRs½yelIkarvaytMélBImUlehtu bgá[)anc,as;las;. karelIsCatiEdk GacrMxandMNak;kalNamYyénkar vivtþn_EpñkpøÚvePTdUc)anerobrab;xagelI edayb:HBal;xus²KñaBImñak;eTAmñak;. dUecñH krNInImYy²tMrUveGayeFVIeraKvinicä½yedayRbugRby½tñ edayEp¥kelIkar BinitüKøInikeGayl¥itl¥n;. karBüa)alkMcat;CatiEdkRtwmRtUv mannaTIsMxan; kñúgkareFVI[RbesIrnUvplvi)akTaMgenaH. elIsBIenHeKeRbIfñaM Sexual steroid ¬Testosterone cMeBaHekμgRbus nig Oestrogen cMeBaHekμgRsI¦ edIm,ICYy CMrujkarrIkFMFat;RsbKñanwgkarvivtþn_lkçN³pøÚvePT nig begáInTMhMsrIragÁpøÚv ePT. RbB½n§RkeBjbeBa©jkñúg enAEtgayTTYlrg\T§iBlénkarelIsCatiEdk eTaHCa 93

GñkCMgW)anTTYleBjv½yFmμtarYcehIyk¾eday eRBaHCatiEdkEdlekIneLIg eBleRkaymkeTot GacenAEtbgákarb:HBal;dl;RkeBj Pituitary b¤ RkeBj ePT dEdl kñúgkrNIEbbenH rdUvrbs;RsþIGac)at;vij ¬Secondary amenorrhoea¦ ÉbursvijGacGsmtßPaBpøÚvePT ¬Secondary Importence¦ karplitTwkkamfycuH ehIysmtßPaBpþl;kMeNIt ¬Infertility¦ k¾fycuHEdr. Hypothyroidism

RkeBj Thyroid sßitenARtg;k ehIyedIrtYsMxan;kñúgkarFana[)annUvkar vivtþn_Fmμtarbs;xYrk,alkñúgbNþaqñaMdMbUgénCIvit ehIyeRkaymkedIrtY kñúgkarvivtþn_ nig rIkFMFat;TUeTA. Thyroxin CaG½rm:Unplit nig beBa©j edayRkeBj Thyroid rYmcMENkdl;kMritfamBlTUeTA nig emtabUlIs ¬Metabolism¦ énbuKÁlmnusSmñak;². b:uEnþeBlEdlEdkeTAkkpþúMenA RkeBjenH naMeGayfylT§PaBplitG½rm:Un Thyroxin ehIyekItCasPaB mYyehAfa Primary hypothyroidism. GñkCMgWTTYlrg Primary hypothyroidism GacTTYlGarmμN_rgarxøaMg nig gguyedk ehIyCaerOy²GñkCMgWbgðajnUvsPaBemIuk²EpñkragÁkay nig pøÚvcitþ ehIyeLIgTMgn;. karb:HBal;F¶n;F¶rcMeBaHRkeBj Thyroid bgáedaykarkkpþúM CatiEdk k¾Gacb:HBal;dl;muxgarebHdUgpgEdr. b:uEnþ hypothyroidism minEmnsuT§EtmaneraKsBaØaKøInikeT dUecñHeKeFVIeraKvinicä½y)andwgc,as;eday saretsþmnÞIrBiesaFn_eTogTat; ¬TSH, T3 nig T4¦ vaerogral;qñaM eRkayBI GñkCMgWQancUldl;Gayu 10qñaM . eBletsþmnÞIrBiesaFn_bBa¢ak;faman hypothyroidism ehIy ¬TSH ekIn Et T4 esrIenAFmμta b¤ cuHfy¦ eBlenaH eKBüa)aledayG½rm:Un Thyroxin eTaHbIGñkCMgWmaneraKsBaØaKøInik b¤ Kμank¾eday.

Hypoparathyroidism

RkeBj Parathyroid manTaMgGs;bYn EdlP¢ab;eTAnwgRkeBj Thyroid. muxgarsMxan;rbs;RkeBjTaMgenH KW RKb;RKgkMritkal;süÚm ¬Calcium¦ kñúgsarBagÁkaytamry³ Parathyroid hormone (PTH) EdlCaG½rm:Unplit nig beBa©jedayRkeBjenH. PaBelIsbnÞúkCatiEdk b¤ sPaBkgVHRKab;Qam Rkhm eFVI[b:HBal;dl;muxgarrbs;RkeBj Parathyroid ehIy[ekItCa hypoparathyroidism. karenHbgá[kMritkal;süÚmkñúgQamFøak;cuH sPaB mYyEdleKehAfa Hypocalcaemia EdlbnÞab;mkeTot manplb:HTgÁic eTAelIkMritsarFatuKImIsMxan;epSgeTot KW pUs½Vr. kMritkal;süÚm nig pUs½Vr manTMnak;TMngeTAnwgeraKsBaØaKøInikmYycMnYn. kMrit kal;süÚmTabGacbgá[manGarmμN_ QW-rYy enAtaméd nig eCIg CYnkal bgá[rmYlRkeBI nig rwgsac;dMu. karelceLIgnUvsPaBRbkac;TUTaMgxøÜnR)aN nig PaBGak;xanénmuxgarrbs;ebHdUgGacCaeraKsBaØacugeRkay. kartam danEpñkmnÞIrBiesaFn_eTAelIkMrit kal;süÚm/ pUs½Vr nig PTH kñúgQamGac CYykñúgkareFVIeraKvinicä½y. karBüa)aledayeRbI kal;süÚm nig vItamIn ed ¬Vitamin D¦ GacEkkunPaB minRbRktIén emtabUlIs enH)an. kñúgkrNIkMr EdlekItmankarrwgsac;dMu F¶n;F¶redaysarkMritkal;süÚmkñúgQamFøak;cuHxøaMg eKGaccak;kal;süÚmbBa©Úl tamsrés. CMgWTwkenamEp¥m ¬Diabetes mellitus¦ PaKeRcInénplvi)akcMeBaHkarelIsCatiEdkrauMér: mandUcCa CMgWeføImraMuér:/ karbgáeraKedayvIrus nig ktþaBUC. TaMgenH Cakar)at;bg;tulüPaBsárEdlcug bBa©b;vaGaceFVI[ekItCaCMgWTwkenamEp¥m. CMgWTwkenamEp¥m eKeGayniymn½y fa CakareLIgCatisárkñúgQameBlminTan;hUbGahar elIs 126 mRk¼d>l (mg/dl) b¤ eBlhUbGaharehIy elIs 200 mRk¼d>l Ékar)at;PaBTb;Tl; 95

CamYysárvijmanniymn½yfa Cakar)at;bg;lT§PaBrbs;ekasika β rbs; lMEBg kñúgkarbeBa©j Insulin eGay)anbrimaNsmRsb edIm,IeqøIytbnwg CatisárEdlcUleTAkñúgxøÜn. esÞIrEtBak;kNþalénGñkCMgWtaLasesmIukMritF¶n; suT§EtTTYlrgnUvkar)at;PaBTb;Tl;CamYysár É 10 - 30° eTotekItCa CMgWTwkenamEp¥m eBlNamYykñúgCIvitrbs;eK. RbvtþiRKYsarekItCaCMgWTwkenam Ep¥m CaBiesskñúgcMeNambgb¥ÚnExSrdMbUg )anesckþIfaCa «Buk b¤ mþay enaHeRKaHekItCMgWTwkenamEp¥mmankMritx<s;. TwkenamEp¥mekIteLIgeBlEdl emtabUlIs rbs; Catisrá RtUvkat;paþ c;ehIysárminGaccUlkñgú ekasika edIm,Ipþl;famBl EdlvaRtUvkaredIm,IbMeBj muxgar. srIragÁTTYlxusRtUvcMeBaH emtabUlIs Catisár KWlMEBgEdlsßitenAEk,rRkBH Edltam ry³ekasika β vaplit Insulin EdlCaG½rm:nU eRbIsrM ab;eFVeI mtabUlsI Catisrá . FatuEdkGaceFVI[xUcxatekasika β TaMgenH dUecñHlT§PaBeRbIR)as;sárrbs; sarBagÁkayRtUvkat;bnßyeFVI[sárCujeRcInkñúgQam. GñkCMgWTwkenamEp¥m kñúgTMrg;mFüm eKehAfa kar)at;PaBTb;Tl;CamYysár ¬Glucose intolerance¦ KμanbgðajsBaØaKøInikeT eKeFVIeraKvinicä½y)anedayetsþmnÞIr BiesaFn_. karRsavRCav)anbgðajfa GñkCMgWtaLasesmIu munQaneTAekItCMgWTwkenam Ep¥m RtUvqøgkat;dMNak;kal )at;PaBTb;Tl;CamYysár sin ehIykñúgkMLúg eBlenHkarplit Insulin mankarekIneLIgya:gEsþgEtmþg CakareqøIytb nwgskmμPaBvaEdlxUcxat. dMNak;kalenHeKehAfa karsaMueTAnwg Insulin ¬Insulin resistance¦ TamTarnUvkartamdanedayykcitþTukdak; GñkCMgWRtUv GnuvtþtamrbbGahar[)anRtwmRtUv ebIcaM)ac;RtUvsMrkTMgn; RBmTaMgkMcat; CatiEdk[)anxøaMgkøa. kar)at;PaBTb;Tl;CamYysár nig CMgWTwkenamEp¥m RtUveFVIeraKvinicä½ytam 96

ry³etsþmnÞIrBiesaFn_rkkMritCatisárkñúgQamenAdMNak;kalepSg² mun nig eRkaykarjaMu. ]TahrN_ kMritCatisárkñúgQamesμI b¤ elIs 7 mmol/l ¬b¤ 126 mg/dl¦ enAeBlRBwkminTan;jaMuGVITaMgGs; RtUveKkMNt;famanCMgW TwkenamEp¥m. kMritCatisárkñúgQamelIs 11 mmol/l ¬b¤ 200 mg/dl¦ enA 2 ema:geRkayBIcak;bBa©ÚlCatisárkñúgxøÜn k¾kMNt;)anfa CaCMgWTwkenamEp¥m )anEdr. kMritCatisárkñúgQamBI 8 - 11 mmol/l ¬140 - 200 mg/dl¦ enA 2 ema:geRkayBIjaMusár 75 Rkam eKehAfa etsþkMNt;PaBFn;RTaMcMeBaHsár bBa¢ak;BkI ar)at;PaBTb;Tl;CamYysár. etsþrkkar)at;PaBTb;Tl;CamYysár RtUveFVImYyqñaMmþgRKb;GñkCMgWTaMgGs; eBleKQancUldl;GayuCag 10qñaM. kñúgkrNICMgWTwkenamEp¥m/ TMrg;F¶n;F¶rxøaMg CakrNImankarb:HBal;F¶n;F¶r dl;karplit Insulin EdlGñkCMgWTamTarkarcak; Insulin eRkamEs,k erogral;éf¶ edIm,IeFVI[kMritCatisárkñúgQammkFmμtavij. TwkenamEp¥m CaCMgWGacBüa)al)an EtkarBüa)alCabnÞúkbEnßmmYyeTot. dUecñHkarKaMRT BIRKUeBTü RKYsar nig mitþPkþi CakarsMxan;. GñkCMgWTwkenamEp¥m RtUvEtBinitü emIlkMritCatisárkñúgQamenApÞH bIeTAbYndgkñúgmYyéf¶ edayeRbI]bkrN_ vas;kMritCatisár ehAfa Glucometer. lT§plénkartamdanenApÞHenH)an CYyRKUeBTükñúgkaréllkkMritfñaM Insulin eTAtamesckþIRtUvkarrbs;GñkCMgW. etsþKøInik nig mnÞIrBiesaFn_epSgeTotRtUveKGnuvtþn_edIm,IvaytMélplvi)ak rbs;CMgWTwkenamEp¥m KW Binitümuxgarrbs;tMrgenam nig ftrUbPaBemIlépÞ )atRkBH EdlCaBYksrIragÁgayb:HBal;bMputkñúgCMgWTwkenamEp¥m. eKRtUvelIk TwkcwtþGñkCMgWtaLasesmuI [Rbkan;x¢ab;nUvkarBüa)aleTogTat;edayfñaM DFO edayehtufa kareRbIfñaMenH)anRtwmRtUvGacCYykat;bnßy)aneRcInnUveRKaH ekItCMgWTwkenamEp¥m. CMgWBukq¥wg ¬Osteoporosis¦ q¥wgesþIgehIypuyeKeXIjmanCaTUeTAelIGñkCMgWtaLasesmuI bNþalmkBI ktþaCaeRcIn dUcCa kgVHRKab;QamRkhm/ xYrq¥wgskmμxøaMg/ cMNIGaharman

Typical sites of osteoporotic fractures

Example of DEXA results on a patient with osteoporosis (z score-5.9)

kMritkal;süÚmTab/ kMritCatiEdkkñúgq¥wgekInx<s;/ kgVHcMNIGahar/ PaBRkeBj v½y b¤ Hypogonadism nig bBaðabNþaRkeBjbeBa©jkñúgepSg²eTot RBmTaMg ktþaBUC TaMgGs;enHGacrYmcMeNkdl;karkekItCMgWBukq¥wg ¬emIlrUb 5e¦. GñkmanCMgWq¥wg rEmgmanskmμPaBragkayGn;fy ehIyGvyv³bþÚrTMrg;xøaMg ehIyeFVI[)ak;q¥wgF¶n;F¶r. CaTUeTAeKeFVIeraKvinicä½y CMgWq¥wgedayvas;dgsIuetq¥wgenAtMbn;q¥wgkg nig q¥wgRtKakedayeRbI DEXA ehIyebItMrUv[eFVI eKeRbIkartamdaneday mnÞIrBiesaFn_epSg²eTot. GgÁkarsuxPaBBiPBelakkMNt;fa fydg;suIetq¥wg ¬Osteopenia¦ eBlNadgsIuetq¥wgRtUvkat;bnßydl;BinÞú ³ -1 eTA -2.5 eRkamkMritFmμta ehIyBukq¥wg ¬Osteoporosis¦ eBlEdl ma:s;q¥wgTabCag -2.5 ¬emIlrUb 5f¦ . karBüa)alCMgWq¥wg sMxan;KWepþateTAelIkarkarBar tamry³karbBa©ÚlQam [)aneTogTat; karkMcat;CatiEdk[)anl¥/ Büa)alCMgWRkeBjbeBa©jkñúg/ eFVIlMhat;R)aN[)aneTogTat;. edIm,IkarBarkMu[Bukq¥wg GñkCMgWEdlman fydg;suIetq¥wg RtUvQb;Ck;)arI/ jaMuGaharsMbUrCatikal;süÚm ehIyelb vItamIn D bEnßm RBmTaMghat;R)aN[)aneTogTat;. elIsBIenHGñkCMgW Hypogonadism RtUvTTYlG½rm:UnePTedIm,IkarBarkMu[Bukq¥wg. ebIBukq¥wgekIt ehIy kareRbIfñaMmYycMnYn dUcCa Biphosphonates (Pamidronate, Aledronate) pþl;plRbeyaCn_xøHEtb:ueNÑaH . karpþl;kMeNIt nig bnþBUC ¬Fertility and reproduction¦ RsþImanCMgWtaLasesmuI GacmanépÞeBaH edaysuvtßiPaB. b:uEnþkarsMerccitþman épÞeBaH RtUvEtBicarNaeGayhμt;ct; edayKUrRskrRtUvRbwkSaCamYyRKUeBTü.

RsþIEdlmanbMNgcg;manépÞeBaHRtUveFVIkarvaytMél[)aneBjeljcMeBaH sPaBpøÚvcitþ nig sPaBKøInikrbs;Kat; . GñkCMgWtaLasesmuI ePTRsIEdlman rdUvFmμta GacmanépÞeBaHedayÉkÉg. EtGñkEdlKμanrdUvdMbUg b¤ )at;rdUv nwgRtUvkarBüa)aledayG½rm:UnedIm,IeP£ac[mankarplitemCIvitRsþI ehIyvaRCuH Føak;cUlmkkñúgs,Ún. GñkCMgWtaLasesmuI ePTRbusEdlmanEtTwkkamEtKμan emCIvit CaerOy²eqøIytbnwgkarBüa)aledayG½rm:UnbBa©ÚlKñakñúgry³eBl mYyqñaM. eBlNaeKdwgfaGñkCMgWmanépÞeBaHehIy eKRtUvRbkan;viFankarN_ mYycMnYn dUcteTA ³ 1> eKRtUvEtbBaÄb;kareRbIfñaM DFO eBlEdldwgfamanépÞeBaHPøam edaysar Et\T§iBlrbs;fñaMeTAelITarkminTan;dwgc,as;enAeLIy. ya:gNak¾eday tamkarsikSaelIstV fñaMenHmanplb:HBal;F¶n;F¶reTAelITarkstV. ebIsin GñkCMgWmanépÞeBaH mankarelIsCatiEdkxøaMgkøa b¤ manbBaðaebHdUgF¶n;F¶r enAdMNak;kalcugeRkayénépÞeBaHeKKYreRbIva kñúgkMritTab 20 - 30 mRk¼KRk¼éf¶. 2> RsþImanépÞeBaHRtUvbBa©ÚlQamkñúgbrimaNtic² Etjwkjab;CagFmμta edIm,IrkSa Hb [sßitkñúgkMritKYrcg;)an ¬10 - 15 Rkam¼d>l¦ ¬g/dl¦. 3> muxgarrbs;ebHdUgRtUvEttamdan[ditdl;. karRsavRCavbgðajfa RsþIEdlcab;manépÞeBaHkñúgeBlEdlkMrit Ferritin Tab/ muxgarrbs; ebHdUgmanPaBRbesIrCageBlEdlkMrit Ferritin x<s;. 4> eKRtUvRtYtBinitüGñkCMgW ERkgekItmanCMgWTwkenamEp¥m b¤ CMgWRkeBj beBa©jkñúgepSg²eTot. kñúgbNþaqñaMcugeRkayenH manGñkCMgWtaLasesmuICaeRcIn Edl)anTTYlkar Büa)albBaðabgákMeNIt)anRtwmRtUv)ansMerckñúgkarmankUnEdlmansuxPaB l¥. KUrRskrEdlédKUrTaMgBIrnak;manCMgWtaLasesmuIkMritF¶n; ehIyman bMNgcg;)ankUnEdlmansuxPaBl¥/ emCIvitburs b¤ RsþI Edl)anmkBIGñkeFVI 99

GMeNaysuxPaBFmμta RtUveKeRbIkñúgdMeNIr[mankarPBRbsBV bgákMeNIt ¬Fertilisation¦ EdlRtUveFVIenAeRkAsarBagÁkaykñúgmnÞIr BiesaFn_ rYcehIyemCIvitRsþIEdl)anPBRbsBVCamYyemCIvitbursehIy enaH RtUveKykbBa©Úlkñúgs,Únrbs;RsþI. karBüa)al nig kartamdanGñk CMgWEbbenH TamTarkarykcitþTukdak;CaRkum BIbNþaRKUeBTüÉkeTsCa eRcIn rYmman GñkÉkeTsEpñkelahitviTüa/ ÉkeTsEpñkkumar/ ÉkeTs EpñkRkeBjbeBa©jkñúg nig ÉkeTsEpñkeraKRsþI. snñidæanmk/ bc©úb,nñenHkarbnþrBUCcMeBaHGñkCMgWtaLasesmuIkMritF¶n; nig mFüm KWCasPaBBitCak;EsþgmYy. ]TahrN_ enARbeTssIuRBUs kñúgcMeNam RsþtI aLasesmuI 62 nak; ¬50 nak;krM ti F¶n; nig 12 nak;krM ti mFüm¦ manGayu mFüm 25qñaM sMerc)ankñúgkarmanépÞeBaHdl;eTA 90° kñúgenaH ³ 14 nak; tamry³karCYyeRCamERCg[manépÞeBaH KW eFVI[mankarRCuH emCIvitRsþImkkñúgs,Ún/ karPBRbsBVénemCIvitkñúgmnÞIrBiesaFn_ ¬In vitro fertilisation = IVF¦ nig kardak;bBa©ÚlemCIvitburs ¬Insemination¦ . ekIt)anTarkmansuxPaBl¥ 87 nak; kñúgenaH 69 nak; RKb;Ex/ 12 nak; minRKb;Ex ¬Edlman 4 épÞeBaHkUnePøaH nig 1épÞeBaHkUn 3¦. man 7 rlUt ehIysøab;ekIt 2 krNI. eKBMueXIjmanplvi)akF¶n;F¶rkñúgkar RbsURteT ehIyGñkCMgW 9 nak;manplvi)akeTAelIebHdUgmYyry³xøI b:ueNÑaH.

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100

CMBUkTI 6 karbgáeraKkñúgCMgWtaLasesmuI CMgWtaLasesmuIkMritF¶n; maneRKaHfñak;bgáeraKx<s;edaysar ³ kgVHeKalikaQamRkhm karkat;GeNþIkecj elIsbnÞúkCatiEdk karbBa©ÚlQam kareRbIfñaM Desferrioxamine kgVHeKalikaQamRkhm ¬Anemia¦ krNIGñkCMgWKμankarbBa©ÚlQamTal;EtesaH b¤ bBa©Úlmin)anRKb;RKan;/ kgVH RKab;QamRkhmCabuBVehtud¾sMxan;bMputbgá[manCakarbgáeraKF¶n;F¶r dUcCa rlaksYtCaedIm. eTaHbIbBaðaenHvakMrmanenARbeTselakxaglic eRBaH gayrkQambBa©Úl)anRKb;RKan; EtenARbeTskMBugGPivDÆn_mYycMnYnvij karbBa©ÚlQamminRKb;RKan; CabBaðaEtgekItmanCaeTogTat; ehIykarbgáeraK EbbdUecñHGacenAEtekItmanteTAeTot. karkat;GeNþIkecj ¬Splenectomy¦ GñkCMgWEdlmin)ancab;epþImkarBüa)aledaybBa©ÚlQamRKb;RKan;)anTan; eBlevla b¤ GñkCMgWEdl)ankat;GeNþIkecj RbQmmuxnwgeRKaHfñak;xøaMgkøa cMeBaHkarbgáeraKF¶n;F¶r dUcCa rlaksYtedayBYkemeraK Streptococcus, Haemophilus Influenza nig Neisseria Meningitides suT§EtCa)ak;etrI EdlmansMbkB½T§CMuvijkarBarxøÜn. manBYk )ak;etrI/ vIrus nig )a:ra:sIutepSg² eTotk¾GacbgáeraKF¶n;F¶rcMeBaHGñkCMgWEdlkat;GeNþIkecjEdr. enHedaysar GeNþIkCaGñkkarBarsarBagÁkayTl;nwgkarbgáreraK.

101

elIsCatiEdk ¬Iron load¦ GñkCMgWEdl)anbBa©ÚlQamRtwmRtUv b:uEnþkMcat;CatiEdkmin)anl¥/ edaysar mankarlM)aknwgrk)anfñaM Desferrioxamine b¤ k¾karGnuvtþn_tamkar eRbIR)as;min)anditdl;/ k¾GacgayeRKaHfñak;bgáeraKy:agF¶n;F¶rEdr. enHk¾ edaysarPñak;garbgáeraKrIkFMFat;edaysarFatuEdk ³ FatuEdkkan;Etx<s; kñúgxøÜn enaHPñak;garbgáeraKkan;EtrIkFMFat; begáIncMnYn ehIybgáeraKF¶n;F¶r. eraKEdleKsÁal;c,as;CageK KW bgáeday Yersinia Enterocolitica. )ak;etrIenHmindUc)ak;etrIepSgeToteT vaCa)ak;etrIBiessEdlKμanynþkar pÞal;xøÜnedIm,IRbmUlyk nig eRbIR)as;EdkBIbrisßanCMuvijxøÜnva)aneT. cMeBaH buKÁlmansuxPaBl¥ )ak;etrITaMgGs;enHKμaneRKaHfñak;eT ehIyKμansBaØaKøInik b¤ manEtbnþicbnþÜc. EtcMeBaHGñkCMgWtaLasesmIukMritF¶n; EdlelIsCatiEdk esrI b¤ EdkP¢ab;nwgm:UelKulfñaM Desferrioxamine, BYk Yersinia rIkFMFat; begáIncMnYnya:gelOn ehIybgáeraKF¶n;F¶r KMramkMEhgdl;Civit. fIVebImankarsikSaCaeRcIn sþIBItYnaTIrbs;EdkkñúgkarbgáeraKeday)ak;etrIk¾ eday Etk¾mankarsikSaCaeRcInEdr eTAelItYnaTIrbs;EdkkñúgkarbgáeraK edayvIrus ¬dUcCarlakeføIm nig eGds_¦ edayBinitüeTAelIrebobEdlEdk b:HBal;eTAelIkarvivtþn_eTAmuxénkarbgáeraKTaMgenH nig kareqøIytbcMeBaH karBüa)aledayeRbIfñaMtamkarENnaM. lT§plénkarGegát)anbgðajfa cMeBaHGñkCMgWtaLasesmuIkMritF¶n;/ karelIsbnÞúkCatiEdk GaceFVIeGayeCaK vasnaénkarrlakeføImruaMér:RbePT B nig C kan;EtGaRkk;eTA ehIykar Büa)alrlakeføImenHmanlT§plminsUvl¥. dUecñH RbsiT§iPaBénkarkMcat; CatiEdk hak;bIdUcCaedIrtYnaTIsMxan; kMNt;eCaKvasnaénkarrlakeføImraMuér: bgáedayvIrus. eK)anbgðajpgEdrfa cMeBaHGñkCMgWtaLasesmuIkMritF¶n;/ karbgáeraKeday vIrus HIV manlkçN³F¶n;F¶rxøaMgeLIg eBlNaeKeRbIkMritfñaMkMcat;CatiEdk DFO ticCag 40 mRk¼KRk b¤ eBlNakMrit Ferritin kñúgQamelIs 1935 102

mIRkURkam¼l ¬μg/l¦. niyayCarYm CatiEdkGacedIrtYnaTIsMxan;kñúgkarbegáInPaBF¶n;F¶rénkarbgáeraK cMeBaHGñkCMgWtaLasesmuIkMritF¶n; eRBaHEdkGac ³ [i] CacMNIGaharsMrab;karFMFat;Pñak;garbgáeraK [ii] CacMNIGaharsMrab; Gg;sIum ¬Enzyme¦ EdlRTRTg;karbegáIncMnYnén GtisuxumR)aN [iii] kMcat;sarFatuKImIsMxan; ehAfa Gg;TIGuksIudg; ¬Antioxidants¦ EdlCa sarFatuKImIkarBarekasika Tb;Tl;nwgkarrlak ¬Inflammation¦ [iv] eFVI[xUcRbePTekasikamYycMnYn EdledIrtYsMxan;kñúgkar karBarsarBagÁkay Tl;nwgkarbgáeraK. karbgáeraKbNþalmkBIkarbBa©ÚlQam QamRtUv)anTTYlsÁal;fa CaRbPBsMxan;énPñak;garbgáeraK EdlGaccMlg eTAGñkCMgWtamry³karbBa©ÚlQam. dUcenH eTaHCaQamGacCYyseRgÁaHCIvit vak¾GacbgáRbtikmμmYycMnYnEdleyIgmincg;)an rYmmankarbgáeraKF¶n;F¶r KMramKMEhgGayuCIvit. GtisuxumR)aNtUc²CaeRcIn Gacrs;ranmanCIvitmYyry³eBlkñúgQam eday bgáeraKdl;GñkCMgWeBlQamRtUv)anbBa©Úl. kñúgcMeNamenaHmanrlakeføIm B nig C, HIV-1 nig HIV-2 TaMgenHCaPñak;garbgáeraK EdlGacmanGakar³raMuér: F¶n;F¶r. enAGWr:ub nig GaemrikxageCIg/ esvabBa©ÚlQaml¥ kmμviFIcak;fñaMbgáar karRtYtBinitüQam)anBIGñkeFVIGMeNayQam nig esvasuxPaBsaFarN³ manKuNPaBx<s; CaTUeTA)aneFVI[karcMlgeraKTaMgenH naeBlbc©úb,nñCakarkMr KWfa karbgáeraKeday HBV, HCV nig HIV RtUv)ankat;bnßyesÞIrEtdl; kMritsUnüenAkñúgRbeTsTaMgenH. b:uEnþenARbeTskMBugGPivDÆn_CaeRcIn kar bgáeraKenAEtekItman eRBaHesvabBa©ÚlQamminsUvmanKuNPaB esvaEfTaM suxPaBminl¥ FnFanenAmankMrit nig mankarBuHBarnwgGaTiPaBsuxaPi)al 103

epSg²eTot. CalT§plGñkCMgWtaLasesmuIkMritF¶n; enARbeTskMBugGPivDÆn_ enAEtmankarqøgeraKtamry³karbBa©ÚlQam. rlakeføImRbePT B nig¼b¤ C manlkçN³F¶n;F¶rEpñkKøInik cMeBaHGñkCMgWtaLasesmuIkMritF¶n; edaysar rlakeføImTaMgenH GaceFVI[kan;EtF¶n;F¶reLIgcMeBaHCMgWeføIm bNþalmkBI CatiEdkelIs EdlmanCaTUeTAkñúgCMgWtaLasesmuI bNþalmkBICMgWxøÜn ÉgpÞal;Etmþg b¤ mkBIkarBüa)alkMcat;CatiEdkmin)anl¥. CMgWeføImCabuBVehtu rYménGRtamanCMgW nig søab; CaBiesskñúgcMeNamGñkCMgWtaLasesmuI Edl manv½ycMNas; enAbNþaRbeTs]sSah_kmμ nig GñkCMgWesÞIrEtRKb;v½y enARbeTskMBugGPivDÆn_. karbgáeraKedayvIrusrlakeføImRbePT B (Hepatitis B = HBV) rlakeføImraMuér:RbePT B enAEtCabBaðasuxPaBsaFarN³ F¶n;F¶renARbeTs kMBugGPivDÆn_CaeRcIn eTaHbImanfñaMbgáarmansuvtßiPaB nig RbsiT§iPaBk¾eday RBmTaMgetsþRbkbedayKuNPaBx<s;Cak;lak;manlk;enATIpSaredIm,I RtYtBinitüQamGMeNayk¾eday.

104

karcMlgeraK eRkABItamry³karbBa©ÚlQam/ vIrusrlakeføImRbePT B Gacqøgtam pøÚvepSg²eTot dUcCa karrYmePT/ qøgBImþayeTAkUnkñúgGMLúgeBl manépÞeBaH enAeBlTarkekIt b¤ eBlbMe)AedaHTark. EtGñkCMgW taLasesmIukMritF¶n; vIrusrlakeføImRbePT B jwkjybMputqøg tamry³karbBa©ÚlQam. karcMlg HBV GackarBar)anedayGnuvtþn_eKalneya)aysmRsbeday ³ [i] eRCIserIs nig eFVIetsþGñkeFVIGMeNayQam [ii] cak;fñaMbgáar HBV nig kmμviFIsMGatemeraK [iii] karBarkarqøgBImþaymanCMgWeTATarkeTIbekIt savtarrbs; HBV RbmaN 5 - 10° énGñkCMgWEdlqøg HBV nwgmanemeraKenHGs;mYy CIvit )anesckþIfaBYkeKnwgkøayCaGñkpÞúk HBV raMuér:edaymaneRKaHfñak; xøaMgkøaGacekItCMgWeføIm. cMeBaHkumar/ ebIKμanviFankarN_karBareT 90° énkumar EdlekItBImþaymanemeraK nwgkøayCaGñkpÞúkvIrusrlakeføImGs; mYyCIvit. kñúgenaHmanRbmaN 25 - 30° manpÞúkvIrusrlakeføImraMuér: RbePT B ehIybnþicmþg²nwgekItCMgWeføIm. PaKryénGñkCMgWtaLasesmIu kMritF¶n;EdlmanCMgWrlakeføImRbePT B ehIyvivtþn_eTACaCMgWeføImF¶n;F¶r mankarERbRbYlBIRbeTsmYyeTARbeTsmYy GaRs½yedaykarGnuvtþn_ viFankarN_karBar dUcCa karcak;fñaMbgáar HBV, eRCIserIs nig sMraMgGñk pþl;Qam ehIysMxan;bMputenaH GaRs½yelIGRtaekIt HBV kñúgtMbn;. eyagtamTinñn½y)anecjpSay/ man 2 - 35° énGñkCMgWtaLasesmuI kMritF¶n;TUTaMgBiPBelak CaGñkpÞúkrlakeføImRbePT B ehIycenøaH 20 90° manPsþútagmnÞIrBiesaFn_bgðajfa manqøgvIrusrlakeføIm B kñúgeBlNa mYykñúgCIvitrbs;BYkeK ¬qøgeraKkñúgGtItkal¦ . enARbeTsGPivDÆn_ GñkCMgW mYyPaKFMEdlpÞúkCMgWrlakeføImRbePT B suT§swgEtmanv½ycMNas; KW)anqøg 105

Serological profile of chronic HBV infection

106

eraKmunkarbegáIt nig kareFVI[RbesIrénkarcak;fñaMbgáar nig eKalneya)ay eFVIetsþQam. b:uEnþenARbeTskMBugGPivDÆn_ GñkCMgWRKb;GayubnþqøgvIrus rlakeføIm. enAeBleKeFVIeraKvinicä½yfa CaCMgWtaLasesmuIkMritF¶n; munnwgcab;epþIm bBa©ÚlQam GñkCMgWRtUveFVIetsþBinitü HBV )anesckþIfa Kat;CaGñkpÞúk emeraKrlakeføImraMuér: b¤ knøgmkmanrlakeføImRbePT B. RbsinebIGñkCMgWKμanGVIeT eKRtUvEtcak;fñaMbgáar edayminKitBIGayueLIy. GñkCMgWtaLasesmuIkMritF¶n;TaMgGs; RtUveFVIetsþtamdan HBV erogral; qñaM. etsþTaMgenHrYmman Gg;TIEhSnelIépÞekasika ¬HBsAg¦/ GgÁbdibkç RbqaMg HBV ¬Anti-HBs¦/ Gg;TIEhSn e ¬eAg¦/ GgÁbdibkçRbqaMg eAg ¬anti-HBe¦ nig GgÁbdibkçRbqaMgnwgsñÚl ¬anti-HBc¦. ebIcaM)ac; eKGac eFVIetsþGñkpÞúk HBV raMuér: ¬emIlrUb 6a¦ [)anjwkjab;edIm,IvaytMél eBlevlad¾smRsbbMputsMrab;cab;epþImkarBüa)al. karelIsbnÞúkCatiEdk elIGñkCMgWtaLasesmuIkMritF¶n; ehIymanrlakeføImraMuér:RbePT B KWktþabEnßm mYyEdlrYmcMENkd¾xøaMgkøaeFVI[xUceføIm ehIykarBüa)alkMcat;CatiEdk d¾manRbsiT§iPaB CakarsMxan;xøaMgNas;. karBüa)alrlakeføImrauMér:RbePT B karBüa)alrlakeføImraMuér:RbePT B maneKaledAkat;bnßy nig lubbM)at; HBV ehIykarBarplvi)akBIkarrlakeføIm. kñúgbNþaqñaMcugeRkayenHkar Büa)alrlakeføImRbePT B mankarrIkcMerInKYr[kt;sMKal;. »sfRbqaMg vIrusEdleKeRbITUeTArYmman Alpha - recombinant interferon EdleK)an eRbIyUrmkehIy. Alpha - interferon CasarFatuKImImYymanlT§PaBEkERb RbB½n§karBar RtUv)anbgðajfaGacCYy[GñkCMgWrlakeføImskmμraMuér:Ca)an 25 - 40°. »sfRbqaMgvIrusEdleTIbnwgbegáItfμI KW Lamivudine. Lamivudine (EpivirTM - HBV, 3TC) eRbIEtÉg b¤ pÁúMKñaCamYy Interferon )aneFVI[karBüa)al HBV raMuér:manPaBRbesIrya:geRcIn eTaHbIeKKitfa

vamanKuNvibtþiedaykarekItnUvPaBsaMu kñúgcMnYnGñkCMgW 14 - 32° kñúgqñaM dMbUg ehIy 67° kñúg 4 qñaMeRkay . fñaMfμImYyeTot manlk;elITIpSarsMrab; karBüa)alrlakeføImraMuér:RbePT B ¬CHB¦ KWfñaMelb Adefovir dipivoxil EdlbgðajfamanRbsiT§iPaBdUc Lamivudine Edr kñúgkarkMcat;vIrus ehIyKμan RBYy)armÖBIPaBsaMu. fñaMl¥²epSgeTot dUcCa [i] Interferon EbbfμIman RbsiT§iPaBl¥CageKehAfa pegylated Interferon nig [ii] Entecavir. TaMgBIr RbePTenHkMBugsßitkñúgkarsikSaenAeLIysMrab;karBüa)alrlakeføImRbePT B. karsMerccitþeBlNaRtUvBüa)al nig CMerIsfñaM b¤ eRbIfñaMbBa©ÚlKña RtUveFVI eLIgedayRKUeBTüBüa)al edayshkarN_ya:gCitsñiT§CamYyGñkCMnajEpñk CMgWeføIm. ya:gNak¾eday CarYmebIFnFan nig lT§PaBGacrkfñaM)an kareRbI fñaMTaMgenHeTAtammKÁúeTsn_Edlmankaryl;RsbCaGnþrCati )aneFVI[RbesIr ya:gFMeFgcMeBaHkarBüa)alCMgWrlakeføImraMuér: ehIyCYykat;bnßyya:geRcIn nUveRKaHvivtþin_eTAkan;CMgWeføImF¶n;F¶r dUcCa RkineføIm ¬sþarvij)an nig sþarvijmin)an¦ nig mharIkeføIm. rlakeføImRbePT C (Hepatitis C = HCV) HCV naMeq<aHeTArkkarrlakraMuér:Gs;mYyCIvitCag 80° énGñkqøgCMgW. vamin dUcrlakeføImRbePT B eT/ HCV mingaycMlgtampøÚvepSgeRkABIkar bBa©ÚlQameLIy. enAminTan;manva:k;saMgmansuvtßiPaB nig RbsiT§iPaB RbqaMg HCV enAeLIyeT EteTaHya:gNak¾manetsþmnÞIrBiesaFn_ EdlGacdwgya:gc,as;BIvIruskñúgQamEdr. dUecñHmeFüa)ayEtmYyKt; manRbsiT§iPaBkarBarkarqøg HCV nig kat;bnßyGRtaCMgW KW RtUvFana[ )anfa QamEdlpþl;RtUv)aneFVIetsþedayRbugRby½tñ. eTaHbIya:gNa k¾eday pÞúyBIvIrusepSg² HCV manry³eBlsMgMyUr KwfaeBlvacUldl; kñúgQam vasMgMes¶óry³eBlyUr CYnkalminGacdwgedayeFVIetsþGgÁbdibkç ¬antibody tests¦ EdleKeRbITUeTAbMputenAtammCÄmNÐlpþl;QamPaK eRcIn. kñúgGMLúgeBlenaH/ QamRtUv)anbBa©Úl ehIyGñkCMgWk¾TTYlkarqøg. etsþrk HCV )anTTYlkarykcitþTukdak;ya:gxøaMgkñúgkic©RbwgERbgedIm,I

HCV virus Envelope

Nucleocapsid protein Single-stranded RNA

Approximatly 10,000 nucleotides 50-60mm diameter

107

Natural history of HCV infection

Global geographic distribution of HCV genotypes (Zein, 2000)

108

bnßyry³kalcMlgCMgW ¬eKehAmüa:geTotfa “ window phase “ b¤ immunosilent period¦ [)aneRcIntamEdl GaceFVI)an ehIybnßyCaGtibrimanUvkarqøg HCV tamry³Qam. savtarrbs; HCV ¬emIlrUb 6b¦ RbmaN 20° énGñkpÞúk HCV raMuér:nwgekItCMgW eføImFn;Rsal ¬Fibrosis¦ kñúgenH 20° Gac vivtþn_CaCMgWeføImF¶n;F¶rCag dUcCa RkineføIm nig mharIkeføIm. vivtþn_eTACaRkineføImrvag10qñaM RbmaN 10 - 20° énGñkCMgW rlakeføImraMuér:RbePT C. rlakeføImRbePT C CamUlehtucMbgcMeBaHbnSaM eføIm ¬bþÚrefIøm¦. man ehSNUTIb (Genotypes) TaMgGs; 6 RbePT ¬rUb 6c¦/ RbePTnImYy² mankarEbgEcktMbn;PUmisaRsþ nig sPaBKøInikxus²Kña. cenøaH 10 - 80° énGñkCMgW taLasesmuIkMritF¶n;TUTaMgBiPBelak manqøgvIrusrlak eføImRbePT C ehIyvaenHCamUlehtusMxan;bgá[manCMgW eføImraMuér:F¶n;F¶rcMeBaHGñkCMgWTaMgenH. dUcrlakeføImRbePT B Edr/ karelIs CatiEdkkñúgxøÜn GñkCMgWEdlmin)ankMcat;CatiEdk b¤ kMcat;EdrEtmin)anRtwmRtUv KWCaktþabEnßmEdlGacrYmcMENkxøaMgkøadl;karxUcxateføIm. dUecñHsMxan; GñkCMgWtaLasesmuIkMritF¶n;TaMgGs;EdlmanvIrus HCV RtUvTTYlkar Büa)alkMcat;CatiEdk[)anRtwmRtUv ehIyeFVIetsþmnÞIrBiesaFn_ya:g BiesstamdanrlakeføImRbePT C erogral; qñaM ¬emIlrUb 6d¦. karman HCV nig HBV rYmKña Edl]sSah_ekItmanelIGñkCMgWtaLasesmuI kMritF¶n;EdlqøgtamkarbBa©ÚlQam GacrYmcMENkya:gxøaMgdl;karvivtþn_eTA muxya:gelOneTArkCMgWeføImF¶n;F¶r.

karBüa)alrlakeføImraMuér:RbePT C ¬CHC¦ kalBIdMbUgeLIy karBüa)alrlakeføImraMuér:RbePT C rYmmankareRbI fñaMmYymuxRbePT recombinant α-Interferon fVIebIvaeqøIytbnwgkar Büa)almankMritTab ¬10 - 25°¦. Etya:gNak¾eday kñúgbNþa qñaMcugeRkayenHkarBüa)alrlakeføImRbePT C ruaMér:mankarvivtþn_ KYr[kt;sMKal;. bc©úb,nñkalenH karBüa)alEdleKENnaMrYmman recombinant α-Interferon rYmCamYyfñaM Ribavirin EdlCafñaMelb tammat;manKuNsm,tþiRbqaMgnwgvIrus. b:uEnþkarBüa)aleday Ribavirin bgáEbkRKab;QamRkhm eRBaHfñaMenHvaeFVI[mankarfycuHKYr[kt; sMKal;nUvFatupSMsMxan;mYyrbs;eKalikaQamRkhm KW ATP ¬Adenosine Triphosphate¦ EdlTTYlxusRtUvdl;karrs;ranmanCIvitrbs;eKalikaQam Rkhm. GñkCMgWtaLasesmuIkMritF¶n;EdleRbI Ribavirin GacnwgCYbRbTHkar EbkRKab;QamRkhmeRcIn ehIyGacRtUvkarbBa©ÚlQameRcInEfmeTot ¬ekInRbmaN 30°¦ EdlvanwgTamTarkarBüa)alkMcat;CatiEdkxøaMgeLIg EfmeTot edIm,IkMcat;CatiEdkEdlelIs. fμI²enH/ man Interferon RbePTfμI RbesIrCagmuneQμaHfa pegylated Interferon RtUv)aneKeRbIEtÉg b¤ pÁúMnwgfñaM Ribavirin EdleFVI[GRtaeqøIytbnwgkarBüa)al)anRbesIreRcIn ehIy)anpþl; CMerIsBüa)alepSgmYyeTotsMrab;GñkCMgWrlakeføImRbePT C ruaMér:. RbePT nig ry³eBlénkarBüa)alGaRs½yeTAelI ehSNUTIb énvIrusrlakeføIm RbePT C EdleKsÁal; kñgú enaH type-1 eKKitfa mankarlM)akBüa)alCageK. karBüa)alRtUvsMercedayQrelIkarRbwkSaCamYyGñkCMnajEpñkelahitviTüa. rebobrbbBüa)alfμI)anbegáInGRtaeCaKC½yBIkMritTabRtwmEt 10- 25° edayeRbIEt recombinant α-Interferon EtmYymux rhUtdl;kMritCag 60° cMeBaHRbePTeRkABI type-1 ehIyrhUtdl; 48° sMrab; type-1. karBüa)alRbqaMgnwgvIrus HBV nig HCV Gs;esah‘uyéføNas; ehIyCa Gkusl kareRbIR)as;fñaMTaMgenHkñúgRbeTskMBugGPivDÆn_CaeRcInenAmankMrit.

Serological course of HCV infection evoluting to chronicity; other antibody patterns are possible

Healthy Liver

Fibrosis

Cirrhosis

109

karRbugRby½tñTUeTAmYycMnYnTak;Tinnwgkarqøg HCV nig HBV KWRtUveCosvag kareRbIR)as;rYmKñanUvRcas;duseFμj/ LamekaBukmat; nig vtßúeRbIR)as;pÞal;xøÜn epSgeTotEdlGacmut)an. HBV gayqøgCag )ann½yfavagayqøg BImñak; eTAmñak;Cag HCV, b:uEnþkarcak;fñaMbgáarRbqaMgvIrus)anRtwmRtUv GaclubbM)at; eRKaHqøgesÞIrEtTaMgRsug. 6e

110

Human immunodeficiency virus (HIV)

vIrsu huIv (HIV) CaGtisxu mu R)aNtUcmYy ¬emIlrUb 6e¦ EdlbgáCaCMgW eGds_ (AIDS = Acquired Immunodeficiency Syndrome). HIV sßitkñúgRkumvIrusmYymaneQμaHfa “Retroviruses” EdlmanlkçN³ CIvsaRsþBiessEdlRKb;RKgviFIbegáIncMnYnkUnecArbs;va nig RKb;RKglkçN³ sm,tþirbs;vaenAxagkñúgekasikaénsarBagÁkayEdlvavayRbhar. eKalika QamsEdleKsÁal;faCa Lymphocytes (CD4) Caekasikamansar³sMxan; bMputrbs;sarBagÁkayEdlGacP¢ab;CamYyvIrusenH ¬emIlrUb 6f¦. vIrusenH cUleTAkñúgekasikaenH edayeRbIynþkarmYyEdlGnuBaØati[vakøayCaEpñkén DNA rbs; CD4 enaH ehIybegáItrUbragÁxøÜnvapÞal;CaRbcaMenAkñúgsarBagÁkay énbuKÁlEdlmanCMgW KW begáIncMnYnkUnecAenAkñúgekasika Lymphocytes rYc bMpøajekasikaenaHecaleTA ¬emIlrUb 6f¦. Lymphocytes edIrtYnaTIsMxan; enAkñúgRbB½n§karBarsarBagÁkay b:uEnþeBlNamankarQøanBaneday Retrovirus, BYk Lymphocytes nwg)at;bg;lT§PaBkarBarsarBagÁkay Tl;nwgkarbgáeraK. ebIKμankarBüa)aleT CMgWnwgFøak;kñúgsPaBF¶n;F¶rkñúg ry³eBlRbEhl 7 - 11qñaM eTaHCaeraKsBaØaKøInik nig PaBF¶n;F¶rGacekIt qab;CagenHk¾eday ehIyKμankarkMNt;eBlc,as;las; sMrab;karcab;epþIm énsPaBCMgWF¶n;F¶reLIy. kñúgdMNak;kaldMbUg BMumanbgðajsBaØaKøInik b¤ sBaØamnÞIrBiesaFn_eT ¬eKehAfa KμaneraKsBaØa¦. eBleraKsBaØaekItman b¤ lT§plmnÞIrBiesaFn_ enaHbBa¢ak;famankarxUcxatF¶n;F¶rcMeBaHekasika ehIy karlUtlas;rbs;vIrusmankMritelOn mann½yfaCMgW)anQaneTAdl;dMNak;

kalkMritF¶n; ehAfa CMgWeGds_. mUlehtusMxan;bNþal[søab; cMeBaHGñkCMgWeGds_ Edlmin)anTTYlkar Büa)al KWkarbgáeraKF¶n;F¶r eBlEdlRbB½n§karBarKμanRbsiT§iPaB. esÞIrEt RKb;Pñak;garbgáCMgWNak¾eday nwgbgá[mankarbgáeraKF¶n;F¶r rhUtdl;)at;bg; CIvit cMeBaHGñkCMgWeGds_Edlmin)anBüa)al kñúgenaH Pneumocystic carinii KWCabuBVehtujwkjybMputbNþal[søab;. HIV qøgBIbuKÁlmñak;eTAbuKÁlmñak;tamry³Qam/ karrYmePTKμaneRbIeRsam b¤ BImþaymanCMgWeTAkUnEdleKehAfa Vertical route of transmission. dUckrNI HBV nig HCV Edr/ karBarkarcMlg HIV tamry³Qam rYmman kareFVIetsþQamedaymnÞIrBiesaFn_ edayRbugRby½tñral;QamGMeNay ehIy esvapþl;QamRtUvEtmanKuNPaB. karcMlg HIV tamry³pøÚvepSgeTot Gackat;bnßy)anya:geRcInedayeCosvagkarrYmePTédKUreRcIn ehIyKμan eRsamGnam½ykarBar. dUckrNI HCV Edr/ minTan;manva:k;saMgman RbsiT§iPaB nig suvtßiPaBRbqaMg HIV enAeLIyeT. manfñaMCaeRcInRtUv)aneKykmkeRbItaMgBIqñaM 1996 CaBYk Anti-retrovirals ¬fñaMRbqaMgnwgBYk retrovirus¦ Edl cUleTAeFVIskmμPaBenAdMNak;kalepSg² ¬emIlrUb 6g¦ eBlEdlvIruscUl nig ecjBIekasika b¤ GMLúgeBlvIrusva 6g

Life cycle of HIV showing opportunities for drug intervention

111

eFVIkarbMEbkxøÜnenAkñúgekasikaEdlvavayRbhar. edayeRbIbBa©ÚlKñanUvfñaM BIrmux/ bImux b¤ eRcInCagenH GnuelameTAtammKÁúeTsn_GnþrCati EdlGñk CMnajkñúgvis½yenHeFVIkaresIerIfμICanic©/ fñaMTaMgenH)aneFVI[mankarpøas;bþÚr muxmat;RbvtþiedImrbs;CMgW. cab;taMgBIfñaMTaMgenHRtUv)andak;[eRbImk/ TaMgkar rs;ranmanCIvit nig KuNPaBCIvitrbs;GñkCMgW HIV manPaBRbesIrya:g FMeFg. Cag 80° énkrNIqøgBImþaymanCMgW eTATarkkñúgépÞ b¤ eTAkUnekIt mkehIy RtUv)ankarBaredayeCaKC½yedayeRbI Anti-retrovirals pgEdr. ya:gNak¾eday dUckñúgkrNIBüa)al HCV nig HBV Edr fñaMeRbIkñúg karBüa)al HIV mantMéléføxøaMgNas;Edr. GñkCMgWmYyPaKFMrs;enARbeTs RkIRk eTaHbImankarxitxMRbwgERbgya:gNa k¾fñaMdUcenHenAmankMNt; ehIyCa erOy²mantMéléføminGacTijeRbI)an. rhUtmkTl;eBlenH eTaHbITinñn½y ecjpSaybBa¢ak;BIGRtaTabénkarqøg HIV kñúgcMeNamGñkCMgWtaLasesmuI kMritF¶n;k¾eday k¾tYelxTMngCamankarekIneLIgx<s; eBlEdleKTTYl)an Tinñn½yBIRbeTsCaeRcIncUlKña. manGtisuxumR)aNepSg²CaeRcIneTotEdr EdlGacqøgtamry³Qam eTaHCa vaminGacbgáeraKraMuér:cMeBaHGñkCMgWtaLasesmIukMritF¶n; b¤ GñkCMgWkgVHRKab; QamRkhmedaykarEbkRKab;Qam CaBiessGñkEdlmanRbB½n§karBar cuHexSayxøaMgkøa ]TahrN_ eRkayBIkarvHkat;pSaMsrIragÁk¾eday k¾Gtisuxum R)aNTaMgenHGacbgákarlM)akEpñkKøInikEdr. vIrusepSg²eTot Human Parvovirus B-19

CavIrusepSgmYyeTotEdlGacqøg tamry³Qam ¬eTaHCaQamminEmnCapøÚvcMlgsMxan;k¾eday¦. \T§iBlb:H Bal;sMxan;bMputrbs;vIrusenHeTAelIGñkCMgWtaLasesmuI b¤ CMgWkgVHRKab;Qam Rkhm edaykarEbkRKab;QamepSgeTotdUcCaCMgW Sickle cell KWfavaGac bgáeGayp¥akkarplitRKab;QamRkhmmYyry³ EdleKehAfa vibtþi Human Parvovirus B-19 (HPV-B-19)

112

beNþaHGasnñénkarplitRKab;Qam ¬Transient Aplastic Crises¦. dMNak;kalRsYcRsavbgáeday HPV B-19 sMKal;edaykarFøak;cuH eGm:UkøÚb‘Ín (Hb) Pøam² ehIynwg)at;bg; eKalikaQamRkhmedIm enAtamsésQam KW)at;BYk Reticulocytes enHÉg. kñúgkrNIeKeFVIeraKvinicä½yTan;eBlevla eKEtgBüa)alGñkCMgW edaykarbBa©ÚlQamRss; EdlmanpÞúkGgÁbdibkç RKb;RKan;sMrab;RbyuT§nwgvIrus. b:uEnþGñkCMgWEdlexSayRbB½n§karBar dUcCaGñk CMgWEdlpSaMxYrq¥wg b¤ GñkCMgWmanpÞúkvIrus HIV CaedIm eBlEdlqøgvIrus HPV B-19 GacekItmansPaBF¶n;F¶r CYnkalmanplvi)akEpñkKøInikraMuér:. Human Cytomegalovirus

vIrusmYyEdlecaTeRKaHfñak;FMeFgcMeBaHGñkCMgWexSayRbB½n§karBarKW Human Cytomegalovirus (CMV). vIrusenHbgáCMgWF¶n;F¶rcMeBaHGñkCMgWpSaMsrIragÁ ehIyrEmg[)at;bg;CIvitebIeFVIeraKvinicä½y nig Büa)alminTan;eBlevla. lkçN³sMxan;rbs;vIrusenH KwvamanlT§PaBP¢ab;xøÜnvaCaGciéRnþy_kñúgekasika EdlvaQøanBan ¬sMxan;KWeKalikaQams¦ edayeFVI[eKalikas BuHkeRBa¢al eLIgedayminGacdwgmun)an ehIybgá[manCMgWeBlNamYy b¤ CMgWsacuH saeLIgF¶n;F¶r. dUecñHsMxan;GñkCMgWtaLasesmuIkMritF¶n; CaBiessGñkEdl eRtomeFVIkarbNþúHxYrq¥wg b¤ GñkEdlkMBugdMeNIrkarbNþúHxYrq¥wg RtUvbBa©ÚlQam Edl)aneFVIcMeraHEdleK)anykecjnUveKalikaQams nig Pñak;garbgáCMgW epSg²eTotEdlmanCab;CamYyva rYmman CMV CaedIm. CMgWRKuncaj nig CMgW Chagas CMgWRKuncaj;eRkaykarbBa©ÚlQam nig CMgW Chagas ¬CaCMgWbgáedayBYk ):ar:asIutmü:agrs;kñúgQameQμaH Trypanosoma¦ RtUv)aneKsÁal;Cag 50qñaMmkehIy. RbePT Plasmodium EdlCaPñak;garbgáCMgWRKuncaj; nig Trypanosoma cruzi EdlCaPñak;garbgáCMgW Chagas GacmanCIvitkñúg smasFatuQamkøaes nig rhUtdl;kñúgpøasμakk ya:gehacNas;k¾)an BIrs)aþrh_Edr. 113

CalT§pl/ eKRBYy)armÖxøaMgcMeBaHeTscrN_eTAkan;RbeTsEdlmanekItCMgW CaRbcaM ehIykMeNInGenþaRbevsn_ BIRbeTsekItCMgWeTARbeTsKμanekItCMgW GacbegáInkarcMlgCMgWRKuncaj; nig CMgW Chagas. GgÁkarsuxPaBBiPBelak/ RkumRbwkSaGWr:ub/ GaC£aFrsuxaPi)al nig esvapþl;QamCatirbs;shrdæ Gaemrik )anrYmKñadak;ecjnUvbNþasþg;da ¬niyam¦ kñúgeKalbMNgkarBarCMgW RKuncaj; nig CMgW Chagas eRkaykarbBa©ÚlQam rYmmankareFVIetsþQam EdlCaGMeNay. Pñak;garbgáeraKfμI ehAfa Pñak;garbgáeraKfμI CaPñak;garEdleKrkeXIjenAcenøaHqñaM 1995 98 rYmmanvIrusbgárlakeføImepSgeToteRkABI RbePT B nig C enaHKWrlak eføImRbePT G ¬HGV b¤ GBV-C¦/ SEN-V nig TTVirus. eTaHbIman Éksarc,as;las;bBa¢ak;BIkaeqøgvIrusTaMgenHtamry³karbBa©ÚlQamk¾eday k¾tYnaTIvIrusTaMgenHkñúgkarbgá[ekItCMgWeføIm enABMuTan;c,as;enAeLIy. karRBYy)armμN_fμI cMeBaHPñak;garbgáeraKcas; CMgW Creutzfeld-Jakob (CJD) nig TMrg;fμIrbs;va b¤ ExSRsLayva (vCJD) EdlCaTMrg;ekItelImnusSecjBI Bovine Spongiform Encephalitis (BSE) EdlmanenAelIstVeKaRkbI CaCMgWpþac;CIvitedayb:HBal;eTAelIRbB½n§RbsaT. CMgWenHbgáedayRbUetGuInbgáCMgWmü;:ag ¬Prion protein¦ RsedognwgvIrus Et minEmnCavIrusBitR)akdeLIy ehIyrcnasm<½n§rbs;vamankarlM)aknwgeFVI cMNat;fñak; edayeFobnwgPñak;garbgáeraKepSg²eTot ¬mIRkub/ )ak;etrI nig )a:ra:sIut¦. eRkamlkçxN½ÐxøHminc,as;las; eKdwgfasarBagÁkaymnusS)an bþÚrRbUetGuInbgáCMgWenaHeTACaPñak;gareRKaHfñak;mYy bgá[ekItCaCMgWsrés RbsaTpþac;Civitedayvivtþn_eTAmuxyWt². eTaHeK)ansÁal;CMgWenHCaeRcInqñaM mkehIyk¾eday EtsarsMxan;énCMgW CJD nig ExSRsLayrbs;va eTIbEtelc ecjeFøaeLIgenABak;kNþalTsvtSr_ 1990 eBlEdleKBinitüelIGñksøab; 114

ehIyeK)anrkeXIjCaeRcInkrNI BiessenAcRkPBGg;eKøs bnÞab;mkman enARbeTsepSg²eTotEdr EtkñúgcMnYntictYc. enAExFñÚ 2001 man 113 keNIenAcRkPBGg;eKøs/ 4 krNIenARbeTs)araMg nig 1 krNIenA saFarNrdæeGoLg; nig 1 krNIenAekaHhugkug. RKb;krNITaMgGs;énExSRsLayfμIrbs; CJD EdlmankñúgÉksarrhUtmkTl; eBlenH eKKitfabNþalmkBIGñkCMgW)anhUbplitplecjBIstVeKaRkbIman CMgW. dUcKñaEdr karcMlgeraKRtUv)aneKdwgfaekItmancMeBaHGñkCMgWEdl)an TTYlG½rm:UnmYycMnYn)anmkBImnusS ¬G½rm:UnlUtlas;¦ nig GñkEdleKbnSaM eRsamxYrk,al nig bnSaMRsTab;kBa©k;EPñk. eKmanr)aykarN_pgEdr sþIBIkar qøgtamry³sMPar³Edlmin)ansMGatl¥eRkaykarvHkat;EpñksrésRbsaT nig rebobftskmμPaBxYrk,al. BMumanr)aykarN_sþIBIkarqøgtamry³kar bBa©ÚlQameLIy. edaysarPaBF¶n;F¶rénCMgW nig vtþmanénRbUetGuInbgáCMgW kñúgCalikaTwkrég/ RbeTselakxaglicmYycMnYn CaBiesscRkPBGg;eKøs )ancMNayFnFanya:geRcInelIeKalneya)aykarBarkarqøgCMgWtamry³ Qam. eKalneya)aymYyénbNþaeKalneya)ayEdleKGnuvtþ KW kareFVI cMeraHQamCaskl KWeFVIcMeraHral;QamEdlRbmUl)an tamry³enaH eKalikaQamsmYyPaKFMbMput ¬Lymphocytes¦ RtUv)anykecj. suvtßiPaBQam ral;kic©RbwgERbgCaGnþrCati KWepþateTAelIkic©karBarkarcMlgPñak;garbgáeraK tamry³karbBa©ÚlQam. bNþaktþaEdlrYmcMENkdl;suvtßiPaBQamrYmman ³ [i] begáIteKalneya)ayEdlmaneKalbMNgFana[)ankareFVIGMeNay QamedayKμanbg;R)ak; elIeKalkarN_sμ½RKcitþ ehIyeTogTat; BIGñkeFVI GMeNayQam. [ii] esvapþl;QammanKuNPaBx<s; rYmmankareFVIetsþmnÞIrBiesaFn_Edl caM)ac;cMeBaHQamGMeNay edayrk HIV, HBV, HCV, Syphilis nig Pñak;garbgáeraKepSg²eTotEdlmankñúgtMbn;. 115

begáItviFankarN_suxaPaBsaFarN³ dUcCa karcak;fñaMbgáar nig CMhan epSg²kñúgkarsMGatemeraK. kñúgbNþaqñaMcugeRkayenH eTaHtMéléføya:gNak¾eday k¾bNþaRbeTs ]sSahkmμ)anykmkeRbIpgEdr nUvbec©kviTüaBiessEpñkmnÞIrBiesaFn_ DNA eKehAfa Nucleic Acid Testing (NAT) edIm,IBinitüQam Edleq<aHeTAkat; bnßyKYr[cab;GarmμN_EfmeTot nUveRKaHénkarcMlgvIrus EdlmanlkçN³ F¶n;F¶rEpñkKøInik. [iii]

karcMlg)ak;etrI eRkABIvIrus/ GtixumR)aNepSg²eTotdUcCa )ak;etrI nig )a:ra:sIut GacmanCIvit nig begáIncMnYnkñúgQamqøgeTAGñkCMgWtamkarbBa©ÚlQam. tamBiteTAGRta énkarqøgtamQamrbs;)ak;etrImanBI 50 - 250 dg x<s;CagvIrus. CaerOy² karqøgekIteLIgtamviFIsamBaØ ehIyGaceCosvag)anya:ggay. ]TahrN_ RbPBcMlgeRcInCageK KWkarxVHGnam½y KwfaEs,kGñkeFVIGMeNay QamsMGatmin)anl¥muncak;m¢úlbUmQam. eBlEdlm¢úlmutcUlkñúgEs,k va)annaM)ak;etrIBIépÞEs,kcUleTAkñúgQamEdleyIgRtUvbUmyk. bNþaCMhanfμI² edIm,I)ansuvtßiPaBQam kñúgkic©RbwgERbgmYy edIm,Ibnþkat;bnßylT§PaBcMlgeraKedayPñak;garbgá eraKRKb;RbePTTaMgGs;/ eK)anbegáItbec©kviTüafμI²kñúgeKalbMNgkMeTc ecalnUv DNA rbs;BYkPñak;garbgáeraK eBlNaeKbBa©ÚlvaeTAkñúgfg;Qam GMeNay. viFIEbbenHeKehAfa Pathogene inactivation. vaCaviFId¾skmμ dMbUgbMputedIm,IsuvtßiPaBQam edayvaeFVIeGayemeraKElgmanskmμPaB teTAeTot eTaHeK)andwg b¤ mindwgfamanemeraKenAkñúgfg;Qam b¤ Gt;. karRsavRCav nig karsikSaEpñkKøInikénbec©kviTüaenH )anmkdl;dMNak;kal cugeRkay ehIybec©kviTüamYykMBugRtUv)anyl;RBm ehIyecjlk;elITIpSar enAGWr:ub cMeBaHpøaEkt/ EtvtßúbMNgcugeRkay KWRtUveRbIbec©kviTüaenH cMeBaH 116

RKb;smasFatuepSg²eTotrbs;Qam rYmmaneKalikaQamRkhmpgEdr. suvtßiPaB nig manQameRbIR)as;enAbNþaRbeTskMBug GPivDÆn_ enARbeTskMBugGPivDÆn_CaeRcIn eRKaHfñak;bNþalmkBIkarbBa©ÚlQamman PaBFMeFgeRcInCagbNþaRbeTs]sSahkmμ. enAbNþaRbeTskMBugGPivDÆn_ bBaðasMxan;KwkareRbIR)as;QamxN³EdlKuNPaBesvapþl;Qam dUcCa eKalneya)ayCMerIsGñkpþl;Qam nig kmμviFIcak;fñaMbgáar minTan;)anQan dl;sþg;daEdleKcg)andUcenAGWr:ub nig shrdæGaemrik. karxVHxatEpñk hirBaØvtßú karRbECgKñaEpñkGaTiPaB nig GesßrPaBneya)ay TaMgGs;enH)an eFVI[yWtya:vdl;dMeNIrGnuvþn_sþg;dax<s;énsuvtßiPaBQam. eyagtamTinñn½y EdlRbmUl)anedayGgÁPaBEpñksuvtßiPaBQamrbs;GgÁkarsuxPaBBiPBelak )an[dwgfa 80° énRbCaBlrdæTUTaMgBiPBelak rs;enAkñúgRbeTskMBug GPivDÆn_ b:uEnþBYKeKTTYl)anmindl; 40° pgnUvkarpÁt;pÁg;QamsrubRbcaMqñaM rbs;BiPBelakEdlmandl; 75 lanfg;. RbEhlCa 16° b:ueNÑaHénkar pÁt;pÁg;Qam EdlRbmUl)anenAbNþaRbeTskMBugGPivDÆn_ )anmkBIGñkbric©ak eFVIedaysμ½Rkcitþ Kμanbg;luykak; xN³Edl 43° énQam min)aneFVIetsþ )aneBjeljeLIy. CalT§pl eKrMBwgfaenARbeTskMBugGPivDÆn_CaeRcIn ekItmankarcMlgeraK HIV, HBV nig HCV tamry³karbBa©ÚlQam ehIy kñúgenaHRbeTsmYycMnYnmankMritx<s;. GaC£aFr nig GgÁkarsuxPaBCati nig GnþrCatiTaMgGs; RtUvxitxMRbwgERbgeRcInEfmeTot edIm,IeFVI[suvtßiPaBQam kan;EtRbesIr nig manQameRbIR)as;RKb;RKan;kñúgRbeTsTaMgenaH.

117

segçbbBaðaEdlGacekItmancMeBaHGñkCMgwtaLasesmuIkMritF¶n; Edl min)anBüa)al srIragÁTTYlkarb:HBal;edaykarbBa©Úl QamminRKb;RKan; ERbRbYlTMrg;q¥wgrbs;mux ¬q¥wgfÁamelIrIke)a:g¦

srIragÁTTYlkarb:HBal;edaykarkMcat;CatiEdk minRKb;RKan; RkeBj Pituitary ¬b:HBal;karFMFat;/ srIragÁ ePT/ RkeBj Adrenal, RkeBj Thyroid¦ RkeBj Thyroid ¬kMr¦ RkeBj Parathyroid ¬Hypoparathyroidism [naMekIt Hypocalcemia¦ ebHdUg ¬exSayebHdUgb:HBal;xøaMgCageK¦ eføIm ¬rIkeføIm¦

Hypersplenism (Thrombocytopenia) Pancreas

Qam ¬kgVHRKab;QamRkhm¦

¬TwkenamEp¥m¦

¬Es,k sPaBeLIgRbepH enAcugRkck/ Ekgéd/ CgÁg;/ EPñkeKal¦ Pigmentation

Rbdab;ePT ¬mankarlM)akkñúgkarrIkFMFat; nig dMeNIrkar¦

)ak;q¥wg q¥wg femoral xageRkambiTmunkalkMNt; Epiphyse

bNþalmkBIkarrIxYrq¥wg

118

Bukq¥wg/ QWq¥wg/ QWsnøak; ¬Tak;Tgnwg Hypoparathyroidism ¦

CMBUkTI 7 taLasesmIukMritmFüm nig taLasesmIuepSg²eTot taLasesmIukMritmFüm CasPaBevC¢saRsþmYyEdlGñkCMgW)anTTYltBUCBI mþay nig «BuknUv Bn§úEbtta (B - gene) EdlmanCMgW ¬)ann½yfaGñkCMgWCa GUm:UhSIúháÚt cMeBaH Ebtta taLasesmIu¦ b:uEnþGñkCMgWTaMgenHmaneraKsBaØa KøInikRsalCagtaLasesmIukMritF¶n;. GñkCMgWtaLasesmIukMritmFüm varkSa )ankMrit eGm:UkøÚb‘Ín kñúgQamenAcenøaH 6 - 9 Rkam¼d>l (g/dl) ehIyGacmin caM)ac;bBa©ÚlQamCaeTogTat;eLIy. ya:gNak¾eday karRsavRCavCaeRcIn)anbgðajfa taLasesmIukMritmFüm mansBaØaKøInikeRcInya:g kñúgenaH sBaØaxøHGacmanlkçN³F¶n;F¶r. kñúgkrNI F¶n;F¶rbMput GacbgðajsBaØaKøInik nig sBaØamnÞIrBiesaFn_enAGayuBI 2 - 6 qñaM fVIebIkarrIkFMFat; nig karlUtlas;ragÁkaymansPaByWtCagFmμtak¾eday k¾GñkCMgWGacrkSa)annUvKuNPaBCIvit edaymincaM)ac;bBa©ÚlQameTogTat;dUc krNItaLasesmIukMritF¶n;eLIy. kñúgkrNIminsUvF¶n;F¶r GñkCMgWminbgðajeraK sBaØaNamYyeLIy rhUtdl;eBjv½y KWRKan;EtmansPaBxVHRKab;QamRkhm bnþicbnþÜcb:ueNÑaH ¬8 - 10 Rkam¼d>l¦ ehIykMrnwgbBa©ÚlQamebIKμan bBaðaGVIenaHeT. GeNþIkGacmanTMhMFM ¬Splenomegaly¦ dUckrNICMgWtaLasesmIukMritF¶n;Edr edaysareKalikaQamRkhmEbkelOn ehIykkCujenAkñúgGeNþIk ehIyCYn kalbgá[sPaBkgVHRKab;QamRkhmkan;EtF¶n;F¶rcMeBaHCMgWtaLasesmIu kMritmFüm. krNIEbbenH GñkCMgWGacnwgRtUvbBa©ÚlQamya:geTogTat;. karkat;GeNþIkecj GacEkkun)annUvplvi)akenH Etya:gNak¾edaykarsMerc citþeFVIdUcenHCakarF¶n;F¶r EdlRtUvmankarcUlrYmpþl;mtieyabl;BIGñkCMnaj edayBicarNaeTAelIplb:HBal;EdlGacekItmaneLIg dUcCalT§PaBénkar 119

bgáeraK EdlCaRCugepSgeToténsuxPaBGñkCMgW eRkABIkarCYyseRgÁaHsPaB xVHRKab;QamRkhm. dUc)anelIkeLIgkñúgCMBUkTI2/ buBVehtucMbgEdlbgá[maneraKsBaØakñúgCMgW taLasesmIukMritF¶n; KWedaysarkarkkCujkñúgeKalikaQamRkhmnUv ExSGal;hVa (a) esrI begáIt[manGtulüPaBrvag ExSGal;hVa nig ExSRbUetGIunEdlFmμtaCaédKUrrbs;va KW ExSEbtta (β). edayvaÉkeka EtÉg/ ExSGal;hVa )ancUleTArMxan esÞIrEtRKb;dMNak;kalén vdþeBjv½y ¬Cycle of maturation¦ rbs;eKalikaQamRkhm bgáeGaymansPaBxVH RKab;QamRkhmF¶n;F¶r nig sBaØaepSg²eTot dUc)anCMrabxagedIm. dUckarGHGagxagelIenH/ manehtupl[eyIgrMBwgfa eraKsBaØarbs; taLasesmIunwgminsUvF¶n;F¶r kalNa ExSGal;hVa EdlelIsRtUv)anbnßy mkvij. karGegáteTAelIm:UelKul)anbgðajfa lkçx½NÐmYycMnYnEdlExS Gal;hVacuHfy mandUcCa ³ [i] vtþman Bn§úEbtta (β+ gene) EdlGacplit ExSEbtta xøHEdr eTaHCa plit)anticCagFmμta/ ExSEbtta enHnwgeTAcab;KUrCamYy ExSGal;hVa dUcenHva)anbnßy ExSGal;hVaesrI EdlKμanKUr. karpøas;bþÚr (Mutations) cMeBaH Bn§úEbtta (β+ gene) EdlnaM[ecjCalT§pl KøInik minsUvF¶n;F¶rxøaMg CYnkalenaH eKRtUvdak;nam[fa β++. [ii] karxUcxatcMeBaHBn§ú EdlTTYlxusRtUvdl;karplit ExSGal;hVa eFVI[ ExSGal;hVamYycMnYnRtUv)at;bg; rYck¾eFVI[RbesIreLIgvijnUvtulüPaB rvag ExSGal;hVa nig ExSEbtta. [iii] Bn§úháama: ¬γ - genes¦ begáInskmμPaBCagmun edaybegáInkarplit ExSháama: ¬γ - chains¦ EdlGaceTAP¢ab;CamYYy ExSGal;hVa edIm,I begáItCa eGm:UkøÚb‘ÍnTark [Foetal haemoglobin (a2γ2)] dUcenHvaCYy bnßy ExSGal;hVaesrI EdlmaneRKaHfñak;. sßanPaBEdlGacCYydl; karplit ExSháama: man ³ CMgW β- taLasesmIu edayman eGm:UkøÚb‘Ín 120

TarkCaGciéRnþy_ nigkarpøas;bþÚrkEnøgNamYyCayfaehtuenA Bn§úháama: EdleKehAfa Xmnl. dUc)anbgðajxagelIenH RKUeBTüGacelIkkMBs;cMeNHdwgrbs;eKcMeBaHsPaB GñkCMgW tamkaryl;nUvEbbEpnxUcxatBitR)akdcMeBaH DNA rbs;GñkCMgW . dUecñHbnÞab;mkeTot vakan;Etgay[eyIgdak;ecj)annUvkmμviFIBüa)al smRsbsMrab;GñkCMgWmñak;². ebIGaceFVIeTA)an viFIBinitüm:UelKulEbbenH kMBugEtbgðajnUvCMnYyminGackat;éfø)an cMeBaHkarBüa)alCMgWtaLasesmIu. kareFVIeraKvinicä½y kñúgkareFVIeraKvinicä½yCMgWtaLasesmIukMritmFüm sMxan;RtUvrk[)annUvBt’man KøInik nig mnÞIrBiesaFn_mYycMnYnedIm,I[eyIgEjk[)anBItaLasesmIu kMritF¶n;. b:uEnþenHminEmnecHEtgayRsYlenaHeT b¤ eFVI)anRKb;eBlenaHeT eTaHbImankarrIkcMerInEpñkbec©keTsmnÞIrBiesaFn_xagm:UelKulk¾eday. eTaHya:gNa cMNucsamBaØ nig manGtßRbeyaCn_sMrab;eFVIkarEbgEck eraKvinicä½ymandUcteTA ¬tarag 7a¦ ³

121

eraKsBaØabgðajCMgWtaLasesmIu sBaØaKøInik GeNþIkrIkFM xan;elOg bMErbMrYlq¥wg bMErbMrYlmux

kMritF¶n; kMritmFüm kMritRsal cab;epþImBIkumarPaB cab;epþImeBleRkay KμaneraKsBaØa ++++

+++ - ++++

0-+

+++

+ - +++

0-+

++++

++ - ++++

0 - ++++

sBaØaEpñkelahitsaRsþ PaBesøksøaMg eKalikaQamRkhm

++++

++ - +++

0-+

N -

Microcytosis

NRBC

++ - ++++

+ - ++++

10 – 95 + %

sBaØaEpñkCIvKImIsaRsþ HbF HbA2

b¤

N N

b¤ < 10%

b¤ (> 3,5%)

(Proceding from a conference, MA Nov. 14, 1996

CasnñidæanmkBaküfa taLasesmIukMritmFüm KWeKeRbIedIm,Ierobrab;eraKsBaØa KøInik nig EpñkelahitsaRsþ cMeBaHGñkCMgWEdlmanTMrg;RsalCag Ebtta taLasesmIu eGetr:UhSIúháÚt (Heterozygous b - Thalassemia) b:uEnþva F¶n;F¶rCagTMrg; taLasesmIu eGetr:UhSIúháÚt. 122

karBüa)al taLasesmIukMritmFüm kñúgCMgWtaLasesmIukMritmFüm/ sMnYrsMxan;bMputKWfaetIeBlNaEdlRtUvcab; epþImBüa)aledaybBa©ÚlQam. sßanPaBevC¢saRsþmYycMnYn dUcxageRkam enHGacbNþalmkBIkgVHRKab;QamRkhmraMuér: ehIyCaehtuplBitR)akd sMrab;eKcab;epþImBüa)aledaykarbBa©ÚlQam ³ karrIkFMFat;manPaByWtya:v q¥wggaynwg)ak; plvi)akcMeBaHebHdUg ERbRbYlTMrg;q¥wgrbs;mux skmμPaBragÁkayFmμtafycuH GeNþIkskmμrIkFMxøaMg dUckñúgkrNICMgWtaLasesmIukMritF¶n;Edr/ sMxan;eKRtUvtamdanGñkCMgW[)an eTogTat;tamry³karBinitüevC¢saRsþ nig mnÞIrBiesaFn_ kñúgeKalbMNgqab; )andwgTan;eBlevla kalNamanplvi)akNamYyEdlekIteLIg. elIsBIenH edaysarGñkCMgWtaLasesmIukMritmFümcab;epþImkarbBa©ÚlQamenAeBl EdlmanGayueRcInbnþic mindUckrNICMgWtaLasesmIukMritF¶n; dUcenHsMxan; RtUvykcitþTukdak;eTAelIRbtikmμEdlGacekItmanCayfaehtu ¬Rbtikmμ Alloimmunisation¦ dUc)anerobrab;BIxagedIm edayRbtikmμEbbdUcenHEtg ekItmaneLIgeBlbBa©ÚlQamcab;epþImenAGayuFM. dUcenH/ sMxan;Qamrbs; GñkCMgW nig Qamrbs;Gñkpþl; RtUvEtRkumQamdUcKña nig pÁÚrpÁg[sIuKña ral;eBlmuneFVIkarbBa©ÚlQammþg². vasMxan;pgEdr edaykt;sMKal;fa RsþICaGñkCMgWtaLasesmIukMritmFüm ehIymanépÞeBaH GacRtUvkarbBa©Úl QamEdr.

123

karkMcat;CatiEdk dUckñúgkrNICMgWtaLasesmIuF¶n;F¶rEdr/ CatiEdkelIskñúgCMgWtaLasesmIu kMritmFüm GacbNþalmkBI ³ [i] karpliteKalikaQamRkhmKμanRbsiT§iPaB [ii] karEbkeKalikaQamRkhm [iii] brimaNEdkRsUbBIeBaHevonekIneLIg mankarsikSaeRbobeFobmYycMnYnb:ueNþaHGMBIkarkkpþúMCatiEdk cMeBaHGñkCMgW taLasesmIukMritmFüm. b:uEnþkarsikSamYy)anbgðajfa Caerogral;qñaMman CatiEdkkkpþúMkñúgxøÜnGñkCMgWtaLasesmIukMritmFüm 2 - 5Rkam KWesμI 0/1 mRk¼KRk¼éf¶. )anesckþIfa CatiEdkRtUv)anRsUbBIcMNIGaharx<s;CagFmμta 20 - 70°. dUecñH kñúgkrNIPaKeRcIn eRkayBIGayuCag 10 qñaMeTA/ GñkCMgW taLasesmIukMritmFümgaynwgmanplvi)akbNþalmkBIEdkswgEtdUcKña nwgGñkCMgWtaLasesmIukMritF¶n; Edl)anTTYlkarbBa©ÚlQamCaeTogTat;. karlM)akkñúgkarsMercfa eBlNaRtUvcab;epþImkMcat;CatiEdkBIkñúgxøÜnCMgW taLasesmIukMritmFüm KWkMNt;edaysPaBelIsCatiEdkBitR)akdkñúgxøÜn GñkCMgW eRBaHfakMrit Ferritin kñúgQam minGacpþl;rgVas;rgVal;BitR)akdeLIy EdlvadUcKñanwgkrNICMgWtaLasesmIukMritF¶n;Edr. ehtuenHkarENnaM[eFVIkar vaytMélkMhab;CatiEdktammeFüa)ayRcwbsac;eføIm ¬liver biopsy¦ b¤ tamry³viFIfμIEdlgaydwg ehIymin[GñkCMgWQWcab; dUcCa SQUID b¤ MRI. eBlEdlsMerccitþfa cab;epþImkarBüa)alkMcat;CatiEdk/ eKENnaM[eRbIfñaM DFO dUckñúgkrNICMgWtaLasesmIukMritF¶n;Edr. EtcMeBaHGñkCMgWtaLasesmIu kMritmFümeKKYreRbIkarcak;eRkamEs,kmin[elIs 2 b¤ 3éf¶kñúgmYys)aþh_. eKENnaM[tamdankarBüa)alkMcat;CatiEdkcMeBaHGñkCMgWtaLasesmIukMrit mFüm eGaydUcKñanwgkrNIGñkCMgWtaLasesmIukMritF¶n;Edr. 124

edayGñkCMgWtaLasesmIukMritmFümRsUbykCatiEdkBIeBaHevoneRcInCag Fmμta GñkCMgWRtUveCosvagcMNIGaharNaEdlsMbUrCatiEdk ¬]TahrN_ és<exμA eføImstV nig RbePTsENþkmYycMnYn¦ RBmTaMgkarelbCatiEdk bEnßm. pwkTwkEtexμACamYycMNIGahar GacCYykat;bnßybrimaNCatiEdk RsUbtameBaHevonEdr. bBaðaEpñkevC¢saRsþkñúgCMgWtaLasesmIukMritmFüm [1] bMErbMrYlqw¥wg> xYrqw¥wgskmμxøaMg bNþalmkBIsarBagÁkayxitxMplit RKab;QamRkhm edIm,ITb;Tl;nwgsPaB kgVHRKab;QamRkhm eFVI[q¥wg mansPaBrmYlxusTMrg;edIm puyehIyesþIg rMxandl;karrIklUtlas; ehIyeFVI[GñkCMgWgayrgkar)ak;q¥wg. b:uEnþbBaðacMeBaHq¥wgF¶n;F¶rGacCMnH )antamry³karBüa)aledaybBa©ÚlQameGay)aneTogTat;. PaBBukq¥wg (Osteoporosis)> GñkCMgWRtUveKelIkTwkcitþ[hat;R)aN nigbegáInCatikalsüÚb Calcium kñúgcMNIGahar edIm,IeCosvagCMgWq¥wg F¶n;F¶r ¬PaBBukq¥wg¦. RKab;fñaM Calcium nig vItamIn ed (Vitamin D) Gacpþl;KuNRbeyaCn_bEnßm. RtUveCosvagkarCk;)arI evC¢bNÐitxøH)an bgðajBIGtßRbeyaCn_BIkareRbI Biophosphonates elbtammat; b¤ cak;bBa©ÚltamsrésQam/ b:uEnþtYnaTIrbs;vakñúgkarRbyuT§nwgPaBBukq¥wg BMuTan;RtUv)aneKbBa¢ak;enAeLIy. [2] karmanskmμPaBxøaMg b¤ karrIkFMénxYrqw¥wg nig hVÚlikGasIut (Folic acid)> edaysarxYrq¥wgGñkCMgWtaLasesmIukMritmFüm RbwgeFVIkarxøaMg edIm,IRbyuT§nwgPaBkgVHRKab;Qamrbs;sarBagÁkay edayxMplit eKalikaQamRkhm[)aneRcIn/ GñkCMgWRtUvkarbEnßmbrimaNvItamIn mYycMnYn CaBiess hVÚlikGasIut. kgVH hVÚlikGasIut GaceFVI[PaB xVHeKalikaQamRkhmkan;EtF¶n;F¶rcMeBaHGñkCMgWtaLasesmIukMrit mFüm. hVÚlikGasIut eKeXIjmankñúgFmμCati dUcCa sac;/ bEnøébtg. 125

[3]

[4]

[5]

126

b:uEnþbrimaNbEnßm Kwelb 1RKab;¼éf¶ vaGacbMeBjesckþIRtUvkarbEnßm rbs;GñkCMgW . RKYsRbmat;> GñkCMgWtaLasesmIukMritmFümekItRKYsRbmat; ¬Cholelithiasis¦jwkjyeRcInCagFmμta. RKYsRbmat; ekItecjBI plitplsMNl; ¬Bile pigment = sarFatuBN’TwkRbmat;¦ Edl)an mkeBleKalikaQamRkhmEbk/ ehIysarFatuenHeTAkkpþMúkñúgfg;Twk Rbmat;EdlGacbgá[sÞHfg;TwkRbmat; ehIybNþal[cukeBaH. vtþman RKYskñúgfg;TwkRbmat;GacbBa¢ak;)anedayBinitü eGkU ¬Ultrasound¦. ebIcukeBaHenaHCab;rhYtmin)at; eKGackat;fg;TwkRbmat;enaHecal. dMe)AeCIg> GñkCMgWtaLasesmIukMritmFüm CajwkjyekItdMe)ACMuvij keCIg CaBiessGñkCMgWv½ycMNas; bNþalmkBIcrnþQamnaM GuksIuEhSn min)anl¥eTAEp¥kxøHénsarBagÁkay. dMe)AenHmingayCasH ehIyBi)ak Büa)alNas;. b:uEnþkarbBa©ÚlQameTogTat;edIm,IbegáInkMrit eGm:UkøÚb‘Ín (Hemoglobin) nwgCYYypÁt;pÁg; GuksIuEhSn [)anRbesIreTACalikadMe)A enaH RBmTaMgviFankarsamBaØ²dUcCa edkelIkeCIg nig RbGb;eCIg[x<s; CagebHdUg[)an 1 - 2ema:g kñúgmYyéf¶ ehIyBak;eRsameCIgkarBar keCIg TaMgenHGacCYysMral)anxøH. fñaMxøH dUcCa Zinc sulphate RbePT RKab; CYnkalGacCYy)anEdr/ RBmTaMgfñaM Hydroxyurea eRbIEtÉg b¤ pÁúMcUlKñaCamYyfñaMepSgeTot GacbegáInkMrit HbF dUcCa Erythropoietin nig Butyrates. plvi)akeTAelItMrgenam> bBaðecaTEpñkevC¢saRsþepSg²eTot EdleK raykarN_mkfaekItmankñúgcMeNamGñkCMgWtaLasesmIukMritmFümKwkar xUcxatcMeBaHtMrgenam EdlGacbNþalmkBIkarelIsCati Uric acid kñúgQam. Uric acid KWCakaksMNl;sMxan;bMputbNþalmkBIxYeq¥wg skmμxøaMg. fñaM Allopurinol GacCYykat;bnßybrimaN Uric acid EdlRtUv)anplitelIs.

[6]

[7]

[8]

kMeNInpøaEkt ¬Thrombophilia¦> plvi)akepSgeTotKW eRKaHekIt kMNkelIPñassrésQam ¬Thrombosis¦ bNþalmkBI pøaEkt ¬sUmemIlEpñk Qam¦ kkCujkñúgsrésQambegáItCadMukMNk ¬Clots¦ EdleFVI[sÞHcrnþQamrt;Fmμta ehIybnßykarpþl;GuksIuEhSneTAciBa©wm ekasika nig Calika. karrab;cMnYnpøaEktCaeTogTat; GnuBaØati[ evC¢bNÐitkMNt;)anfaRtUveRbIfñaMRbqaMgnwgkMNkpøaEkt RbsinebIpøaEkt ekIn b¤ eRbIfñaMRbqMagnwgkMNkQam ¬Anti-coagulants¦ ebImanKMerag RtUveFVIkarvHkat; b¤ ekItman Thrombosis . karpliteKalikaQamRkhmenAeRkAxYrq¥wg> mindUcGñkCMgW taLasesmIukMritF¶n;EdlRtUvTTYlkarbBa©ÚlQamCaeTogTat;taMgBI Gayuekμgmkemø:HedIm,IbM)at;skmμPaBrbs;xYrq¥wgmin[xøaMgkøa/ GñkCMgW taLasesmIukMritmFümBMuTTYlkarbBa©ÚlQamCaeTogTat;dUcenHeT GBa©wgvabnþpliteKalikaQamRkhmkñúgkMritx<s; rYmTaMgkarplitenA kEnøgeRkAxYrq¥wg sMxan;enAtMbn;edImRTUg nig Ek,rq¥wgkgxñg. karft edaykaMrsμI Guic ¬X-rays¦ GacbgðajeGayeXIjCalikabegáItRKab; QamEdlekItCadMu²enAtamtMbn;TaMgenaH. karplitRKab;QamenAEk,rq¥wgkgxñg Gacbgá[manplvi)akdl;RbsaT EdlekItmaneLIgeBlNavasgát;BIxageRkA eTAelIxYrq¥wgkgxñg. skmμPaBEbbenHGacdwg)antamry³ftkaMrsμI Guic b¤ eRbIviFIgay[ eXIjc,as;dUcCa MRI. CafμImþgeTot/ sßanPaBEbbdUcenH rEmgRKb;RKg )antamry³karBüa)aledaybBa©ÚlQamEdlnwgbM)at;karbegáIt RKab;QamenAeRkAxYrq¥wg ehIyCalT§pl bnßy)annUvdMubegáItRKab;Qam TaMgenaH. krNIsßanPaBEpñkRbsaTmanlkçN³F¶n;F¶r eKRtUvkarviFan karxøaMgCagenH dUcCa karBüa)aleday viTüúsaRsþ ¬Radiotherapy¦. plvi)akeTAelIeføIm nig ebHdUg> kgVHeKalikaQamRkhmraMuér: k¾GacbgábBaðaeTAelIebHdUgEdr xN³eBlEdlebHdUg nig eføIm 127

GacTTYlrgplxUcxatBIkarelIsbnÞúkCatiEdk. sßanPaBTaMgBIrGac RKb;RKg)an dUckñúgkrNItaLasesmIukMritF¶n;Edr. taLasesmIubNþalmkBI {eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘Ín Edlmanrcnasm<½n§køay {eGm:UkøÚb‘ÍnxusFmμta (Abnormal hemoglobin)} manrcnasm<n§½ nig lkçN³mindUc eGm:UkøÚb‘ÍnFmμta eLIy. eGm:UkøÚb‘ÍnxusFmμta sMxan;²man ³ eGm:UkøÚb‘Ín S (HbS) eGm:UkøÚb‘Ín C (HbC) eGm:UkøÚb‘Ín E (HbE) eGm:UkøÚb‘Ín D (HbD) eGm:UkøÚb‘Ín Lepore eGm:UkøÚb‘Ín TaMgGs;enHcMlgtamtMNBUC dUcCMgW Ebtta taLasesmIu Edr. ]TahrN_ GñkTaMgLayNaEdlTTYltMNBUC Bn§ú ¬gene¦ mYykMNt;)an eGm:UkøÚb‘ÍnFmμta ehIyBn§úmYyeTotkMNt;)aneGm:UkøÚb‘ÍnxusFmμta S, C, E b¤ D GñkTaMgenaHRtUveKehAfa GñkpÞúk HbS, HbC, HbE b¤ HbD eTAtamenaH. GñkpÞúkTaMgenaH eKGacdwg)anedayeFVIetsþmnÞIrBiesaFn_ dUcKñanwgGñkpÞúk Ebtta taLasesmIu Edr. lkçN³GUm:UhSIúháÚt rbs;eGm:UkøÚb‘ÍnxusFmμta buKÁlTaMgLayGacTTYltMNBUC eGm:UkøÚb‘ÍnxusFmμta ¬S, C, E b¤ D¦ mYyBI nrNamñak;én «Buk b¤ mþay ehIynig eGm:UkøÚb‘ÍnxusFmμta ¬S, C, E b¤ D¦ mYyepSgeTotBImñak;epSgeTotén «Buk b¤ mþay. b:uEnþmanEtGñkEdlTTYl tMNBUC HbS BI«Bukpg nig BImþaypgeTEdlmansßanPaBKøInikKYr[kt; sMKal; eKehAfa GUm:UhSIúháÚt ¬Homozygote¦ edaysar HbS b¤ ehAfa CMgWkgVHeKalikaQamRkhm RbePT Sickle Cell. PaKeRcInénkrNI

128

GUm:UhSIúháÚt edaysar HbC, D b¤ E Kμanbgá[ekItCaCMgWeT ehIyKμansar³ sMxan;EpñkKøInikEdr. PaBpÁúMKñaén {eGm:UkøÚb‘ÍnxusFmμta} CamYy Ebtta taLasesmIu karpÁúMcUlKñaEdleKeXIjjwkjyCageKén Ebtta taLasesmIu CamYy eGm:UkøÚb‘ÍnxusFmμta man ³ HbS/ β – taLasesmIu HbE/ β – taLasesmIu HbS/β – taLasesmIu - vabgáCasßanPaBKøInikmYymanlkçN³Rsedognwg CMgW Sickle Cell CagCMgWtaLasesmIukMritF¶n; b¤ kMritmFüm. HbE/β – taLasesmIu - enHCasßanPaBEdlmaneRcInCageKbMputrbs; {eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘ÍnkøaypÁúMCamYyCMgWtaLasesmIu nig manGRtaekIteRcInCageKenAGasIuGaeKñy_. sPaBpÁúMcUlKñaEbbenH k¾bgðaj [eXIjpgEdrnUveraKsBaØaKøInik b¤ PaBF¶n;F¶r eRcInEbbeRcInya:g. tameraKsBaØaKøInik/ eKEck β – taLasesmIu /HbE Ca 3 kMrit eyageTA tamPaBF¶n;F¶réneraKsBaØa ³ β – taLasesmIu /HbE kMritRsal - eXIjman 15° énGñkTTYlrg karb:HBal;TaMgGs;enAtMbn;GasIuGeKñy_. RkumGñkCMgWEbbenHminekItman bBaðaKøInikeT ehIyrkSakMrit Hb rvag 9 - 12 Rkam¼d>l (g/dl). β – taLasesmIu /HbE kMritmFüm - GñkCMgWmYycMnYnFMkñúgRkumenH ekItmaneraKsBaØaKøInikRsedognwgGñkCMgW β – taLasesmIukMritmFümEdr ehIymankMrit Hb efr enAcenøaH 6 - 7 Rkam¼d>l. β – taLasesmIu /HbE kMritF¶n; - GñkCMgWkñúgRkumenHmaneraKsBaØa KøInikF¶n;F¶rdUcCMgWtaLasesmIukMritF¶n;Etmþg ehIymankMrit Hb cuHTabdl; 4 - 5 Rkam¼d>l. GñkCMgWTaMgenHRtUvBüa)aldUcGñkCMgWtaLasesmIu kMritF¶n;Edr.

> > >

129

karbBa©ÚlQam ³ GñkCMgWRbePTenHRtUvtamdan[)anditdl; kñúgGMLúgeBl bBa©ÚlQam edIm,IeCosvagplvi)akEpñkevC¢saRsþsMxan;² dUcCa eLIgsMBaF Qam/ Rbkac; nig hUrQamenAxYrk,al. edIm,IRKb;RKgkareLIgsMBaFQam EdlGacbNþal[søab;edaykarhUrQamenAxYrk,al eKGaceRbIfñaMTb;Tl; kareLIgsMBaFQam. GñkCMgWEdlKμankgVHRKab;QamRkhmF¶n;F¶reT eKmin RtUvbBa©ÚlQameLIy. Gal;hVa taLasesmIu Bn§ú rbs; Gal;hVakøÚb‘Ín (a-globin genes) rbs;mnusSmanBIrKUr manTItaMg sßitenAelIRkUm:UsUmelx 16. karfycuHén ExSGal;hVa (a-chains) EdlRtUv)ansMeyaK ekItmaneLIgkalNaPaKxøHrbs;ExS DNA EdlmanBn§ú rbs;Gal;hVakøÚb‘Ín mYy b¤ TaMgBIr RtUv)anrlb;)at;. - ebIrlb;Bn§ú cMnYnmYy/ GñkCMgWRtUveKcat;fa RbePT es¶oms¶at; b¤ CaGñkpÞúk aa/a- ebIrlb;Bn§ú cMnYnBIr/ eKcat;fa bgðjtMruy aa/-- b¤ a-/a- ebIrlb;Bn§ú cMnYnbI/ enaHeKcat;fa manCMgW Hb/H bNþalmkBImanExS RbUetGIunEbtta (B-chains) eRcInelIslb;. CaCMgWmYysMKal;edaykarEbk eKalikaQamRkhmkMritmFüm/ GeNþIkrIkFM ¬nig vibtþiEbkeKalika QamRkhmRsYcRsav eBleRbIfñaMmYycMnYn b¤ cak;fñaM¦. - ebIrlb;Bn§ú cMnYnbYn/ manCMgW Hydrops foetalis ¬b¤ Hb Barts¦ ekIteLIg )anesckþIfa Tarksøab;kñúgépÞ.

130

CMBUkTI 8 rbbBüa)alEdl)aneFVI nig CMhannaBalGnaKt 8a

karbnSaMxYrq¥wg karbnSaMxYrq¥wg ebITTYleCaKC½y Gacpþl;karBüa)al )anCadac; cMeBaHGñkCMgWtaLasesmIukMritF¶n;. karbnSaMxYrq¥wg sMrab;CMgWtaLasesmIu RtUv)aneKcab; epþImeFVItaMgBIqñaM 1981 rhUtmkdl;eBlenH manCag 1500 krNI. GVIeTACakarbnSaMxYrq¥wg ? xYrq¥wgKWCaCalikaes<at sßitenAkñúgq¥wg ¬emIlrUb 8a¦ EdlplitekasikaTaMgGs;EdlmankñúgQam. enAGayu v½yCMTg; xYrq¥wgmankñúgq¥wgCMnI/ q¥wgsnÞHRTUg/ q¥wgk,al/ q¥wgRtKak nig q¥wgkgxñg b:uEnþenAeBleTIbekItBIépÞmþay eKeXIjxYrq¥wgmanenAtamq¥wgepSg²eTotEdr. xYrq¥wgmanpÞúkekasikaedIm ¬emIlrUb 8b¦ EdlecjBIenaH ekItecjCaeKalikaQamRkhm/ eKalika Qams nig pøaEkt. karbnSaMxYrq¥wg KWCakarykxYrq¥wgBIbuKÁlEdlmansuxPaBl¥ ¬ehAfa Gñkbric©ak¦ edIm,Ipþl;eTA[GñkCMgW ¬ehAfa GñkTTYl bnSaM¦. xYrq¥wgKμansuxPaBl¥rbs;GñkCMgWTTYlkarbMpøicbMpøaj dMbUgedayfñaM b¤ CYnkaledaykaMrsμIviTüúskmμ (Irradiation) kñúg dMeNIrmYyEdleKehAfa sPaBeCosminput xYrq¥wgEdll¥ rEmgykecjBIq¥wgRtKakrbs;Gñkbric©ak bnÞab;mkpþl;va[eTAGñkCMgWkñúg sPaBravedaybBa©ÚlkñúgcrnþQam RsedogKñanwgkarbBa©ÚlQameTogTat;

131

dUecñaHEdr ¬emIlrUb 8c¦. eBlcUldl;kñúgcrnþQamrbs;GñkTTYl/ ekasika xYrq¥wgGñkbric©ak eFVIdMeNIreTAkan;q¥wgFM² EdlenATIenaHvacab;epþImplit ekasikaQamFmμtamansuxPaBl¥. dMeNIrenHRtUveRbIry³eBl 2 - 3 s)aþh_. etImaneRKaHfñak;GVIxøH ? eTaHCadMeNIrenHGacTTYleCaKC½yeRcInk¾eday vak¾maneRKaHfñak;F¶n;F¶rEdr. dUecñHmunnwgGnuvtþbnSaM eKRtUvBicarNaedayykcitþTukdak;GMBIeRKaHfñak; nig plRbeyaCn_cMeBaHkrNInImYy². edIm,ITTYl)anlT§pll¥ xYrq¥wgRtUvEtpþl; edaybgb¥ÚnRbus b¤ RsI EdlmanCalikasIuKñaeBjelj. TMraMEtxYrq¥wgEdlbNþúHGacCMnYsxYrq¥wg rbs;GñkTTYlbnSaM)anenaH varEmgekIt manplvi)ak dUcCakarbgáeraK nig karhUrQam. plvi)akelIsBIenHeTA eTotenaH KWCMgWTMnas;bnSaM ¬Graft versus host disease = GVHD¦ kalNa xYrq¥wgEdlbBa©ÚleTAyl;fasarBagÁkayGñkCMgWKWCavtßúeRkAcMElk edaybegáIt CabBaðacMeBaHEs,k eBaHevon nig eføIm kñúgsPaBRsal/ F¶n;F¶r b¤ rhUtdl; søab;k¾man. bNþaGñkCMnaj)anraykarN_fa eRkayBI)aneFVIbnSaMxYrq¥wg/ bBaðaTaMgLay Tak;TgnwgCMgWtaLasesmIu dUcCa karelIsbnÞúkCatiEdk/ rlakeføImraMuér:/ bBaðaebHdUg nig RkeBjbeBa©jkñúg mansPaBgayRsYlRKb;RKgeRcInCagmun rIÉsrIragÁEdlTTYlrgkarxUcxat CYnkalGacCasHes,Iyvij. eTaHCaya:g Nak¾eday eKRtUvbnþtamdanGñkCMgW edayykcitþTukdak;eBjmYyCIvit. eRkayBI)aneFVIbnSaMxYrq¥wg FatuEdkEdlelIskkkujkñúgsarBagÁkayGñkCMgW muneBleFVIbnSaMRtUvEtkMcat;ecj. eKGaceFVI)anedayeRbIfñaMkMcat;CatiEdk b¤ edayRKan;EtbgðÚrQamecjerogral;BI 1 - 2s)þah_. Cakarl¥bMput eKGac bUmQamykecjBIsrésEv:n 6 m>l kñúgmYyKILÚRkamTMgn;xøÜn erogral; RbEhl 14éf¶ . 132

sMxan;RtUvyl;fa karbnSaMxYrq¥wg RKan;EtBüa)alxYrq¥wgb:ueNÑaH. dUecñHGñkCMgW EdlmanKMeragmanépÞeBaHRtUvcgcaMfa Bn§ú rbs;eKenAEtmanCMgWtaLasesmIu dEdl Edlnwgpþl;bnþeTAkUnrbs;eK)an. etIeKGacbnßyeRKaHfñak;[enAticbMput)andUcemþc ? k> CMerIsGñkbric©ak> meFüa)aysMxan;bMputedIm,IrkSaeRKaHfñak;[enAtic KWRtUv[manPaBsIuKñaBitR)akd ¬exact match¦ rvagCalikaGñkCMgWCamYy CalikaGñkbric©ak. Gñkbric©akl¥bMput EdlBitCaman HLA sIuKñaCamYyGñkRtUveFVIbnSaMxYrq¥wg KWbgb¥ÚnePøaH. PaBsIuKñaEk,rbMputTI2 KWbgb¥ÚnbegáItEdllkçN³ HLA rbs; eKmanPaBsIuKñaya:geRcInnwg HLA rbs;bgb¥ÚnRbus b¤ RsIrbs;GñkEdlRtUveFVI bnSaMxYrq¥wg. »kasGacrk)annUvPaBsIuKñaBIbgb¥ÚnRbus b¤ RsI KW 4 man 1 ¬25°¦ vaBi)akNas;nwgrkGñkmanPaBsIuKñaenHenAeRkArgVg;RKYsar. Bn§úEdl kMNt;RbePTCalikaeKehAfa Human Lymphocyte Antigen (HLA) genes EdlR)ab;[eyIgdwgBIbNþaRbePTCalika EdlEsþgeLIgenAelIeKalikaQam s TaMgLay. kñúgcMeNam Antigen TaMgenH/ HLA-A, HLA-B, HLA-C nig HLA-DR edIrtYnaTIsMxan;CageKkñúgkarpÁÚrpÁg[manPaBsIuKñasMrab; bnSaMxYr q¥wg. man Antigens cMnYn 59 enATItaMg HLA-A, cMnYn 118 enATItaMg HLA-B, cMnYn 36 enATItaMg HLA-C nig cMnYn 137 enATItaMg HLA-DR. RbB½n§enHTaMgmUlman HLA-loci CaeRcIneTot RBmTaMgmanBn§úCag 500 eday begáIt[manlT§PaBpÁúMcUlKña)aneRcInekad kñúgcMeNammnusSkMNt;mYy. ya:gNak¾eday edayBn§úTaMgenHmanTMnak;TMngnwgKña nig bnþeTAkUnecAkñúg lkçN³CasMNuMenaH/ bgb¥ÚnRbus nig RsI RtUvTTYlCalkçN³tMNBUC nUvsMNMu NamYyénsMNMuTaMgBIr BI«Buk nig mþayTaMgsgxag eFVI[»kasénsMNMuman lkçN³dUcKñadl;eTA 25°. »kasCaTUeTA/ ya:gehacNas;manbgb¥Ún RbusmYy b¤ RsImYyEdlmanxYrq¥wgsIuKña b:uEnþKμanCMgWtaLasesmIukMritF¶n;/ 133

134

GaRs½yeTAelITMhMRKYsarmanbgb¥Úntic b¤ eRcIn. CamFümmanGñkCMgWRbmaN 30° b:ueNÑaHEdlEsVgrkkareFVIbnSaMxYrq¥wg nwgrk)annUvGñkbric©akxYrq¥wg EdlmanlkçN³sIuKñaeBjelj. dUckarKitRsém:/ karlM)akkñúgkarpÁÚrpÁg[manPaBsIuKña énxYrq¥wg)anBIGñk bric©akEdlKμanCab;sac;Qam ¬eRkArgVg;RKYsar¦ kan;EtmanPaBFMeFgEfm eTot. karKNnaCaRTwsþIbgðajfa PaBxusKñaénTMrg; HLA BIbuKÁlmYyeTA buKÁlmYyepSgeTot EdlKμanCab;sac;Qam manemKuNxusKñadl;eTA mYyBan;lan. CakarsMxan;/ RtUvFana[manPaBsIuKñaebHbiTénxYrq¥wgGñkeFVI bric©ak nig GñkRtUvTTYlkarbnSaMxYrq¥wg edayehtufa»kasTTYleCaKC½y manticNas; kalNaPaBsIuKñaenHmankMrittic. dUcenH karEsVgrkPaBsIuKña BIGñkbric©akeRkArgVg;RKYsar eFVIeLIgEteBlNaeKBMuGacrk)annUvPaBsIuKña kñúgrgVg;RKYsar nig smaCikRKYsarCab;sac;QamCamunsin. elIsBIenHeTot Gñkbric©akxYrq¥wgRtUvmanGayuya:gticcab;BI 3qñaMeLIgeTA edIm,IeGaymuxgar xYrq¥wgrbs;eKmanPaBcas;TMuRKb;RKan;. kic©RbwgERbgkñúgbNþaqñaMcugeRkayenH)anRbmUlpþúMeTAelIkareFVI[RbesIrdl; bec©keTspÁÚrpÁg[manPaBsIuKña edIm,IGacpSaMxYrq¥wgrvagGñkeFVIbric©akminCab; sac;QamehIymanPaBsIuKña (Matched Unrelated Donors = MUDs) CamYyGñkRtUveFVIbnSaMxYrq¥wg/ tamry³enH vapþl;lT§PaBkan;EteRcInkñúgkarrk Gñkbric©ak. eK)anbegáItnUvbBa¢Irkt;RtalkçN³rbs; HLA énGñkeFVIbric©ak CMuvijBiPBelakEdlsμR½Kcitþ[eKeFVIetsþ edaymanrayeQμaH nig Gas½ydæanrbs; BYkeK. enAeBleKeXIjfa GñkRtUveFVI bnSaMxYrq¥wgman HLA sIuKñaGacTTYlyk )an/ PøamenaH eKCUndMNwgeTAGñkbric©ak ehIyGnuvtþkarbnSaMxYrq¥wgPøamEtmþg. KMnitbegáIt[manbBa¢IrGñkeFVIbric©akxYr q¥wgEdlminCab;sac;Qam ekIteLIg

dMbUgenAqñaM 1987 ehIybc©úb,nñenHmanGñkbric©aksuxPaBl¥cuHeQaμH RbmaNCa 3/5lannak; CaBiessenAshrdæGaemrik nig GWr:ub. eTaHbIya:g Nak¾eday eTaHCa)anGnuvtþkargarmYycMnYnTak;TgnwgGñkbric©akminCab;sac; Qamk¾eday eKenARtUvkarRsavRCavCaeRcIneTot edIm,Ikat;bnßyeRKaHfñak; Tak;TgnwgkarbnSaMxYrq¥wgEdlmankMritx<s;CagkarykxYrq¥wgBIGñkbric©akkñúg cMeNambgb¥ÚnbegáIt EdlmanPaBsIuKña . x> CMerIsGñkRtUveFVIbnSaMxYrq¥wg ¬GñkCMgW¦> GñkCMgWmYycMnYnCYbeRKaHfñak; BIkarbnSaMxYrq¥wgkñúgkMritx<s;CagGñkCMgWepSgeTot. eK)ansÁal;ktþabgá eRKaHfñak;mYycMnYn ³ eføImrIkFM - FMCag 2 s>m eføImxUcxat - sPaBcak;es£Hedaysrésevov ¬Fibrosis¦ b¤ eføImman søaksñamdMe)A karRKb;RKgsPaBelIsbnÞúkCatiEdkmin)anl¥ QrelIktþaTaMgenH eKeFVIcMNat;fñak;GñkCMgWCa bI RbePT ehIyRbePTnImYy² manGRtaTTYleCaKC½yxus²Kña. cMNat;fñak; I manktþabgáeRKaHfñak;EtmYy/ ÉcMNat;fñak; III manktþabgáeRKaHfñak;TaMgbIEtmþg. ktþabgáeRKaHfñak;mYyeTotdac;EtÉg KWGayuGñkCMgW/ lT§plénkarbnSaMxYrq¥wg elIGñkCMgWGayuelIsBI 16qñaMeLIgeTA mansPaBya:b;yWunCagGñkCMgWv½yekμg ¬sUmemIltaragxageRkam¦ ³

kumar ¬16qñaM¦

cMnYn

cMNat;fñak; I cMNat;fñak; II cMNat;fñak; III eBjv½y ¬17-22qñaM¦

121 272 125 70

GRtars; manCIvit ° 95 85 78 70

KμanRBitiþkarN_ GVIekIteLIg ° 90 81 59 66

[Pesaro Experience 1995]

135

Cakarl¥ GñkCMgWbnSaMxYrq¥wg KYrmanGayueRkam 16qñaM rYmCamYyeføImman suxPaBFmμta manelIsbnÞúkCatiEdktictYc ehIymanbgb¥ÚnRbus b¤ RsI Edlman HLA sIuKñaBitR)akd. dUc)anbBa¢ak;kñúgtaragxagelI GRtars;ran manCIvitmankMritTab cMeBaHGñkCMgWEdleFVIbnSaMxYrq¥wgenAGayuelIsBI 16qñaM. CMhanepSg²eTot cMeBaHkarbnSaMxYrq¥wgEdlmanPaBsIuKñaBit R)akd ¬Cab;sac;Qam¦ bNþaevC¢bNÐitkMBugRbtibtiþkaredIm,IeFVI[GRtaeCaKC½y)anRbesIr nig kat; bnßyplvi)akbNþalmkBIkarbnSaMxYrq¥wg)anBIGñkbric©akmanPaBsIuKñaEt minCab;sac;Qam ¬MUDs¦/ BIsmaCikkñúgRKYsarmanPaBsIuKña EdlminEmn Cabgb¥ÚnbegáIt nig BI«BukmþayEdlminmanPaBsIuKña. TidæPaBmYy KWRtUvpøas; bþÚrkarBüa)al ¬sPaBeCosminput¦ edIm,Ikat;bnßyeRKaHfñak;ekItecjBI nitiviFIénkarbnSaM. eTaHya:gNa lT§plTTYl)anmanPaBERbRbYl ehIy eRKaHfñak; nig plcMeNjRtUvEtBicarNaedayQrelImUldæankrNInImYy² ¬sUmemIltaragxageRkam¦. lT§pl énkarbnSaMxYrq¥wg )anBIGñkbric©akepSg²kñúgCMgWtaLasesmIu

GñkCMgWtaLasesmIukMritF¶n; cMnYn 29nak; Gayu 1/1 - 33qñaM ¬GayumFüm 6qñaM¦ - 6nak; mansac;jatiman HLA dUcKñaebHbiT - 2nak; mansac;jatiKμanPaBsIuKña - 13nak; manbgb¥ÚnbegáItKμanPaBsIuKña - 8nak; man«BukmþayKμanPaBsIuKña minTTYl ¬Rejection¦¼braC½y 55° ¬minTak;TgnwgKμanPaBsIuKña¦ TMnas;bnSaMRsYcRsav ¬Acute GVHD¦ I-IV 47° TMnas;bnSaMraMuér: ¬Chronic GVHD¦ 38° GRtaenArs;manCIvit ¬FU 7/5qñaM¦ 65° ¬21° KμanRBitiþkarN_GVIekIteLIg¦ søab;edaykarbnSaM 34° ¬50° TMnas;bnSaM/ 30° karbgáeraK¦

[Gaziev et al, Pesaro BMT 2000]

136

esckþIsnñidæan karbnSaMxYrq¥wg edayykBIbgb¥ÚnbegáItEdlmanPaBsIuKñaeBjelj pþl;»kas GacCHsHes,Iyya:gx<s; ehIyeRKaHfñak;bNþal[søab;edaysarnitiviFIén karbnSaMmankMritTabEt 5° cMeBaHGñkCMgWEdlRtUv)aneRCIserIsl¥. kargar kñúgTisedAbnßyeRKaHfñak;BIkarbnSaMenAEtbnþeTAmuxeTot rYmmankareFVI[ RbesIreLIgnUvlT§plbnSaMxYrq¥wg)anmkBIGñkbric©ak EdlminEmnCabgb¥Ún begáItEtmanPaBsIuKñaeBjelj. bnSaMxYrq¥wgedayeRbIQamTgsuk bec©keTseFVIbnSaMxYrq¥wgmYyepSgeTot KW eFVIetsþBinitülkçN³ HLA rbs; TarkEdlsßitkñúgépÞmþayrbs;kumarmanCMgW. ebITarkkñúgépÞman HLA sIuKña CamYynwgkumarenaH/ eKGaceFVIbnSaMedayeRbIQamTgsukCMnYsxYrq¥wgeTAkñúg xøÜnkumarEdlmanCMgW. QamTgsuk KWCaQamEdlsßitkñúgsuk ¬Placenta¦ nig Tgsuk ¬umbilical cord¦ eRkayBITarkekItrYc vaCaRbPBsMbUreday ekasikaedIm ¬Stem cells¦ EdlTTYlxusRtUvdl;karpliteKalikaQam. eTaHya:gNak¾eday EdnkMNt;sMxan;cMeBaHbec©keTsenH KwfaQamTgsuk rbs;Gñkbric©akRtUvmanpÞúkekasikamansñÚl ¬Nucleated Cells¦ kñúgcMnYn Gb,brimamYy ¬>1x107¦ kñúgmYyKILÚTMgn;énGñkRtUveFVIbnSaMxYrq¥wg. elIsBI enHeTot/ eføIm/ tMrgenam/ ebHdUg nig sYt rbs;GñkCMgWRtUvmandMeNIrkar l¥pgEdr. karTTYleCaKC½ydMbUgbMput cMeBaHkarbnSaMedayeRbIQamTgsuk RtUv)aneK GnuvtþtaMgBICag 10qñaMmun. bnÞab;BIenaHmk RbtibtiþkarEbbenHcMnYn 500 RtUv)aneFVITUTaMgBiPBelak. bec©keTsenHk¾)anTTYleCaKC½ypgEdrkñúgkar Büa)alGñkCMgWtaLasesmIukMritF¶n;mYycMnYn. eTaHya:gNak¾eday eKenARtUv karRsavRCavbnþeTot edIm,IeFVI[GRtaTTYleCaKC½ykan;EtRbesIreLIg . 137

bnSaMedayQamTgsuksMrab;CMgWtaLasesmIu bnSaM enArs;manCIvit CasHes,Iy taLasesmIuminCasHes,Iy

cMnYnkumar 10 10 4 4

[Rocha et al. 1998, Eurocord]

KuNRbeyaCn_EdlGacekItmanBIkareFVIbnSaMEbbdUcenHman ³ QamTgsukminsUvmanGtisuxumR)aNbgáeraK eRKaHfñak;CMgWTMnas;bnSaM ¬GVHD¦ mankMritTab eRBaH T-lymphocytes EdlbgáTMnas;bnSaMminTan;GacdMeNIrkar)aneBjelj enAeBlTark eTIbnwgekIt ¬enHeKRtUvEtbBa¢ak;cMeBaHGñkCMgWtaLasesmIu¦ PaBsIuKñaén HLA minsUvTamTarxøaMgdUckrNIbnSaMxYrq¥wgFmμta epSgeTot enaHeT mkTl;eBlenH/ minTan;manlT§plRKb;RKan;edIm,Ibgðajfa etIKuNRbeyaCn_ TaMgenHGacEsþgecjCaplsMercya:gRbesIr b¤ ya:gNaenaHeT. bc©úb,nñenH/ kargarkMBugdMeNIrkaredIm,IbegáItRbB½n§mCÄmNÐlsþúkQam Tgsuk dUcKñanwgRbB½n§mCÄmNÐlpþl;Qamedaysμ½RKcitþnaeBlbc©úb,nñenH Edr. kñúgkrNIGnuvtþkarEfTaM)anRtwmRtUv/ QamTgsukGacrkSaTukenA sItuNðPaB -19oC )anry³eBl 20qñaM. mCÄmNÐlQamTgsukd¾FMbMput KW mCÄmNÐlpþl;QamTIRkug jÚy:k ¬New York Blood Center¦ EdlRbmUl )anKMrUQamCag 7000 fg; ehIy)anCYydl;krNIbnSaMCag 200 krNI. bc©úb,nñenH/ edIm,ITTYl)an nig eFVIbnSaMQamTgsukBImCÄmNÐlpþl;Qam TIRkug jÚy:k eKRtUvcMNay 28 000 duløashrdæGaemrik. RbeTsGaLWm:g;/ )araMg/ cRkPBGg;eKøs/ GIutalI/ EblhSik/ hULg; nig RbeTseGs,aj k¾)anbegáItmCÄmNÐlpþl;QamTgsukpgEdr.

138

CMBUkTI 9 CMhanepSg²eToteq<aHeTArkkarBüa)al »sfCMrujbegáIt eGm:UkøÚb‘ÍnTarkkñúgépÞ (HbF inducers) bNþaGñkviTüasaRsþ kMBugEteFVIkarRsavRCavya:gCab;lab; rkmeFüa)ay epSg²eTot edIm,IBüa)alCMgWtaLasesmIu edayrkmeFüa)ayBüa)alEdl mineFVITukçxøaMg maneRKaHfñak;tic nig Gs;R)ak;kasticCagviFIBüa)albc©úb,nñ. CMhanfμIPaKeRcIn)ancab;GarmμN_eTAelIkarkat;bnßyGtulüPaB rvagExS køÚb‘Ín. Ca]TahrN_ fñaMmYycMnYnRtUv)aneKsÁal;faGacbegáInkarplitExS RbUetGIunepSgeTot dUcCaExSRbUetGIunháama: ¬γ-chains¦ EdleRkayBI)an cab;KUrCamYyExSGal;hVa begáIt)anCa eGm:UkøÚb‘ÍnTarkkñúgépÞ HbF (a y ) dUecñHva)anbnßyExSGal;hVaesrI. fñaMTaMgenHeTAbnßyskmμPaBxYrqw¥wg ehIy eP£aceGayplit ExSháama:. fñaMTaMgenHrYmman 5-azacytidine, Cytosine arabinoside, Hydroxyurea, Erythropoietin, nig ExSRsLay Butyric acid. TaMg 5-azacytidine nig cytosine arabinoside )anbgðajnUv\T§iBl BulcMeBaHGñkCMgWtaLasesmIu ehIytaMgBIenaHmkeK)anbBaÄb;kareRbIR)as; fñaTM aMgenH ebIeTaHbIExSRsLayrbs;vafμI ehIyminsUvBulkMBgu RtUv)anRsavRCav k¾eday. eKsegáteXIjeCaKC½ymYycMnYnCamYy Hydroxyurea nig Erythropoietin eTaHbIrhUtmkTl;eBlenH KuNRbeyaCn_KwmancMeBaHGñkCMgW taLasesmIukMritmFüm eRcInCagGñkCMgWtaLasesmIukMritF¶n;. eKenAminTan;yl;c,as;enAeLIynUvtYnaTIrbs;ExSRsLay Butyric acid kñúgkareP£ac[mankarsMeyaK ExSháama:. eTaHya:gNak¾edayBYk butyrates d¾manRbsiT§iPaBbMput KW Arginine butyrate EdleKeRbIedaycak;bBa©Últam srésQam. GñkCMgWmYycMnYnmankareqøIytb)anl¥ cMeBaHkarBüa)alEbb enH Etehtuplrbs;vaeKminTan;yl;c,as;las;enAeLIyeT. karvivtþn_naeBl GnaKtGacBwgEp¥kelIkareRbIR)as;bBa©ÚlKñanUv Arginine butyrate nig 2 2

139

b¤ Arginine butyrate nig Erythropoietin b¤eRbIfañ TM aMgbIclU Kña. kñúgkrNICMgWtaLasesmIukMritF¶n;/ eKalbMNgénkareRbIfñaMTaMgenH KWedIm,IBnüa cenøaHeBlbBa©ÚlQam ÉcMeBaHCMgWtaLasesmIukMritmFümvijKWbeBa¢ósb¤Bnüa tMrvU karbBa©lÚ Qam ehIykat;bnßykarQWcku cab;qgw¥ bNþalmkBIkarrIkénxYrq¥gw . Büa)aledayBn§ú (Gene therapy) karBüa)alcugeRkaycMeBaH CMgWtaLasesmIukMritF¶n; TamTarkarbBa¢ÚnBn§ú rbs;GñkmansuxPaBl¥cUlkñúgekasikaedIm ¬Stem cells¦ énxYrq¥wg KWCaCMhan mYyEdleKehAfa karBüa)aledayBn§ú. 10qñaMcugeRkayenHmankarvivtþn_ ya:gFMeFgkñúgkarbegáItRbB½n§CIvsaRsþ EdlmansmtßPaBGacnaMykBn§úGñk mansuxPaBl¥cUleTAkñúgekasikaedImrbs;GñkCMgW. kñúgbNþaqñaMcugeRkay/ eK )aneRbIGtisuxumR)aNCavIrus ¬Retrovirus¦ TTYl)anlT§plya:gRbesIr. ya:gNak¾eday/ bBaðaecaTCamYyviFIEbbenH KWRtUvFana[)anfa ExSEbtta RtUvekItecjBIBn§úmansuxPaBl¥EdleKbBa©lÚ eTAenaHmanKuNPaB nig brimaN x<s;RKb;RKan;kñúgry³eBlmYyEvg. elIsBIenH/ enAmankarRBYy)armÖN_BI suvtßiPaBénkarbBa©ÚlvIrus nig karb:HBal;Cayfaehtudl;Bn§úepSg²eTot. eraKvinicä½yrkPaBsIuKñaén HLA munkarbgákMeNIt kñúgbNþalqñaMcugeRkayenH karRsavRCav)anepþatelIbec©keTsmYyeKehAfa eraKvinicä½yBn§úmunkarbgákMeNIt b¤ PGD ¬Pre-implantation Genetic Diagnosis¦ kñúgbec©keTsenH eKyk HLA rbs;kumarmanCMgWmkpÁÚrpÁgeGay sIuKñaCamYy HLA rbs;emCIvitRsþIEdlKμanpÞúkCMgWtaLasesmIu bnÞab;mkeKyk mkbgákMeNItkñúgs,ÚnRsþI. kumarEdlnwgcab;kMeNIt nwgmanlT§PaBpþl;[ bgbegáItrbs;va nUvPaBdUcKñaebHbiTénxYrq¥wgsMrab;eFVIkarbnSaM. viFIEbbenH/ va)anecaTCabBaðadl;EpñksIlFm’CaxøaMg Etva)anpþl;nUvkþIsgÇwmfμIsMrab;kar Büa)al. elIsBIenHeTot/ viFITaMgenHRtUvkarcMNayya:geRcIn nig TamTarnUv bec©keTsx<s;. eTaHya:gNak¾eday/ karvivtþn_eTAmuxya:gelOneFVIeGay bec©keTsenHGaceRbIR)as;)anya:gTUlTM lU aykñgú eBlGnaKtxagmuxya:gxø.I Hydroxyurea

140

CMBUkTI 10 bBaðaecaTEpñkcitþsgÁm cMeBaHGñkCMgW minxusBICMgWraMuér:epSg²eToteLIy GñkCMgWtaLasesmIukMritF¶n;RtUvRbQmmux nwgkarlM)akF¶n;F¶rmYycMnYn. tMrUvkarragÁkay k¾dUcCakgVHkaryl;dwgCa saFarN³tambNþaRbeTsCaeRcInEdlmanekItCMgWenH )aneFVI[GñkCMgW «Bukmþay nig RKYsarrbs;eK CYbkarlM)akpøÚvcitþkñúgkarxitxMedaHRsaybBaða CMgWtaLasesmIuenH. kñúgeBlNamYykñúgCIvit/ GñkCMgWGacnwgCYbbBaðapøÚvcitþ dUcCa³ karGs;Twkcitþ/ karxkbMNg/ PaBesakesA/ xwgcMeBaHxøÜnÉg/ karFøak; Twkcitþ/ sPaBfb;GarmμN_/ karP½yxøacesckþIsøab;/ kar)at;bg;PaBeCOCak;/ PaBÉkeka/ mYrem:A/ Gs;sgÇwm/ manGarmμN_xVHesckþIRsLaj;BImnusSdéT/ karminTukcitþ/ manGarmμN_famanPaBxaXaMgcMeBaHxøÜnRCul/ minsUv[tMél cMeBaHxøÜnÉg nig GarmμN_epSg²eTot. mü:ageTot/ GñkCMgWmanGarmμN_rMCYlcitþCaviC¢manepSg²eTotEdr dUcCa PaBkøahan nig karBuHBarGMNt; EdlCYyBYkeKeGayqab;manKMnitcas;TMu ehIykøayCaGñkkøahanmanKMnitéqñRbDit nig eCOCak;. cMeBaH«BukmþayGñkCMgW eRkABIrMCYlcitþGviC¢man nig viC¢man dUcGñkCMgWEdr/ «BukmþayenAmanTTYl GarmμN_faxøÜnCaGñkTTYlxusRtUv b¤ mankMhuscMeBaHsßanPaBsuxPaBrbs; kUnxøÜnÉgeTot. «BukmþayPaKeRcInrn§t;citþCaxøaMg eBleKdwgfakUnrbs;eK maneraKvinicä½yCaCMgWenH. enARbeTsEdlKμankaryl;dwg b¤ mankaryl;dwg bnþicbnþÜcGMBICMgWenH nig karBüa)alrbs;va/ matabitaminGacpþl;karEfrTaM edayRtwmRtUvenAÉpÞHeLIy EdlvaCacMENkd¾sMxan;cMeBaHeCaKvasna 141

¬buernimitþ¦ kñúgry³eBlEvgrbs;kumar. eraKvinicä½yCMgWtaLasesmIu Gacbgá pgEdrdl;PaBtantwgF¶n;F¶rcMeBaHTMnak;TMngbþIRbBn§ CYnnaMeTAdl;karEbk)ak; b¤ ElglHKña. müa:gvijeTot/ CMgWk¾GaceFVIeGaybþIRbBn§rbs;«Bukmþaykan; EtCitsñiT§EfmeTotk¾man edaytaMgcitþfanwgkarBarKaMRTkUnrbs;BYkeK. dUcKñaenHEdr matabitaGacykcitþTukdak;EfrkSaeRcIncMeBaHkUnmanCMgW taLasesmIu bgáPaBtantwgdl;TnM ak;TnM gCamYysmaCikepSg²eTotkñgú RKYsar CaBiesskUn²epSgeTot . cMeBaHGñkCMgW nig «Bukmþay GarmμN_GñkCMgW k¾dUcCa«Bukmþay EdlmancMeBaHkarBüa)alRbkbedaykar QWcab;kñúgry³eBlyUr edaykarbBa©ÚlQam nig kMcat;CatiEdk rEmgGacnaM [ekItmanbBaðapøÚvcitþ. vaekItecjBIPaB)at;muxmat;kñúgsgÁmedaysarCMgW taLasesmIu nig CMgWBn§úbnþBUCepSgeTot/ «BukmþayPaKeRcInmincg;elIkyk eraKvinicä½ymkBiPakSaCamYyGñkdéTeLIy edayxitxMlak;)aMg sUm,IEt smaCikkñúgRKYsarCitditk¾min[dwgEdr. sUm,IEtbc©úb,nñenH enAbNþaRbeTs EdlkMrit nig KuNPaByl;dwg rbs;saFarN³CnmankMritx<s; RBmTaMgman kareCOnelOneTAmuxEpñkKøInikcMeBaHGayukal nig KuNPaBCIvitrbs;GñkCMgW taLasesmIu/ k¾GñkCMgW nig «Bukmþay CYnkalRkumRKYsarTaMgmUl enAEtman TTYlGarmμN_EbbxagelI rhUtTal;EteK)anyl;dwg TTYlykkarrs;enA CamYytaLasesmIu ehIyyl;dwgBIviFITb;Tl;CamYyCMgW. dUcenH«Bukmþay nig GñkCMgW nwgTTYlrgTukçRBYykñúgry³eBlmYy rhUtdl;bBaðapøÚvcitþGviC¢man d¾xøaMgenH)anFUres,Iyvij ehIyCMgW nig karEfTaMCMgWsMrbsMrYlkñúgCIvPaBRbcaM éf¶eK)an. karKaMRTBIRKUeBTü kñúgGMLúgeBlenHGacRtUvkarcaM)ac;CaTIbMput. eKeXIjPaBesakesAénerOgEbbenH enAbNþaRbeTsEdlTTYlrgCMgW taLasesmIu ehIyFn;FanmankMrit KwfaGñkCMgW nig «BukmþaysikSaEsVgyl; GMBICMgW nig karBüa)al b:uEnþEbrCaman»kastictYckñúgkarbBa¢ak;GMBIbBaða rbs;GñkCMgW. sPaBEbbenHnaM[ekItCaPaBmYhμg Gs;sgÇwm KμankMlaMg 142

kMEhg Føak;Twkcitþ nig TTYlPaBÉkekakñúgsgÁm. GñkCMgWkñúgRbeTsTaMgenH søab;TaMgekμgv½y CYnedaymin)aneFVIeraKvinicä½y b¤ eFVIeraKvinicä½yxusehIy Büa)alminRtwmRtUv b¤ min)anBüa)alesaHEtmþg. TTYlBt’man nig karKaMRT sMxan;matabitamankUnmanCMgW RtUvEsVgrkRbPBBt’man[)aneRcIn tamEdl GaceFVIeTA)anGMBICMgWtaLasesmIu tambNÑaKar tamGIunFWENt nig BI«Buk mþayepSgeTotEdlmankUnekItCMgWtaLasesmIu. smaKmn_CatiCMgW taLasesmIu nig shB½n§GnþrCatiCMgWtaLasesmIu edIrtYnaTICaKnøwH kñúgkar pþl;dl;matabitanUvRbPBBt’mansMxan; nig karKaMRT edayCYyBRgwgnUvCMenO rbs;BYkKat;kñúgkarCYykUnecA[manCIvitrs;enA)anl¥ ebIeTaHbICamanCMgW. «BukmþayEdlTTYlBt’man)anl¥ GMBICMgWtaLasesmIu GMBIkarBüa)al nig karkarBar/ Kat;manlT§PaBya:gl¥ edIm,IKaMRTdl;kUnrbs;Kat; ehIyebIsinCa Kat;manbMNg Kat;GackarBarmin[mankUnTI2manCMgWenH. evC¢bNÐit KWCa RbPBBt’manbEnßmsMxan;mYy EdlCYypþl;karENnaM nig kareCOCak;. tYnaTI rbs;Kilanubdæak-yika k¾sMxan;Edr. kñúgkrNImYycMnYn TMnak;TMng Kilanubdæak-yika nig GñkCMgWxusKñaBITMnak;TMngrvagevC¢bNÐit nig GñkCMgW. edaysarmaneBlevlaeRcIn nig eTogTat;CamYyGñkCMgW Kilanubdæak-yika rEmgEtgEtCaGñkdMbUgEdlTTYl)anBt’mansþIBIbBaðaBiess² EdlGñkCMgWGac CYbRbTH. elIsBIenHeTot/ edayTIkEnøgbBa©ÚlQammanPaBmindUckEnøg déT/ Kilanubdæak-yikaRtUvEtmanTMnak;TMngl¥CamYyRkumRKYsar nig mitþPkþi GñkCMgWEdlBYkKat;RtUvkarcg;dwgBIkarlM)akénkarBüa)al EdlGñkCMgW GacCYbRbTH. CYnkalGñkTaMgenH nwgsμ½RKcitþCYypþl;Bt’manrYcCaeRsc munGñkCMgWeTAeTot. eTaHbIya:gNak¾eday/ sMxan;KWfaBt’manTaMgenH RtUvTuk CUneTAevC¢bNÐit edIm,IsMerceFVIkarBüa)al. EpñkmYyd¾sMxan;bMputéntYnaTIrbs;evC¢bNÐit nig Kilanubdæak-yika KWTak;Tg nwgkarGnuvtþn_tamkarBüa)al. «Bukmþay nig GñkCMgWPaKeRcIn)an[dwgfa 143

TidæPaBlM)akbMputénkarEfTaMCMgWtaLasesmIukMritFn; KWenAeBlcab;epþIm karBüa)alkMcat;CatiEdk. eRkayBI)anRTaMRTnwgkarbBa©ÚlQamral;Exmk GñkCMgW nig matabitaRtUvRbQmmuxnwg]bsKÁmYyepSgeTot. RKUeBTü Büa)al nig buKÁlikEfTaMrEmgGacCYysMrYl)anya:geRcInkñúgdMNak;kalenH edaypþl;[kmμviFIBüa)alTTYl)aneCaKC½y nig KμanPaBtwgEtgeRcIn. GñkCMgWtaLasesmIukMritF¶n; Cakumar CaekμgCMTg; nig CamnusS eBjv½y GñkCMgWCakumar ³ kñúgbNþaqñaMdMbUg kumarTTYlGarmμN_Qwcab; nig karrMxanBI karBüa)al ehIyBYkeKminyl;BIGVIEdlekIteLIgcMeBaHBYkeKeLIy. ya:gNak¾eday/ enHk¾CaeBlevlaEdlkumarekItmannUvCMenO nig kareCOTuk citþcMeBaHGñkEdlemIlEfTaMBYkeK. dUecñHsMxan;Nas;Edl«Bukmþay Rbjab; TTYlyksßanPaBenH edIm,Icab;epþImksagTMnak;TMngl¥mYyrwgmaMCamYykumar EdlCakarCYy[TTYlykkarBüa)al. karlM)akgayekIteLIgNas;cMeBaH kumarGayu 2-3qñaM EdlCav½ykumarcab;epþImmanm©as;karelIxøÜnÉg KWvaecHeFVI kargaredayxøÚnÉg ecHpþÜcepþImKMnit. KwenAeBlenaHÉgehIy Edlkumar cab;epþImyl;faxøÜnvaTTYlnUvPaBxaXaMgGVIEdleKcg;eFVI )ann½yfaxøÚneKmin GaceFVIGVImYy)andUcekμgdéTEdlmanGayuRs)al²Kña. eBlkumarQandl;v½ycUlsalaeron sßanPaBrwtEtlM)akEfmeTot. eRkABI karQWcab; nig karrMxanBIkarBüa)al/ karyl;dwgBIsPaBxøÜnvaxusBIeK CaBiess sPaBrUbragÁxuseKeBlEdlmanbMErbMrYlq¥wg b¤ karlUtlas; yWtya:v bNþalmkBIRkcab;epþImBüa)al b¤ Büa)almin)anRtwmRtUv TaMgenH Gacmanplb:HBal;F¶n;F¶reTAelIsuxPaBpøÚvcitþrbs;eK. KwenAdMNak;kalenH ehIy EdlkumarecHEtTTUcsYrnaM«BukmþayeGayBnül;va. enaHehIyEdl «Bukmþay nig RKUeBTü edIrtYnaTIsMxan;kñúgkarBnül;GMBICMgW ehIysMxan;Nas; eTAeTot tYnaTIkñúgkarBnül;[kumarmanCMenOcitþ. RbsinebI«Bukmþay nig 144

RKUeBTü sMerckic©karenH)anedayKμanbgáPaBTMeyI b¤ KMnitGviC¢mandl;kUn enaH/ fñalenHnwgkøayCafñalbNþúHkumar eBjedayCMenOcitþ rhUtdl;v½yCMTg; nig dl;eBjv½y. GñkCMgWekμgCMTg; ³ enHGacfaCadMNak;kald¾lM)akbMput/ CaeBlEdlGñkCMgW v½yekμggayb:HBal;GarmμN_ pøÚvcitþ dUceKniyayBIdMNak;kallM)akqøgBI yuvPaBeTACaeBjv½y. varEmgCadMNak;kalmYyEdlTidæPaBénCIvitsMrab; karRKb;RKgCMgWtaLasesmIumansPaBEhlhalbMput Etk¾CadMNak;kal mYyEdlGñkCMgWcaM)ac;RtUvTTYlsÁal;xøÜnÉgfaxøÜneKman\T§iBlGacRKb;RKg )annUvKuNPaBCIvitrbs;eK. Gakb,kiriyarwgrUsrbs;ekμgCMTg;manCMgWtaLasesmIudUcKñanwgekμgFmμtaEdr. b:uEnþ cMeBaHekμgmanCMgW/ PaBrwgrUsenaHmanrUbPaBCakarCMTas;nwgkarBüa)al CaBiesskarBüa)alkMcat;CatiEdk. «Bukmþay bgb¥nÚ evC¢bNÐti Kilanub,dæakyika RKUeBTücitsþ aRsþ TaMgGs;Kañ mantYnaTIsxM an;kgúñ karpþlk; arKaMRTdl;ekμg CMTg;manCMgWtaLasesmIu edIm,I[eKmanCMenOcitþehIyrIkray TTYlykkar Büa)al edayrMlwkBYkeKBIeRKaHfñak;énkarminGnuvtþn_tamkarENnaMrbs; RKUeBTü. kñúgkic©xitxMelIkTwkcitþ nig KaMRTdl;GñkCMgWekμgCMTg;/ RKUeBTüxøH elIkCa]TahrN_GMBIGñkCMgWmun² EdlTTYl)aneCaKC½ymansuxPaBl¥ man esckþIrIkray EdlTaMgenH)anmkBIkarGnuvtþn_tamkarBüa)al. GñkCMgWCamnusSeBjv½y ³ eBlcUldl;eBjv½y GñkCMgWRtUvRbQmmuxnwg karCMnHcitþfμI. CakarBit/ GñkCMgWrEmgmanCMenOcitþ nig kþIsgÇwmEfmeTot EdlkumarPaBminTan;man. minxusBI«Bukmþay nig RKYsarrbs;eKeT GñkCMgW manPaBxøaMgkøa nig v½yqøatCagmun xøÜneKeFVICaKMrUsMrab;GñkCMgWv½yekμg nig RKYsarrbs;eK. Etya:gNak¾eday eBlEdlGñkCMgWcab;epþImeFVIkarsMercBI ExSCIvit nig GaCIBrbs;Kat; dUcCaTMnak;TMngyUrGEgVg b¤ karsikSabnþeTot karrkkargareFVI GaBah¾BiBah¾ nig lT§PaBénkarksagRKYsar/ eBlenaHtMrUv karénkarBüa)alCMgWrbs;BYkeK )anbgánUvPaBmYhμgya:gxøaMgcMeBaHBYkeK. 145

146

dUecñHGñkCMgWenAEtRtUvkarkarelIkTwkcitþya:gxøaMg edIm,IrkSa[)annUvrebob Büa)alsMrab;BYkeKpÞal; nig sMrab;GñkenACMuvijBYkeK. PaBtwgEtgmYyeTot k¾GacnwgekItmaneLIgRbsinebIGñkCMgWdwgxøÜnfaKat;KμanlT§PaBeFVICamþay b¤ Ca«Bukrbs;eK)an. karRBYy)armÖcMeBaHesckþIsøab; enAEtCamUlehtucMbgbgá[Føak;Twkcitþ CaBiessGñkCMgWeBjv½yEdlmankaryl;dwgBIplvi)akevC¢saRsþrbs;CMgW ehIyGacmankar)at;bg;mitþPkþiedaysarEtCMgW. ral;bBaðalM)aknImYy²Edl ekItmaneLIg eTAtamv½y nig dMNak;kalepSg² TaMgenHsuT§EtTamTarnUvkar KaMRT nig elIkTwkcitþCab;CaRbcaMBIRkumRKYsar mitþPkþi nig buKÁlikRKUeBTü ehIysMxan;bMputenaHKW\riyabf nig karxitxMRbwgERbgCaviC¢manénGñkCMgWxøÜn ÉgpÞal;. CMenOeCOCak;mutmaM PaBcas;TMu EdlGñkCMgWtaLasesmIukMritF¶n; TTYl)an manTMnak;TMngeTAnwgkMritKaMRTEdlBYkeKTTYl)an ehIynwgKμan lT§plGVITaMgGs; ebIKμankarcUlrYmedaypÞal;BIGñkCMgWxøÜnÉgenaHeT )ann½yfa smrPUmienaHminGacQñH)aneLIy. [EtmankarBüa)aledayRtwmRtUv ehIy GacéllkeTA)an rYmCamYynwgkarKaMRTRKb;RKan;/ GñkCMgWPaKeRcInGac Tb;Tl;nwgTidæPaBGviC¢manrbs;CIvitCamYyCMgWraMuér:RbcaMkay ehIyGacbMeBj )annUvral;bMNg RBmTaMgeFVIeGayescþIR)afña nig kþIsubinþkøayCakarBit)an. CaGkusl/ RbeTsPaKeRcInEdlTTYlrgF¶n;F¶redayCMgWtaLasesmIukMritF¶n; suT§EtCaRbeTskMBugGPivDÆn_ EdlenATIenaH GaC£aFrsuxaPi)alfñak;Cati matabitaGñkCMgW RBmTaMgbuKÁlikeBTüGacpþl;karKaMRTdl;GñkCMgW)ansþÜcesþIg Nas; edaysarEtFn;FanenAmankMrit rcnasm<n½§suxaPi)alTn;exSay nig edaysarmanGaTiPaBsuxaPi)alepSg²eTot. tYnaTId¾sMxan;mYyrbs; shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ Kwkar elIkkMBs;bNþasmaKmCatiCMgWtaLasesmIu pþl;karKaMRT nig elIkTwkcitþCa RbcaMdl;GaC£aFrsuxaPi)alfñak;Cati enAtambNþaRbeTsTTYlrgCMgW taLasesmIu kñúgbMNgeFVI[RbeTsTaMgenaHTTYlsÁal;bBaða nig plb:HBal; d¾F¶n;F¶rénCMgWeTAelIsuxPaBsaFarN³ RbsinebIminRKb;RKg b¤ RtYtRtaeGay )anRtwmRtUveT.

CMBUkTI 11 Bt’bEnßm karrs;ran nig KuNPaBCIvit karGPivDÆn_ya:gsem,Im cMeBaHkarEfTaMBüa)alCMgWtaLasesmIukMritF¶n;kñúg ry³eBl 10 -20qñaMcugeRkayenH )aneFVIeGayekInGayukal nig KuNPaB CIvitKYr[kt;sMKal; cMeBaHGñkCMgwEdlGacTTYl)anesvaenaH nig Gnuvtþ)an tamkarENnaMBüa)alrbs;RKUeBTü. eTaHCaenAbNþaRbeTsEdlmankar EfTaMKøInikcMeBaHGñkCMgW)anRtwmRtUvk¾eday k¾plvi)akbNþalmkBIelIsCati Edk enAEtCamUlehtucMbgbgá[GñkCMgWtaLasesmIukMritF¶n;søab;)anEdr. TaMgRbeTsGPivDÆn_ nig kMBugGPivDÆn_ ktþakMNt;karrs;ranrbs;GñkCMgW Kwkar Gnuvtþn_eGay)antamkarBüa)alkMcat;CatiEdkedayfñaM DFO. xN³Edl GñkCMgWenARbeTskMBugGPivDÆn_ RbQmmuxnwglT§PaBTTYl)anfñaM DFO ehIy GñkCMgWenARbeTsGPivDÆn_gayTTYl)anfñaMenH bBaðaecaTeLIgenaH KwRtUvFana [)anfaGñkCMgW)anRbkan;P¢ab;nUvrbbBüa)al eBareBjedaykarlM)ak nig PaBQwcab; ehIyFana[)anfaGñkCMgW)aneRbIfñaM DFO jwkjab;tamkar ENnaM b¤ eT. karRsavRCavecjpSayqñaM 1996 eday eG> BIka nig shkarI )anbgðajfa GRtars;ranekIneLIgx<s;bMput cMeBaHGñkCMgWTTYlfñaM DFO cak;bBa©Últam srésQam )aneRcInCag 225 dgkñúgmYyqñaM Kwfa ebIGñkCMgWcak;fñaM DFO kan;Etticdg GayuKat;kan;EtxøI. sarsMxan; KwRtUvFana[)anfa GñkCMgW)an Rbkan;P¢ab;nUvkarBüa)alkMcat;CatiEdk )ann½yfa RKUeBTü matabita nig RKYsarGñkCMgWmantYnaTIsMxan;kñúgkarKaMRT nig elIkTwkcitþGñkCMgW.

rUbftGñkCMgWmñak;

147

GñkniBn§

qñaM cMnYnGñkCMgW

Modell et al

1982

Zurlo et al

1989

1087

Brittenham et al

1994

Olivieri et al

1994

Giardina et al

1996

Piga et al

1996

257

Bargna-Pignatti

1998

1146

Modell et al

2000

796

Dewis et al

2001

103

GRtars;rankñúgcMeNamGñkCMgWbBa©ÚlQameRcIn ehIyeRbIfñaM DFO kMcat;CatiEdk 25° enAGayu 25qñaM kñúgcMeNamGñkCMgWenAcRkPBGg;eKøs ekIteRkay qñaM 1963 ehIyTTYlkarBüa)alenAmnÞIreBTüCMnaj nig minCMnaj KW karrs;ranmanCIvit)anRbesIrcMeNamGñkEdl )anTTYl DFO eRbItamkMritmFüm elIsBI 4 Rkam¼s)aþh_. 84/2° enAGayu 15qñaM kñúgRkummnusSekItenAqñaM 1965 - 69 Tl;nwg 96/9° enAGayu 15qñaM kñúgRkummnusSekItenAqñaM 1970 - 74 . GñkCMgWTaMgenHTTYlkarBüa)alenAmCÄmNÐl CMnajcMnYn 7 kEnøg. 100° enAGayu 25qñaM cMeBaHGñkCMgWkMcat;CatiEdk)anl¥ Tl;nwg 35° enAGayu 25qñaM cMeBaHGñkCMgWkMcat;CatiEdkmin)anl¥Edl ekItrvagqñaM 1963 - 87/ sßiteRkamkartamdanmYyry³kal edayGñkCMnaj kñúgmCÄmNÐlEtmYy. 91° kñúgRkumTTYlkarkMcat;CatiEdk)anl¥ Tl;nwg 18° kñúgRkumkMcat;CatiEdkmin)anl¥ eRkayBIBüa)alry³ 15qñaM. GñkCMgWTaMgGs;TTYlkarBüa)alenAmCÄmNÐlCMnaj 3 kEnøg. rs;)anGayumFüm 29qñaM cMeBaHGñkCMgWekIteRkayqñaM 1963 ehIyTTYlkarBüa)alenAmCÄmNÐlEtmYy. 66° enAGayu 25qñaM cMeBaHGñkCMgWekItrvagqñaM 1958 ;- 93 ehIyTTYlkarBüa)alkñúgmCÄmNÐlCMnajEtmYy. 82° enAGayu 25qñaM cMeBaHGñkCMgWekItrvagqñaM 1970 - 74 ehIyTTYlkarBüa)alenAmCÄmNÐlCMnaj 7 kEnøg. GRtasøab; 50° munGayu 35qñaM kñúgcMeNamGñkCMgWenAcRkPB Gg;eKøs EdlTTYlkarBüa)alenAmCÄmNÐlCMnajpg nig minCMnajpg. 78° enAGayu 40qñaM cMeBaHRkummnusSsuT§EtekItenAqñaM 1957 - 97 ehIy 100° enAGayu 25qñaM cMeBaHGñkCMgWekIteRkayqñaM 1974 . GñkCMgWTaMgGs;TTYlkarBüa)alenAmCÄmNÐl CMnajEtmYyenAcRkPBGg;eKøs.

(Advances in experimental medicine and biology, volume 509, p125)

148

taragxagelIenHbgðajlT§plénkarsikSaPaKeRcIn EdleFVIeLIgenAry³eBl Cag20qñacM gu eRkay eTAelIkarrs;ranmanCIvti kñgú CMgtW aLasesmIku rM ti F¶nF; r¶ . Tinñn½yenHbBa¢ak;fa GñkEdlekItCMgWtaLasesmIukMritF¶n; ehIyTTYlkar Büa)al)anl¥BIdMbUgTI PaKeRcInGñkekIteRkayqñaM 1974 eBlEdlcab;epþIm mankaryl;dwgBIkareRbIR)as;)anl¥fñaM DFO ehIyGñkCMgWTTYl nig Gnuvtþn_ )antamkarBüa)alRtwmRtUvmk GñkCMgWTaMenaHman»kasrs;)anyUrehIyman dMeNIrCIvit)anl¥. ktþaKnøwHeFVI[RbesIrcMeBaHkarpþl;esva nig dMeNIrCIvit rbs;GñkCMgWtaLasesmIukMritF¶n; enAbNþaRbeTsCaeRcIn KwkarbegáIt[man mCÄmNÐlevC¢saRsþbEg¥k CalkçN³saFarN³mYy EdlGñkCMgWGacTTYl )ankarBüa)almanKuNPaBl¥bMput. ya:gNak¾eday enAmankargarCaeRcIn eTotRtUveFVIenAtambNþaRbeTskMBugGPivDÆn_mYycMnYn edIm,IelIkkMBs;sþg;da ¬niyam¦ EfTaMKøInikGñkCMgWtaLasesmIu . bBaðaepSg²eTot EdlGacCakarRBYy)armÖrbs;GñkCMgW k¾dUcCamatabita GñkCMgW KWTak;TgnwgbBaðaGaBah¾BiBah¾/ RkumRKYsar/ karhUbcuk nig skmμPaB ragkay. rbbGahar nig taLasesmIu CaTUeTAGñkCMgWtaLasesmIu mincaM)ac;jaMucMNIGaharBiessGVIeT. b:uEnþ GñkCMgW RtUveCosvagGaharsMbUrCatiEdk. CakarRbesIrKYreCosvagsura b¤ pwkkñúg kMrittictYc eRBaHeføImrbs;eKgayTTYlrgkarb:HBal; müa:gedaysareføIm GñkCMgWtaLasesmIupÞúkCatiEdkeRcIn ehIymüa:geTotGacfamanCMgWrlakeføIm ¬sUmGanGMBIcMNIGaharkñúgCMBUkBiess¦. kILa nig taLasesmIu GñkCMgWtaLaesmIuGaccUlrYmkñúgkILaCaeRcInRbePT GaRs½yeTA elIsPaBKøInikrbs;GñkCMgW nig karENnaMrbs;RKUeBTü . 149

rUbftGñkCMgWmñak;CamYykUn rbs;nag EdlmansuxPaBl¥

150

visSmkal nig taLasesmIu shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ )ancgRkgbBa¢IeQμaHGñkCMnaj evC¢saRsþ nig mCÄmNÐlevC¢saRsþCMuvijBiPBelak ¬Gacrk)antameKhTMB½r rbs; s>G>t¦ EdlGacpþl;esvaBüa)alCUnGñkCMgWtaLasesmIukMritF¶n;)an. munnwgeFVIdMeNIreTANa/ GñkCMgWKYrdwgTItaMgmCÄmNÐlevC¢saRsþEdlenAEk,r² nwgkEnøgEdlxøÜncg;eTA ehIyKYrTak;TgCamYyRKUeBTüBüa)alenAmCÄmNÐl enaHmunnwgecjBIlMenAdæaneTA. GñkCMgWRtUvR)akdfa xøÜn)anTTYlkarcak;fñaM bgáarcaM)ac;TaMgGs; ¬Canic©kal RtUvRbwkSaCamYyevC¢bNÐitrbs;xøÜn¦ munnwg eTATsSnaRbeTsNamYy ehIyRtUvyl;dwgBIGRtaCMgWqøgBiessNamYy enAtMbn;EdlxøÜneRKageTATsSna ¬sUmGankarENnaMGMBIkareFVIdMeNIr Edlman pSayelIeKhTMB½rrbs;GgÁkarsuxPaBBiPBelak¦. ral;fñaMEdlcaM)ac; sMrab; rbbBüa)alrbs;GñkCMgW rYmmanfñaMpSH nig sMPar³s¥atKμanemeraK RtUvevcx©b; edayykcitþTukdak; ehIydak;kñúgkabUb b¤ va:lIkan;enAnwgéd. cMeBaHGñkCMgWEdleRKageTAelgtMbn;PñMx<s;² RtUvman eGm:UkøÚb‘Ín kMritFmμta ehIyGñkCMgWxøÜnÉgpÞal;RtUvman»kashVwkhat;pSaMunwgry³kMBs;x<s;Camun sin. dUecñHGñkCMgWRtUvkMritxøÜnÉg kMueTAtMbn;NaEdlmankMBs;x<s;Cag 11000hVIteLIy b¤k¾RtUv[c,as;fa xøÜnRtUvbBa©ÚlQamPøam munnwgecj dMeNIreTAry³kMBs;x<s;. eRkABIenH KμankarhamXat;BITIkEnøgeFVIdMeNIreT. karGaBah¾BiBah¾ nig RKYsar BitNas;GñkCMgWtaLasesmIukMritF¶n; GacerobkarehIymankUn)an. kUnrbs; eKmansuxPaBya:gNaenaH sßitenAelIsßanPaBsuxPaBénédKUrbs;eK )an esckþIfa édKUmansuxPaBl¥Fmμta/ manpÞúkCMgW b¤ manCMgWtaLasesmIuEtmþg. RbsinebIGñkCMgWmñak;erobkarCamYyGñkCMgWtaLasesmIumñak;epSgeTot kUnTaMg Gs;rbs;BYkeKnwgmanCMgWEdr. RbsinebIGñkCMgWmñak;erobkarCamYyGñkpÞúk CMgWEbtta taLasesmIu 50° énkUnekItmkmanCMgW ehIy 50° eTotCa

pÞúkCMgW . müa:geTotRbsinebIGñkCMgWmñak;erobkarCamYyGñkFmμtaKμanpÞúkCMgW enaH 100° kUnekItmkRKan;EtCaGñkpÞúkCMgWb:ueNÑaH. segçbmk GñkCMgWEdlGnuvtþ)antamkarENnaMrbbBüa)al GacmanCIvitrs; enAsuxsb,ayesÞIrEtFmμta. ]TahrN_ enARbeTs sIuRBUs GñkCMgW taLasesmIu 83°)anbBa©b;karsikSaCan;x<s; ehIy 25° eTot)anTTYl sBaØabR½tsalkviTüal½y. GñkCMgW 22° )anerobkar kñúgenaH 73° man kUn xøHmankUn 3 b¤ 4nak;. GñkCMgW 79° enA sIuRBUs Rbkbkic©karkñúgEpñk sib,kmμ nig ksikmμ/ CaelxaFikar/ CaRKUbeRgon/ CaKilanub,dæak-yika/ CabuKÁlikevC¢saRsþ b¤ GmevC¢saRsþ.

151

CMBUkTI 12 eGBIedmIsaRsþ nig karkarBarCMgWtaLasesmIu

affected by 12a Countries malaria before establishment of control programmes

12b

Malaria parasites (trophozoites of P. falciparum in red cells)

152

eGBIedmIsaRsþ BImuneKKitfa taLasesmIuCaCMgWmanedImkMeNItenAEttMbn;smuRTemDIETra:eN eT/ b:uEnþbc©úb,nñenH eKdwgfa vamanenABaseBjRKb;tMbn;CaeRcInén BiPBelak. eKeXIjmantaLasesmIuenABaseBjGWr:ubxagt,Úg cab;BI RbeTs B½rTuykal; eTAdl;RbeTseGs,a:j/ GIutalI nig Rkic nig RbeTsGWr:ubkNþalmYycMnYn RBmTaMgEpñkxøHénGtItshPaBsUevot. taLasesmIuekItmanpgEdrenAtMbn;mCÄwmbUBa’rhUtdl;GIur:g; )a:KIsßan/ \NÐa/ bg;køaeds/ éf/ ma:eLsIu/ \NÐÚeNsIu nig RbeTscinPaKxagt,Úg RBmTaMgRbeTsenAtameqñrPaKxageCIgénTVIbGaRhVik nig enAGaemrik xagt,Úg. taLasesmIumanGRtaQWCaBiess enAtamtMbn;Edlman b¤ Føab;manCMgW RKuncaj; ¬emIlrUb 12a¦. )a:ra:sIutCMgWRKuncaj; KWCaPñak;garbgáCMgWmYyEdl cMlgeTAmnusStamry³mUsEdkeKalxaM ehIybgáCaCMgW edaybMpøajeKalika QamRkhm ¬emIlrUb 12b¦. eKKitfa enAtMbn;EdlFøab;manCMgWRKuncaj; srIragÁkayrbs;mnusSmanbMErbMrYltUcmYyelIBn§ú EdlCaKuNRbeyaCn_d¾ RbesIrCagGñkEdlKμanbMErbMrYlBn§ú. karenHeFVI[mankarpøas;bþÚrdl; briyakaseKalikaQamRkhmEdleFVI[)a:ra:sIutCMgWRKuncaj;minGacmanCIvit nig ekItkUnekItecAteTAeTot)an. sPaBpøas;bþÚrenHnaMeGayekItCMgW Ebtta taLasesmIukMritRsal. eKeCOfa minxusBICMgW Gal;hVa taLasesmIu nig CMgW Sickle cell Edr/ GñkCMgW Ebtta taLasesmIukMritRsal suT§EtmanlT§PaB Tb;Tl;CMgWRKuncaj; RbesIrCagbuKÁlsuxPaBFmμta KWfa kñúgry³eBlCaeRcIn qñaMmkenH kñúgbNþatMbn;enATUTaMgBiPBelakEdlmanCMgWRKuncaj; cMnYnGñkCMgW

Ebtta taLasesmIukMritRsal )anekIneLIgya:gxøaMg xN³EdlbuKÁlsuxPaB FmμtaCaeRcIn)ansøab;edaysarCMgWRKuncaj;F¶n;F¶r. eTaHbIkñúgbNþaqñaM cugeRkayenH kmμviFIbM)at;CMgWRKuncaj; )aneFVIeGayFøak;cuHya:gKMhuk énkrNI CMgWRKuncaj;kñúgtMbn;CaeRcInénBiPBelakk¾eday k¾dMeNaHRsayCMgW taLasesmIu nig CMgWeGm:UkøÚb‘ÍnkMritF¶n;epSg²eTot enAEtCabBaðaRbQmKYr[ kt;sMKal;dEdl. kareFVIcMNakRsukrbs;RbCaBlrdæ nig GaBah¾BiBah¾rvag Catisasn_xus²Kña )annaMykCMgWtaLasesmIu eTAesÞIrEtRKb; RbeTsTaMgGs;kñúgBiPBelak dUcCa GWr:ub xageCIgEdlBImun mkminEdlmanCMgWtaLasesmIuesaH Etbc©úb,nñenH )ankøay eTACabBaðasuxPaBsaFarN³d¾cMbgmYy. tamRbPBKYreGayTukcitþ)an)a:n;RbmaNfa RbEhl 1/5° énRbCaBlrdæTUTaMgBiPBelak KW 80 - 90 lannak;CaGñkpÞúkCMgW Ebtta taLasesmIu ehIyCaerogral;qñaM mankumarRbmaN 60>000nak; ekItBImþay mkmanCMgWenH/ mYyPaKFMsßitenARbeTskMBugGPivDÆn_. eKdwgc,as;fa tYelx enHCakarvaytMélTab edaysarfamanTinñn½yBitR)akdtictYcNas;EdleK Gacrk)ansþIBIGRtapÞúkCMgW Ebtta taLasesmIu ¬edaykarBinitüBn§ú¦ kñúg RkumRbCaBlrdæCaeRcIn CaBiesskñúgtMbn;énBiPBelakEdleKsÁal; b¤ rMBwgfa nwgTTYlkarb:HBal;ya:gF¶nF; r¶ . enATUTaMgBiPBelak/ tamÉksarrbs; s>G>t )an[dwgfamanEtGñkCMgW taLasesmIukMritF¶n; RbmaNCa 20>000nak; b:ueNÑaH enAmanCIvitehIy)ancuHbBa¢IreQμaHfa)anTTYlkarBüa)al karenHKUs bBa¢ak;nUvkarBitd¾CUct;fa mYyPaKFMénkumarEdlTTYlrgCMgWenHekItenARbeTs kMBugGPivDÆn_ søab;edayBMu)anTTYlkareFVIeraKvinicä½y b¤ eFVIeraKvinicä½ymin)an RtwmRtUv TTYlkarBüa)almin)anl¥ b¤ min)anTTYlkarBüa)al. EpnTIenH ¬emIlrUb 12c¦ bgðajBIRbeTsEdlb:HBal;eday CMgW Ebtta taLasesmIu. ebIeKKitbBa©ÚlCamYyCMgWeGm:UkøÚb‘ÍnF¶n;F¶repSgeTot dUcCaCMgW

12c

(From TIF’s “Guidelines for the Clinical Management of Thalassaemia”

153

nig HbE/β - taLasesmIu/ enaHmanRbmaN 5° énRbCaCn TUTaMgBiPBelakTTYlrgCMgWEbbenH. karkarBar taLasesmIu KWCaCMgWmYyGackarBar)an. manRbeTsmYycMnYn )anbgðajPaBeCaKC½yya:gc,as; dUcCa GIutalI/ Rkik nig sIuRBYs EdlknøgmksuT§EtsßitkñúgcMeNamRbeTsTImYyEdlbegáIt)an kmμvFi CI atiRbkbedayeCaKC½y edaykat;bnßy)anKYr[kt;sKM al; nUvkMeNItkumarmanCMgWtaLasesmIu ehIykrNIxøHRtUv)anbnßy dl;cN M cu esÞrI EtKμan. pÞyú eTAvijenAcRkPBGg;eKøs knøgmkFøab; manGnuvtþkmμvFi kI arBar EtKaμneKalneya)ayfñak;CatiseM dAkarBar eT GRtakMeNItkumarmanCMgW)anFøak;cuHEt 50° b:ueNÑaH ¬emIlrUb 12d¦ . bc©úb,nñenH TidæPaBKnøwHénkmμviFIkarBarRbkbedayeCaKC½ybMput)anbegáIt CamUldæansMrab;kmμviFIenAbNþaRbeTsepSg²eTot EdlTTYlrgCMgW. TaMgenHman ³ Tak;TajqnÞH nig karebþC£acitþrbs;rdæaPi)al begáItnUvyuT§nakarGb;rMsuxPaBRbkbedayfamBleday pþl;karyl;dwgdl;mRnþIsuxaPi)al nig saFarN³CnTUeTA. begáItmnÞIrBiesaFn_RbkbedayKuNPaBedIm,I ³ - BinitüQamedIm,IRsavRCav - eFVIeraKvinicä½yelITarkñúgépÞ elIkkMBs;esvaEpñksmÖB nig Bn§ú sarsMxan;énkarkarBar tamGgÁkarsuxPaBBiPBelak)an[dwgfa karcMNayRbcaMqñaMénkmμviFIkarBar fñak;CatimYyenAbNþaRbeTsCaeRcIn esÞIrEtesμInwgtMélBüa)alsMrab;ry³eBl mYyqñaM cMeBaHGñkCMgWmYyRkumEdlcab;kMeNItkñúgmYyqñaM. Sickle cell

12d

(WHO/HGN/WG/00.1 2000)

154

tMélcMNaysMrab;karBarRbcaMqñaMenAefrCab;rhUt ÉtMélcMNaycMeBaH karBüa)alvijekIneLIgBImYyqñaM eTAmYyqñaM ¬emIlrUb A¦ dUecñHplcMeNj ecjBIkmμviFIkarBarekIneLIgCaerogral;qñaMeBlGnuvtþkmμviFI. GgÁkarsuxPaB BiPBelak)anbgðajfa ebIKμankmμviFIkarBaredIm,IkMritcMnYnkMeNItkumarman CMgWeTenaH RbeTsPaKeRcInnwgBMuGacmanlT§PaBéllkpþl;karBüa)al[ )anl¥ dl;RKb;GñkCMgWtaLasesmIu)aneLIy. dUecñH kmμviFIkarBarRbkbeday RbsiT§iPaB mansarsMxan;Nas;edIm,ITuklT§PaBcMNayelIkarBüa)aleGay )anl¥dl;GñkCMgWEdlmanRsab; ¬emIlrUb A¦. rebobEdltaLasesmIucMlgCalkçN³tMNBUC dUc)anCMrabkñúgCMBUkTImYy/ Ebtta taLasesmIukMritRsal cMlgBI «BukmþayeTAkUntamrebob GUtUsUm:al er:esssIuv ¬Autosomal recessive¦ )ann½yfa kumarTTYlBn§úbgáCMgW EtmYyBI«Buk b¤ BImþay ¬ebITTYlBn§úbgáCMgWTaMgBIr BImþaypg nig «Bukpg enaHbgáCasPaB KønI ki F¶nF; r¶ ehAfa taLasesmIku rM ti F¶n¦; . eBlEdlTarkcab;keM NIt kñúgépÞ vaTTYlCalkçN³tMNBUC Bn§úEbttakøÚb‘Ín ¬β-globin gene¦ mYy BI«Buk nig mYyeTotBImþay. eBlNaTaMg«BukTaMgmþaypÞúk Bn§úEbttakøÚb‘Ín Fmμta kUnnwgTTYlCalkçN³ tMNBUC Bn§úEbttakøÚb‘Ín TaMgBIrFmμta dUcbgðaj kñúgrUb. eBlNamñak;kñúgcMeNam«Bukmþayman Bn§úEbttakøÚb‘Ín manCMgW )anesckþIfa Kat;KWCaGñkpÞúk Ebtta taLasesmIu ehIymñak;eTotman Bn§úEbttakøÚb‘Ín FmμtaKμanCMgW kUnnImYy²ekItmkmanmñak;kñúgBIrnak; b¤fa 50° man»kas TTYltMNBUCBn§úCMgWtaLasesmIu BI«Buk b¤ mþay EdlmanpÞúkBn§ú QW dUcbgðajkñúgrUb. kUn²TaMgenH GacCa ³ [i] GñkpÞúk Ebtta taLasesmIukMritRsal/ b¤ [ii] Ebtta taLasesmIu eGetr:UhSIúháÚt/ b¤ [iii] Ebtta taLasesmIukMritRsal 155

GñkpÞúktaLasesmIukMritRsal GñkpÞúk taLasesmIukMritRsal minmanCMgWGVIeT. eKBMumaneraKsBaØapøÚvkay b¤ pøÚvcitþeLIy ehIymintMrUveGaymanrbbGaharBiess b¤ karBüa)al evC¢saRsþeLIy. karvivtþn_eTAGnaKt/ sPaBenHminGackøayeTACaCMgWF¶n;F¶r eLIy/ tamBiteTAebImin)aneFVIetsþBiesssMrab;CMgWenHeT PaKeRcInnwgmin)an dwgfa BYkeKmanpÞúkCMgW. ya:gNak¾edayGñkpÞúkxøH GacmansPaBesøksøaMg bnþicbnþÜc EdlGac[eKeFVIeraKvinicä½yedayPan;RcLMfaCaCMgWxVHCatiEdk. kareFVIetsþmnÞIrBiesaFn_ Gac[eKeFVIkarEbgEckkrNITaMgBIr)anedaygay. RsþICaGñkpÞúktaLasesmIu kalNamanépÞeBaH GacmansPaBesøksøaMgkMrit mFümEdlGacFUres,Iyvij edaypþlC; atiEdkbEnßmkñgú GMLgú eBlmanépÞeBaH. ehtuGVIGñkcaM)ac;RtUvdwgfa etIxøÜnGñkCaGñkpÞúkCMgW b¤ Gt; fVIebIGñkpÞúktaLasesmIukMritRsal BMumanplb:HBal;suxPaBk¾eday EtebIGñk pÞúkmñak;mankUnCamYyGñkpÞúkmñak;eTot enaHkñúgkUnbYnnak; manmñak; b¤ faman »kas 25° nwgmanCMgWtaLasesmIukMritF¶n;. dUcrUbPaBbgðajRsab;/ eBlEdl«BukmþayTaMgBIrCaGñkpÞúkCMgW ral;eBlmanépÞeBaH kUnenaHman»kas 1¼4 ¬25°¦ nwgmanCMgWtaLasesmIukMritF¶n;/ man»kas 1¼2 ¬50°¦ CaGñkpÞúktaLasesmIukMritRsal ehIy 1¼2 ¬25°¦ man»kasminekItCMgWesaH. eGm:UkøÚb‘ÍnxusFmμta nig CMgWeGm:UkøÚb‘ÍnepSg²eTot eK)ansÁal;eGm:UkøÚb‘ÍnmnusSFMEdlCaRbePT {xusFmμta} mYycMnYnepSgeTot Edlmanrcnasm<½n§xusFmμta nig sPaBKøInikEsþgecjxus²Kña. eKsÁal; eGm:UkøÚb‘ÍnxusFmμta TaMgenHfaCaeGm:UkøÚb‘ÍnEdlmanrcnasm<½n§køay rYmman HbS, HbE, HbC, HbH, HbD nig Hb Lepore. eGm:UkøÚb‘Ín TaMgenHcMlg CalkçN³tBUCdUcKñanwg Ebtta taLasesmIuEdr. ya:gNak¾eday manEt 156

GñkTTYlcMlg Hb Lepore nig HbS BI«Buk nig mþayb:ueNÑaH EdlmansPaB KøInikF¶n;F¶r eday Hb Lepore mansPaBdUcKñanwgCMgW Ebtta taLasesmIu BIkMritmFüm eTAF¶n;F¶r/ ehIy HbS bgáCMgW Sickle cell EdlCaCMgWeGm:UkøÚb‘Ín mYyF¶n;F¶r epSgBI Ebtta taLasesmIukMritF¶n; TaMgsPaBKøInikEsþgecj nig TaMgkarEfTaMevC¢saRsþ. cMeBaH eGm:UkøÚb‘ÍnxusFmμtaepSgeTot HbC, HbD b¤ HbE KμanEsþgecjCaCMgWeT eTaHCacMlgtBUCBI«BukmþayTaMgBIrk¾eday. ya:gNak¾eday eGm:UkøÚb‘ÍnkøayTaMgenHGacpÁúMKñaCamYy Ebtta taLasesmIu kMritRsal begáItCaCMgWelahit EdlmanrUbPaBKøInikmankMritF¶n;F¶repSg²Kña. β - taLasesmIu HbE/β HbE Ca eGm:UkøÚb‘ÍnxusFmμtamYyeXIjmanTUeTACageK CaBiesskñúgcMeNam RbCaBlrdæEdlmanExSRsLayenAtMbn;GasIuGaeKñy_ dUcCa km<úCa/ evotNam/ éf. ebIsinmñak;kñúgcMeNam«Bukmþay CaGñkpÞúkEbtta taLasesmIu kMritRsal ehIymñak;eTotCaGñkpÞúk HbE eBlmanépÞeBaHmþg² man»kas 25° kUnekItmkmanCMgW HbE/β - taLasesmIu. CMgW HbE/β - taLasesmIu CaCMgWkgVHeKalikaQamRkhmkMritFn;bgÁÜrmYy EdleraKsBaØa EtgEtmansPaBRsedogKñanwgCMgW Ebtta taLasesmIukMrit mFüm b:uEnþvaGacmansPaBF¶n;F¶rdUc CMgWtaLasesmIukMritF¶n;Edr. sPaB KøInikEsþgecj nig karEfTaMevC¢saRsþénCMgW HbE/β - taLasesmIu manEcgkñúgesovePAenH. Hb Lepore/β - taLasesmIu karpÁúMcUlKñaén Hb Lepore CamYy Ebtta taLasesmIu EsþgecjCasPaB KøInikmYyF¶n;F¶r dUcKñanwg Ebtta taLasesmIukMritF¶n; ehIycMlgCalkçN³ tBUCdUckarerobrab;kñúgCMgW HbE/β - taLasesmIu xagelIEdr. 157

- CMgWtaLasesmIu ebIsinmñak;kñúgcMeNam«Bukmþay CaGñkpÞúk Ebtta taLasesmIu kMritRsal ehIymñak;eTot CaGñkpÞúk HbS man»kas 25° kUnekItmkmanCMgW Hbs/β - taLasesmIu. HbS eXIjmanTUeTAelICnCatiGaRhVik b¤ GñkCab;ExSRsLay BItMbn;emDIETra:eN. sPaBF¶n;F¶rénCMgW ERbRbYleTAtam brimaN EbttakøÚb‘ÍnFmμta ¬normal β-globin¦ Edlpliteday Bn§úEbtta ¬β-gene¦. eBlNa Bn§úEbtta minplit EbttakøÚb‘Ín )aneT sPaBCMgW esÞIrEtdUcKñaebHbiTnwgCMgW Sickle cell. eBlNa Bn§úEbtta plit EbttakøÚb‘Ín )anxøH sPaBCMgWmanlkçN³minsUvF¶n;F¶reT. Bt’mankñúg esovePAenH minniyayGMBICMgWenHeT Bt’manbEnßmmanelIeKhTMB½r rbs;GgÁkarsuxPaBBiPBelak. karpÁúMeGm:UkøÚb‘ÍnkøayepSgeTot GaccMlgCalkçN³tBUCEdr mandUcCa β/Hb Lepore nig HbO Arab/β ¬TaMgBIrenHRsedogKñanwgCMgWtaLasesmIukMrit mFüm¦ehIynig HbS/HbC, HbS/HbD Punjab nig HbS/HbO Arab TaMg Gs;enHmansPaBRsedogKñanwgCMgW Sickle cell EdlmankMritF¶n;F¶repSg²Kña. Gal;hVa taLasesmIu HbS/β

Normal alpha globin genes chromosomes 16

158

Chromosome a-thalassaemia minor on the same chromosome

Chromosome a-thalassaemia minor on opposite chromosomes

slient carrier

[i]

CMgW Gal;hVa taLasesmIu ¬a - thalassemia¦ xusEbøky:ag xøaMgBICMgW Ebtta taLasesmIu ¬β - thalassemia¦. Bt’man kñúgesovePAenH BMuniyayGMBIbuKÁlpÞúk Gal;hVa taLasesmIueT. Bt’manbEnßmsþIGMBICMgWGal;hVa taLasesmIu manenAelIeKhTMB½r rbs;GgÁkarsuxPaBBiPBelak. ya:gNak¾eday Bt’manCa mUldæanxøHsþIBIeGBIedmIusaRsþ nig lkçN³cMlgbnþBUCénCMgWenH manCUnenATMB½rbnÞab;. [ii] enAkñúgBiPBelakmanGñkpÞúk Gal;hVa taLasesmIu cMnYn 260lanGñk krNIx<s;bMputmanenA \Nða/ GasIuGaeKñy_ nig GaRhVik ehIymanEtbnþicbnþÜcenAtMbn;emDIETra:eN ehIyman edaykMrenAGWr:ubxageCIg. pÞúyBI EbttakøÚb‘Ín/ Gal;hVakøÚb‘ÍnekIteLIgBIBn§úcMnYnbYn EdlenA mçagBIrelIExSTaMgsgxagénRkUm:UsUmTI 16. Gal;hVataLasesmIukMritRsal pÁúMtamlkçN³epSg² edIm,I [iii] [ekItCaCMgWelahitBIkMritRsaleTAF¶n;F¶r. bNþMúeraKsBaØa Hydrops foetalis én Hb Bart ¬emIlrUb ii¦ CaCMgW Gal;hVa taLasesmIukMritF¶n;CageKbMput KWCasPaB GUm:UhSIúháÚt ¬Homozygous state¦ sMrab;CMgW ao taLasesmIu )anesckþIfa Bn§úGal;hVakøÚb‘Ín ¬a-globin genes¦TaMgbYnmindMeNIrkar ehIyKμanplitExSRbUetGIun Gal;hVa ¬a-chains¦ eT. lkçN³enH bgá[mankgVHRKab;QamRkhmF¶n;F¶r naM[TarkkñúgépÞsøab;. eTaHbIsPaBKøInikEsþgecjmkmansPaBxus²KñaeRcInk¾eday k¾buKÁlmanCMgW HbH ¬emIlrUb iii¦ mYyPaKFM mansuxPaBl¥Fmμta. TMrg;EtmYyKt;énCMgW HbH EdlekItmanedaykMr ehIymansPaBF¶n;F¶r KW Hydrops foetalis. 159

sarsMxan;én Gal;hVa taLasesmIu kñúgdMeNIrKøInikén Ebtta taLasesmIu vtþmanCMgW Gal;hVa taLasesmIukMritRsal mansar³sMxan;cMeBaHGñkCMgW Ebtta taLasesmIu GUm:UhSIúháÚt edaysar Gal;hVa taLasesmIuenH GacCYy bnßyGtulüPaBrvagExSRbUetGIun Gal;haV nig Ebtta EdlCakareFVI[sPaBKøInikCMgW Ebtta taLasesmIueTACaRsal. EsVgyl;fa etIrUbGñkCaGñkpÞúk b¤ Gt;eT karpþl;RbwkSaGMBIkarBinitüBn§ú krNIPaKeRcIn etsþmnÞIrBiesaFn_samBaØGac[dwg)anfabuKÁlmñak;CaGñkpÞúk taLasesmIukMritRsal b¤ ya:gNa. ya:gNak¾eday munGnuvtþetsþmnÞIr BiesaFn_NamYy sMxan;buKÁlenaHRtUvTTYlkarpþl;RbwkSaGMBIkarBinitüBn§ú ebIGaceFVIeTA)an edaypþl;CUnKat;nUvBt’man dMbUnμan karENnaMsþIBImUlehtu EdlRtUveFVIetsþ ehIyniglT§pletsþmann½yya:gNacMeBaHKat;. müa:gvijeTot karpþl;Bt’manTaMgenHRtUvEp¥kelIkmμviFIGb;rMsuxPaBeGayl¥. Gñkpþl;RbwkSanwgTTYlkarhVwkhat;Biess edIm,ImanlT§PaBBiPakSaTidæPaB sMxan;² GMBIkarkarBar nig GMBICMgWpgEdr rYmman ³ TIkEnøgRtUveFVIetsþQam rebobbkRsaylT§pletsþQam Gtßn½yBaküfa GñkpÞúk/ rYmmandak;CMerIsCUndl;GñkpÞúkBIrnak; Edlman KMeragmankUnCamYyKña b¤ dak;CUndl;GñkmanépÞeBaHrYceTAehIy )anesckþIfa KUrRskrRbQmmuxnwgeRKaHfñak;. FmμCati nig karBüa)alCMgWtaLasesmIukMritF¶n; b¤ CMgWeGmU:køÚb‘ÍnepSg NamYyeTot b¤ CMgWBn§ú. Gñkpþl;RbwkSaRtUvpþl;Bt’mandl;buKÁl nig KUrRskr eday[BYkeKeFVIkar sMercciteþ dayÉkÉg BIvFi EI dlBYkeKR)afñaGnuvtþn._ ya:gNak¾edaydMbnU aμ n pþl;edayGñkRbwkSa nig karsMerccitþrbs;BYkeK rEmgTTYl\T§iBlBICMenO

160

sasna nig vb,Fm’. karecjpSayrbs; s>G>t {karkarBarCMgW taLasesmIu nig CMgWeGm:UkøÚb‘ÍnepSg²} PaKTI1 Gacpþl;dl;GñkGan nUvBt’manCMnajsþIBIbBaðaTaMgenH. kareFVIetsþmnÞIrBiesaFn_edIm,IkMNt;GñkpÞúk Ebtta taLasesmIukMritRsal etsþmnÞIrBiesaFn_sMrab;CMgWtaLasesmIu rYmmanetsþQamEdleKeFVICaRbcaM eKehAfa karrab;ya:geBjeljénRKab;Qam ¬CBC = Complete Blood Count¦ Edlmankarvas;kMriteGm:UkøÚb‘Ín nig rgVas;epSg²eTot Tak;TgnwgcMnYn nig maDeKalikaQamRkhmEdleK[eQμaHfa {TMhMmFümrbs;eKalika QamRkhm} ¬MCV = Mean Corpuscular Volume¦ nig { TMgn;mFümén eGm:UkøÚb‘ÍnkñúgeKalikaQamRkhmnImYy²} ¬MCH = Mean Corpuscular Haemoglobin¦. ]TahrN_ mnusSeBjv½y ebI MCV ticCag 75 GacbBa¢ak; )anfa CaGñkpÞúkCMgW ¬Etvak¾GacbBa¢ak;BIsPaBxVHCatiEdkEdr EdletsþepSg eTotnwgEjkkrNInImYy²)an¦. MCV GacmankMritTabcMeBaHkUnekμg ehIy ERbRbYleTAtamGayu. eKBinitüeKalikaQamRkhm edaymIRkUTsSn_pgEdr edIm,IdwgTMhM nig rUbragrbs;va. eKalikaQamRkhmrbs;GñkpÞúktaLasesmIu manragRsemalRkhmesøk ehIymanrUbragreqtrqUt ¬Poikilocytosis¦¬emIlrUb 12f¦ xusBIeKalikaQamRkhmFmμta manBN’RkhmRkem:A ragmUlptkNþal ¬emIlrUb 12e¦. rgVas;RKab;Qam rbs;mnusSFmμtaTUeTA nig GñkpÞúkEbtta taLasesmIukMritRsal mnusSFmμta GñkpÞúkEbtta taLasesmIu Hb

135 g/dl

142 g/dl

HCT

42%

HCT

42,5%

MCV

88,8 fl

MCV

61,2 fl

MCH

28,5 pg

MCH

20,5 pg

MCHC

32,1 g/dl

MCHC

33,4 g/dl

RDW-CV

13,1%

RDW-CV

15,9%

5,3%

161

TM r g; e pSg² énEbtta taLasesmI u kgVHeKalika TMhMeKalikaQamRkhmtUc² QamRkhm (Microcytosis) (MCV < 75fl)

12f

HbF

TMrg;c,as;

0-+

> 3,5%

1 - 5%

βδ

0-+

< 3,5%

5 - 10%

Lepore

0-+

< 3,5%

1 - 5%

< 3,5%

< 2%

GñkpÞúkCMgWkñúg sPaBes¶oms¶at;

12e

HbA2

Blood film from a non-carrier (normal)

Blood film from a β-thalassaemia carrier

162

epSg²

5 - 10% Hb Lepore

ebIetsþmnÞIrBiesaFn_t²eTAeTot [dUcCa lT§PaBP¢ab;CatiEdksrub ¬TIBC = Total Iron Binding Capacity¦ nig Ferritin] Gac[eKTat;ecalnUvkgVHCati EdkEdlbgá[ MCV fycuH enaHeKGnuvtþetsþbEnßmeTot edIm,IbBa¢ak; taLasesmIukMritRsal nig kMNt;RbePTrbs;va. etsþedIm,IkMNt; Ebtta taLasesmuIkMritRsal KWCadMeNIrmYyEdleKehAfa karEjkRbePT eGm:UkøÚb‘ÍnedaycrnþGKÁisnI ¬Hemoglobin Electrophoresis¦ EdlGac[ eKvas;brimaN HbA EdlsarFatupSMcMbgrbs;eGm:UkøÚb‘ÍnmnusSFM nig brimaN HbA2 EdlmanEtbnþicbnþÜc. eGm:UkøÚb‘ÍnepSgeTotEdlmanCaFmμtakñúg RKab;QamRkhmmnusSFM dUcCa eGm:UkøÚb‘ÍnTarkkñúgépÞ (Foetal hemoglobin = HbF) Gacvas;)anEdreday viFIEjkedaycrnþGKÁisnI ¬Electrophoresis¦. krNIPaKeRcInetsþxagelIvaRKb;RKan;edIm,IkMNt;buKÁl CaGñkpÞúkCMgW. cMeBaH Gal;hVa taLasesmIukMritRsal eKEtgEtdwg)aneRkayBITat;ecal bBaðaepSg²EdleFVIeGay MCV fycuH )anesckþIfa KμankgVHCatiEdk/

lT§plFmμtacMeBaHkarEjkRbePTeGm:UkøÚb‘ÍnedaycrnþGKÁisnI/ ehIyKat;man edImkMeNItBICnCatiEdlsMbUrGñkekItCMgWenH/ TaMgenHGacsnμt;)anfa Kat;Ca GñkpÞúk Gal;hVa taLasesmIu. kñúgkal³eTs³xøH eKRtUvGnuvtþetsþ DNA edIm,IkMNt;vtþman b¤ Gvtþman Ebtta b¤ Gal;hVa taLasesmIukMritRsal. etsþBn§úEbbenH kMBug)anRtUveKeRbIR)as;ya:gTUlMTUlayeLIgsMrab;kMNt; taLasesmIukMritRsal. nrNaxøHKYreFVIetsþQam ? edaysarkarkarBarCMgWtaLasesmIu vamansar³sMxan; ehIysPaBBitEdl faCMgWenHmanekItesÞIrEtRKb;tMbn;TaMgGs;TUTaMgBiPBelak karBinitüQamrk CMgWtaLasesmIukMritRsal KYrEtbBa©ÚlkñúgkmμviFIkarBarCMgWfñak;Catiedaycab; epþImeFVIelIkumartUc² b:uEnþvacaM)ac;Nas;munerobGaBah¾BiBah¾ b¤ munman épÞeBaH edIm,Ipþl;CMerIsRbesIrbMputdl;BYkeK. ya:gehacNas;eKKYr BicarNaBinitüQam ³ k> eBldwgfamanbgb¥ÚnCaGñkpÞúkCMgW b¤ manCMgW x> b¤kd¾ gw famanbgb¥nÚ rs;enA b¤ mkBIRbeTsEdlmanGRtaCMgtW aLasesmIxu s< ; PaBrYmExSelahitCamYyKña PaBrYmExSelahitCamYyKña mann½yfa manelahitRbhak;RbEhlKña dUcenH karerobkarrvagbgb¥Únsac;Cit eKrab;fa GaBah¾BiBah¾ExSelahitCamYyKña ¬Consanguineous marriage¦. enAkEnøgCaeRcInkñúgBiPBelakenH eKelIk Twkcitþ nig GnuvtþGaBah¾BiBah¾EbbdUecñH. ya:gNak¾eday TMnak;TMngsac; Qamrvag«Buk nig mþaykan;EtCit eRKaHmankUnekItCMgWtaMgBIkMeNItk¾man kan;EteRcIn dUcCaCMgWtaLasesmIu. KUrRskrEdlKμanCab;sac;QamnwgKañ »kasRbEhl 98° mankUnsuxPaB l¥Fmμta. KUrRskrEdlCabgb¥ÚnCIdUnmYYynwgKña »kasRbEhl 96° mankUn suxPaBl¥. KUrRskrEdlCabgb¥ÚnCIdUnmYYynwgKña ehIyman«Bukmþay nig¼b¤ 163

CIdUnCItak¾CaKUrRskrsac;QamCitEdrenaH »kasRbEhl 94° mankUn suxPaBl¥. dUcKñaenHEdr eRKaHmankUnekItCMgWfycuHya:gxøaMgeTAvij ebI«Bukmþayman TMnak;TMngsac;Qamq¶ayBIKña. kñúgcMeNamkumarekItmk 1000nak; BI«Buk mþayKμanCab;sac;QamnwgKña mankumarRbEhlEt 2 - 3 nak; b:ueNÑaHTTYl Bn§úbgáCMgW/ EtebI«Buk nig mþaymanTMnak;TMngsac;QamnwgKña 2 - 20 nak; nwgmanCMgWEbbenH KwvaGaRs½yelITMnak;TMngsac;QamCit b¤ q¶ay. CMerIssMrab;KUrRskrRbQmmuxnwgeRKaHfñak; kareFVIetsþTarkkñúgépÞ (Prenatal testing) krNIRsþImñak;CaGñkpÞúkEbtta taLasesmIukMritRsal kMBugKitcg;mankUn b¤ manépÞeBaHrYceTAehIy édKUrrbs;nag ¬ebIsinminTan;)andwgBIsPaBpÞúkCMgW xøÜnÉgeT¦ KYreFVIetsþQamPøam edIm,Idwgfa Kat;manpÞúktaLasesmIukMrit RsalEdr b¤ Gt;. RbsinebIGñkTaMgBIrCaGñkpÞúk KUrRskrenHGaceFVIkarsMerc citþ bnþEpnkarRKYsar b¤ ebIsinmanépÞeBaHrYceTAehIy GacBicarNabnþ épÞeBaHeTAeTot b¤ y:agNa ehIyebIGaceFVI)an eyIgbnþeFVIetsþelITark kñúgépÞrkemIlCMgWtaLasesmIu ebIeXIjfaTarkmanCMgW eKGacsMerccitþbBaÄb; KP’. CMerIsepSg²eTotEdlKUrRskrRtUvBicarNa mandUcCa karElglHKña/ sMukUneKmkciBa©wm/ gakeTArkbgákMeNItkñúgmnÞIrBiesaFn_CamYyemCIvitburs b¤ RsþIrbs;Gñkd¾éTEdlmansuxPaBl¥. ebImindUecñaHeT/ «BukmþayEdlRbkan; nUvCMenOsasnamutmaM GacsMerccitþmineFVIetsþelITark edIm,IdwgsPaBCMgW rbs;TarkkñúgépÞ ehIybnþkarmanépÞeBaHteTAeTot. eFVIetsþTarkkñúgépÞrkCMgWtaLasesmIu manetsþ bI ya:gEdlGackMNt;faetITarkkñúgépÞmanCMgWtaLasesmIu b¤ Kμan ³

164

karbUmykTwkePøaH ¬Amniocentesis¦ eKGnuvtþkarbUmykTwkePøaHmkBinitüenARtImasTI2 énépÞeBaH KWeRkay BIépÞeBaH)anRbEhl 15 ¬18 -22¦ s)aþh_. edayeRbI eGkU ¬Ultrasound¦ CaCMnYy/ RKUeBTüCMnajEpñksmÖBcak;m¢últUcqμarcUlkñúg épÞmþay bWtykTwkePøaH 2 - 3 søabRBa)ay. ekasikaTarkEdlman kñúgTwkePøaHRtUvykeTAviPaKkñúgmnÞIrBiesaFn_ edIm,IkMNt;rkCMgW taLasesmIurbs;TarkkñúgépÞ. eKeRbIetsþenHeBlépÞeBaHvivtþ)anGayueRcIn. vaminbgáeRKaHfñak;dl;mþay eT. ya:gNak¾eday vaGacbgá[TarkrlUtrvag 2 - 3 éf¶ eTA 2 - 3 s)aþh_eRkayetsþ. [ii] karbUmykQamTgsuk ¬Cordocentesis¦ edaymanCMnYyBI eGkU eKcak;m¢últUcqμarmYycUlkñúgeBaH eq<aHeTArk TgsukTark. eKbWtQamRbEhl 2 - 3 m>l ehIyQamTarkRtUveK ykeTAEjkkñúgmnÞIrBiesaFn_. kñúgkrNIPaKeRcIn eRkamTwkédGñkCMnaj eKTTYl)anekasikaTarksuT§ 100°. mUlehtubraC½yminTTYl)an QamTarksuT§ bNþalmkBIépÞeBaHmanGayutic KwticCag 18 s)aþh_/ mþayFat;duHk,aleBaH nig sukenAEpñkxageRkayéns,Ún. épÞeBaHman Gayutic k¾CamUlehtusMxan;bMputEdlbgá[manplvi)akF¶n;F¶rkñúgkar bUmykQamTgsuk. karEjkExSkøÚb‘Ín eday viFIEjkedaycrnþGKÁisnIkñúgsarFatuGn§il ¬Gel electrophoresis¦ EdlCaviFIFmμtarbs;mnÞIrBiesaFn_kñúgkarBinitü rkCMgW. kareFVIeraKvinicä½y)anTan;eBl nig c,as;las; edayeRbI viFIsikSam:UelKul ¬Molecular methods¦/ bc©úb,nñenH vaCMnYsesÞIrEt TaMgRsugviFIbUmykQamTgsuk EdleKGnuvtþEtkalNaépÞeBaHmanGayu eRcIn ehIyviFIRcwbsac;sukmkBinitü (CVS) minGac[eKsnñidæan)an nig kalNaKUrRskrRbQmeRKaHfñak; BMuEdl)anBinitüBImun²mk. [i]

165

[iii]

karRcwbsac;sukmkBinitü (Chorionic Villus Sampling = CVS) karRcwbsac;sukmkBinitü ¬CVS¦ GacGnuvtþ)anmun karbUmykTwk ePøaHmkBinitü KWtaMgBIépÞeBaHmanGayu RbEhl 10 - 11 s)aþh_. edaymanCMnYyBI eGkU GñkCMnajEpñksmÖBRcwbyksMNaksac;suk ¬Chorionic villi¦ EdlCaekasikapÞúkBt’manBn§údUcKñanwgTarkkñúgépÞ ehIyEdleRkaymkeTotva[kMeNItCasuk. ekasikaenHRtUveKbWtyk tamry³m¢últUcqμarmYy EdleKcak;cUleTAkñúgeBaHmþay ¬Transabdiminal¦ b¤k¾tamry³ kaetETr ¬Catheter¦ tUcqμarEdlcak; tammat;s,Ún ¬Transcervical¦. bnÞab;mkeKeFVIkarviPaKekasikaenaH rYckMNt;eraKvinicä½y. dUcviFIbUmykTwkePøaHmkBinitüEdr karRcwbsac;sukmkBinitüBMupþl;eRKaH fñak;dl;mþayeT. EtvamaneRKaHfñak;tictYcbgá[rlUtEdr. ebIkarrlUt ekItman vamankarlM)aknwgdwgfaetIvabNþalmkBIkarRcwbsac;sukmk Binitü b¤ ya:gNa eRBaHmankarrlUtCaeRcIn)anekItedayxøÜnÉgenArgVg; s)aþh_TI12 énKP’. GacmaneRKaHfñak;ekIneLIgedayeFVI[Gv³yv³TarkxusFmμta ebIeKqab; GnuvtþkarRcwbsac;sukmkBinitüeBk )anesckþIfa muns)aþh_TI8 énKP’ eRkayrdUvcugeRkay. ya:gNak¾eday BMumanPsþútagbgðajBIeRKaHekIn eLIgénPaBxusFmμtaNamYy eBlNaeKGnuvtþkarRcwbsac;sukmkBinitü eRkayedIms)aþh_TI9 bnÞab;BIrdUvcugeRkay . ehtuenHehIy)anCaeK GnuvtþviFIenHCaTUeTAenAeRkayedIm s)aþh_TI10 bnÞab;BIrdUvcugeRkay .

etIdMeNIrkarBinitüBn§úRbRBwtþeTAya:gdUcemþc ? karbUmykTwkePøaH nig karRcwbsac;sukmkBinitü TaMgBIrenHEp¥kelImUldæan Binitü DNA ehIykMNt;[sÁal; etImanPaBxusRbRktIénBn§ú b¤eT 166

¬karERbRbYlpøas;bþÚr = Mutation¦ EdlmanelI«Bukmþay EdlCameFüa)ay d¾suRkwtkñúgkareFVIeraKvinicä½yCMgWtBUC. Etya:gNak¾eday dUcKñanwgetsþ epSg²eTotEdr eTaHbImantictYck¾eday vaGacmanPaBel¥ógxusEdr. Bn§úkMNt;lkçN³PinPaKEdleyIgTTYlCalkçN³tBUC kñúgenaHmaneGm:UkøÚb‘Ín vaekIteLIgedaysar DNA. ral;CalikakñúgsarBagÁkay edayrYmTaMgsuk rbs;TarkpgsuT§EtmanKMrU DNA edaypÞal;xøÜnTaMgGs;. ]TahrN_ kñúgkar Rcwbsac;sukmkBinitü GñkviTüasaRsþmnÞIrBiesaFn_sikSaBn§úEdlkMNt; sMeyaKeGm:UkøÚb‘Ín EdlBn§úenaHpÞúkenAkñúg DNA rbs;ekasikasuk edIm,I[dwg fa eTAGnaKtetITarknwgmansuxPaBFmμta/ CaGñkpÞúkCMgWtaLasesmIu b¤ nwg manCMgWtaLasesmIukMritF¶n; b¤ya:gNa. kareFVIviPaKenH CaFmμtaeRbIeBl RbEhlmYys)aþh_. karbBaÄb;KP’ ebIsinCaetsþbgðajfaTarkmanCMgW KUrRskrGacsMerccitþbBaÄb;KP’. tYnaTI rbs;Gñkpþl;RbwkSaGMBIkarBinitüBn§ú nig RKUeBTüEp¥ksmÖB eBlenHmansar³ sMxan;xøaMgNas;. enAdMNak;kalenH ebIKUrRskrsMerccitþbnþKP’ enaHRtUv suxcitþTTYlykkarBüa)alCMgWkUnGs;mYyCivit. ebIsineRCIserIsykkar bBaÄb;KP’ ya:gNak¾eday karbBaÄb;KP’RtUveFVItamviFINamYykñúgcMeNamviFI 2 ya:g edayGaRs½yelIdMNak;kalénKP’. karqab;bBaÄb;KP’ eKGnuvtþkarbBaÄb;KP’enH eBlKP’manGayuticCag 14 s)aþh_. eKKYrCMrab R)ab;KUrRskrfa karbBaÄb;KP’enHmineFVI[)at;bg;»kasmankUnelIkeRkay eT. eKKYrBnül;pgEdrfa eBlKUrRskrRbQmeRKaHfñak;manKP’mþg²GacekIt TarkmkmanCMgW. RbsinebIbþIRbBn§manbMNgcg;dwgfa épÞeBaHkUnelIkeRkay eTotmanCMgWtaLasesmIu b¤ ya:gNa eKRtUvGnuvtþkarrkeraKvinicä½yTarkkñúg épÞmþgeTot. 167

karBnüabBaÄb;KP’ viFIbBaÄb;KP’EdlmanGayuelIsBI 14s)aþh_ KWRtUveFVI[mankarQWeBaHqøg Tenøedaycak;G½rm:Un ¬Prostaglandin¦ eTAkñúgs,Ún. karQWeBaHGacRbRBwtþ eTACaeRcInema:g ehIyviFIenHeFVI[RsþIb:HTgÁicGarmμN_ Cagkarqab;bBaÄb;KP’. dUcKñaEdr vaminb:HBal;lT§PaBmanKP’mþgeTotrbs;RsþIeT. CMhanepSg²eTot kareFVIeraKvinicä½yTarkkñúgépÞ nig karbBaÄb;KP’ minTan;yl;RsbTaMgGs;Kña faRtUvEteFVIenAeLIyeT. ya:gNak¾eday ebIKμankareFVIeraKvinicä½yTarkkñúgépÞ nig karbBaÄb;KP’eT karkarBarminGacBwgEp¥kEteTAelIkMNt;sMKal;GñkpÞúk CMgWenaHeT ehIykarBinitüQamedIm,IRsavRCavCMgW minGacmanRbsiT§iPaB nig TTYleCaKC½yeLIy. viFIkarBarepSg²eTotkMBugRtUvbegáIteLIg dUcCa kar viPaKekasikaTarkkñúgQamrbs;mþay. ya:gNak¾eday viFIenHmankMNt; ehIymkTl;eBlenHminGacpþl;CMerIsKYr[Tukcitþ)an ykmkCMnYsviFIetsþ TarkkñúgépÞ. bec©keTsepSgeTot Kw karkMNt;eraKvinicä½yBn§úmunkarbgá kMeNIt ¬Pre-implantation Genetic Diagnosis = PDG¦ EdlCakareRbI bec©kviC¢a DNA edIm,IeRCIserIsrkemCIvitmansuxPaBl¥rbs;RsþIpÞúkCMgWyk eTAbgákMeNItkñúgmnÞIrBiesaFn_ rYcykeTAdak;kñúgs,Ún. karkMNt;eraKvinicä½y Bn§úmunkarbgákMeNIt GacTTYlyk)anBIsMNak;saFarN³CnPaKeRcInEdl CMTas;nwgkarbBaÄb;KP’ ehIyGacRtUv)aneKeRbIya:gTUlMTUlayeRcIneLIg eBlNabec©keTsenHmantMélminsUvéfø ehIyminTamTarbec©kviC¢aeRcIn.

168

sTÞanuRkm Agranulocyte AIDS

Albumin

Aldosterone

Alloimmune

Anaphylaxis

Antibody

Antigen Antihistamine

Antioxidant

Arrhythmia

RbePTekasikaQam>s EdlKμanKulikakñúgsuItU)øas. mkBIBakü Aquired Immuno-Deficiency Syndrome ¬bNþMúeraKsBaØa bNþalmkBIkarcuHexSayRbB½n§karBar¦ Ca CMgWbgáedayvIrus HIV CaTUeTAeRcInEtqøgtamQam b¤ karrYmsgVas. CaRbUetGIunsamBaØmYy EdlmankñúgpøasμaQam sac;duM Epñks éns‘ut nig TwkedaHeKa. CaG½rm:UnesþrUGIutmYyEdlbMeBjmuxgarsMrbsMrYltulüPaBTwk nig GMbil kñúgsarBagÁkay. karbegáItGgÁbdibkçTb;Tl;sarFatuBIxageRkA ]TarhN_ QamEdlbBa©ÚleTA. PaBgayRbtikmμxøaMg cMeBaHsarFatuGIVmYy ]TarhN_ RbUetGIun b¤ fñaMeBTü edaybgáeGaysðúk ¬Shock¦. CaRbUetGIunEdlskmμPaBTb;Tl;nwgGg;TIEhSn ¬Antigen¦ CaEpñkénkareqøIytbrbs;RbB½n§karBarragkay. CasarFatuEdlGacePJaCRbB½n§karBarragkayeGayeqøIytb. smasFatu EdleKeRbIsMrab;Büa)alRbtikmμ Gal;ElhSIú ¬Allergy¦. sarFatuepSg² dUcCa Beta-carotene, Vitamin C nig Alpha-tocopherol EdlTb;;mineGaymanRbtikmμGuksIutkmμ b¤ RbtikmμEdlCMrujeday GuksIuEsn nig EBruksIut EdleK eCOfa vakarBarragkayeGaycakputBI\T§iBlrbs; ra:DIkal;esrI ¬Free radical¦. cgVak;ebHdUgmineTogTat; nig exSay 169

Autosome Blood plasma

RkUm:UhSÚmEdlminEmnCaRkUmU:hSÚmePT. FaturavBN’elOgRBEltkñúgQam EdlekItBIFatuTwk nig sarFaturlaykñúgTwkdUcCa RbUetGIun eGLicRtUlIt sár xøaj; plitplkaksMNl;rbs;emtabUlIs GamINUGasIud G½rm:Un nig vItamIn.

Bovine spongiform encephalopathy

Carbohydrate

Carbone dioxide

Chromosome

Citrate Corticosteroid

CMgWRbB½n§sésRbsaTkNþalvivtþbnþicmþg²bNþaleGaysøab; EdlekItelIstVeKaRkbI RsedogCMgW Srapie EdlekItelIecom nig BEB ehIyRbEhlCabgáedayRbUetGIunbgáCMgW vaqøgtam cMNIGahar ¬GacehAmüa:geTotfa CMgWeKaqáÜt¦. smasFatuepSg²EdlmanpÞúk kae)an GIuRdUEsn nig GuksIuEsn dUcCa sár GamIdug nig EsluyLÚs PaKeRcInekIt edayrukçCatiébtg. ]sμ½nF¶n;KμanBN’ ekItkñúgkardkdegáIm nig karpuyrlYy b¤ cMehHénstV nig rukçCati. rcnasm<½n§dUcsésGMe)aHenAkñúgsñÚlrbs;ekasika. vapÞúk Bt’manbnþBUC kñúgTMrg;CaBn§ú EdlRKb;RKgskmμPaB nig muxgarTaMgGs;rbs;ekasika. GMbilekItBIGasIud sIuRTic ¬Citric Acid¦. bNþa Steroids mYycMnYnrbs;RkeBj Adrenal-Cortex eKeRbICaPñak;garRbqaMgnwgkarrlak.

Creutzfeldt-Jakob disease

Deferiprone

170

CMgWxYrk,ald¾kMrman vavivtþbnþicmþg²bNþaleGaysøab; bgáedayRbUetGIunbgáCMgW ehIysMKal;edayCalikaxYrk,al manRbehagtUc² GñkCMgWmanPaBePøcPøaMgmunGayu ehIyclnasac;dMubnþicmþg²)at;bg;PaBsIusgVak;Kña. eQμaHBaNiC¢kmμénfñaMkMcat;CatiEdk eRbIelbtammat; plitedayRkumh‘un»sf Apotex .

Deoxyribonucleic

Desferrioxamine

GasIudnuyekøGiucepSg²EdlCamU:elKulmUldæanéntMNBUC ksageLIgrebobCaCeNþIrvil. eQμaHBaNiC¢kmμsMrab; Desferrioxamine plitedayRkumh‘un »sf Novartis-Pharma. fñaMkMcat;CatiEdk

DIC

Disseminated Intravascular Coagulopathy

acid (DNA)

Desferal

DNA Electrophoresis

Embolism

Enzyme

Erythroblast

¬kMNkQamkñúgsésQamBaseBjRKb;TIkEnøg¦. sUmemIBakü Deoxyribonucleic acid clnaEjkPaKl¥ittUc²tamry³sarFaturav b¤ xab;eRkam skmμPaBkMlaMgTMnajGKÁisnI rbs;eGLicRtUd EdleKdak; cUlkñúgsarFaturav b¤ xab;enaH. karsÞHPøam²énsésQameday Embolus ¬PaKl¥itxus Fmμta ]TahrN_ dUcCa BBuHxül; EdlGENþtkñúgQam¦ cUreRbobeFobeTABakü Thrombus. bNþMúRbUetGIunEdlCYyCMrujRbtikmμCIvKImIBiess enAkMritsI tuNðPaBrbs;sarBagÁkay. ekasikarbs;xYrq¥wgRkhm EdlsMeyaKeGmU:køÚb‘Ín ehIyCa ekasikasßitkñúgdMNak;kalkNþalkñúgvdþdMbUg énkarbegáIt eKalikaQamRkhm ebIniyay[TMlMTUlayCagenHKWCa ekasikaedImrbs;eKalikaQamRkhm.

Erythroblastosis foetalis (Rhesus or Rh disease)

CMgWEbkeKalikaQamRkhm ekItelITarkkñúgépÞ nig TarkeTIb ekItmanlkçN³sMKal;edaykarekIneLIg Erythroblasts kñúgQam ehIymanxan;elOg. TaMgenHekItmaneBlRbB½n§ Rh GviC¢manrbs;mþayplitGgÁbdibkç RbqaMgnwgGg;TIEhSn kñúgQamrbs;TarkEdlman Rh viC¢man. GgÁbdibkçqøgkat; 171

Erythropoietin

Ferritin

Fibrinogen

Fibrosis

Foetal hemoglobin

Folic acid

Free radical Gamma globulin Genotype Globin

Gram negative

Gram positive

Heme

172

sukeTAbMpøajeKalikaQamRkhmrbs;TarkkñúgépÞ. sarFatuG½rm:UnEdlbegáIteLIgCaBiessedaytMrgenam EdlePJaCeGaymankarpliteKalikaQamRkhm. CaRbUetGIunpÞúkCatiEdkkñúgPaBRkam EdlbMeBjmuxgarkñúg karsþúkCatiEdk ehIyBiessenAnwgeføIm nig GeNIþk. RbUetGIunmankñúgQam plitenAÉeføIm ehIyRtUv)anbþÚr CaRbUetGIunmanlkçN³CasésEdleKehAfa Fibrin kñúgeBlbegáItqñúkQamTb;karhUrQam. CasPaBmYyEdlsMKal;edaykarekInCalikaman sésevoveRcIn. eGm:UkøÚb‘Ín EdlmanExSGal;hVaBIr nig ExSEbttaBIr ehIyCa eGmU:køÚb‘Ín EdlmaneRcInCageKkñúgQamTarkeTIbnwgekIt ehIysßitenAkñúgsmmaRtmYyekIneLIgkñúgsPaBCMgW esøksøaMgmYycMnYn ¬]TahrN_ taLasesmIu¦. RkumvitamIneb ¬Vitamin B complex¦ manlkçN³CaRkam eRbIkñúgkarBüa)alCMgWesøksøaMg bNþalmkBIxVHGaharUbtßmÖ. bMENkm:UelKul b¤ GatUmEdlRbtikmμxøaMg. EpñkRbUetGIunkñúgQamEdlsMbUredayGgÁbdibkç. bNþúMBn§úTaMgGs; b¤ mYyEpñkénbuKÁlmñak; b¤ RkummYy. RbUetGIunmYyKμanBN’ TTYl)anedaykardk Heme ecjBI RbUetGIunmYy dUcCa eGm:UkøÚb‘Ín ¬haemoglobin¦. minCab;BN’RsKaM eBleKbMBak;BN’ eKeRbIsMxan;sMrab; )ak;etrI. Cab;BN’RsKaM eBlbMBak;BN’ eKeRbIsMxan;bMputsMrab; )ak;etrI. sarFatupÞúkCatiEdkmanBN’Rkhmcas; mankñúgeGm:UkøÚb‘Ín nig mIjÚkøÚb‘Ín ¬RbUetGIunenAkñúgsréssac;dMu¦.

Hematopoiesis Hemoglobin (Hb)

HIV

Hormone

Hydrops foeralis Hypogonadism Hypoparathyroidism Hypothalamus

Hypothyroidism Immune system Immunoglobulin Interferon

In Vitro Leukocyte Lymphocyte

karbegáItQam b¤ RKab;Qam. eGm:UkøÚb‘Ín CaFatuEdlmanpÞúkCatiEdk ehIysßitkñúg eKalikaQamRkhm. FatuenHdwknaMGuksIuEhSnBIsYteTA Calika ehIymanExSRbUetGIunbYn KW Gal;hVa/ Ebtta/ háam:a nig Edlta. Human Immunodeficiency Virus CaRkumrbs; Retrovirus EdlbMpøajRbB½n§karBarragkay ehIybNþaleGay ekItCMgWeGds¾. plitplrbs;ekasikamanCIvitEdleFIVdMeNIrkñúgvtßúrav rbs;sarBagÁkay ehIyeFIVeGayman\T§iBlBiessmYy. karehImya:gFM manlkçN³F¶n;F¶rénTarkkñúgépÞ. karcuHexSaymuxgarénRkeBjePT Gonads . karcuHfyénG½rm:UnRkeBj Parathyroid kñúgsarBagÁkay . Epñk)aténxYrk,al Diencephalon EdlCamCÄmNÐl sMrbsMrYlRbB½n§RbsaTsVy½t ¬]TahrN_ sMrab;RKb;RKg karjaMuGahar ¦. skmμPaBcuHexSayrbs;RkeBj Thyroid. RbB½n§karBarragkay. GgÁbdibkç¬GkSrkat; Ig ¦ Cam:UelKulTb;Tl;nwgvIrus )anmkedayekasikaeBIbRbTHnwg vIrus )ak;etrI b¤ fñaMRbqaMgvIrus EdleKplitedaykarsMeyaK. enAeRkAsarBagÁkaymanCIvit Etkñúgbrikassib,nimμit ekasikaQam>s ekasikaQam>s KμanBN’ manedImkMeNItecjBIekasikaedIm ¬Stem cell¦ EdlbegáItCaekasikakñúgTwgrég nig RbB½n§karBarsarBagÁkay. vamanBI 20° - 30° énekasikaQam>skñúgxøÜnmnusSFmμta. 173

Magnetic Resonance Imaging (MRI)

bec©keTseFIVeraKvinicä½y EdlminbgákarQWcukcab;dl;GñkCMgW edaybegáIt)anCarUbPaBedaykMuBüÚT½rénCalikarsarBagÁkay EpñkxagkñúgedayeRbIrlkviTüú edIm,Ivas;rMj½rma:ejTikrbs; GatUmkñúgsarBagÁkay.

Mitochondrion

¬Bhuvcn³ Mitochondia

Mutation Myocardium Oestrogen

Organomegaly Osteoporosis

Ovary Pancreas

Pancytopenia

Parathyroid gland

Phenotype

Pituitary gland

174

rcnasm<½n§TTYlxusRtUvkñúgkarplitfamBlsMrab;ekasika tamry³kardkdegðImrbs;ekasika. bMErbMrYlvtßúFatutMNBUC RsTab;sac;dMukNþalrbs;ebHdUg sarFatu ¬Ca G½rm:UnePT¦ gaynwgCMrujeGaycMNg;ePT ehIyePJaCeGaymankarvivtþn_criklkçN³CaePT®sþI. karrIkFMxusFmμtaénsrIragÁ ¬]TahrN_ eføIm GeNþIk¦ sßanPaBEdlb:HBal;q¥wg sMKal;edayfycuHPaBtan;rbs;q¥wg bgáeGayq¥wgeTACaes<at nig puygay)ak;. RkeBjbnþBUCrbs;RsþI CaFmμtavamanmYyKU. RkeBjFMmYy sßitenABIeRkayRkBH P¢ab;CamYyKl;rbs; eBaHevontUc ¬Duodenum¦ vabeBa©jGg;hSIúmrMlayGahar nig G½rm:Un RbePT Insulin nig Glucagon. karfycuHxusFmμtanUvcMnYneKalikaQamRkhm eKalikaQam>s nig pøaEktkñúgQam. RkeBjbeBa©jkñúgtUc²TaMgbYn EdlCab; b¤ p¥wbeTAnwg RkeBjTIrUGIut ¬Thyroid¦. RkeBjenH plitG½rm:Un )a:r:aTIrUGIut ¬Parathyroid hormone¦ . lkçN³sm,tþi EdlGacemIleXIjnwgEPñkénsrIragÁmanCIvit )anmkedayRbtiskmμrvag Genotype nig brisßanxageRkA. RkeBjbeBa©jkñúg ragBgRkeBItUc BN’RkhmRbepH

Ribavirin

mansésQameRcIn vaP¢ab;eTAnwgEpñksþÜcxageRkam énxYrk,al. FaturavénQam Twkrég b¤ EbbCaTwkedaHeKa Edl)anmk eRkayBIykFatuGENþtkñúgenaHecjGs;. ekasikaragCafastUc² beBa©jBIxYrq¥wgcUleTAkñúgQam. EdlmanlT§PaBkMNt;TMrg;xus²Kña. RbUetGIun EdleKrEmgKitfabgáeGaymanCMgWqøgepSg² enARbB½n§RbsaT. G½rm:Un Steroid ePT®sþIbeBa©jeday Corpus luteum edIm,I erobcMsac;épÞs,Ún eGayTarkEdlcab;kMeNItGaclUtlas;. kñúgeBlKP’kMBugvivtþ G½rm:UnenHbeBa©jedaysuk sMrab;karBar TarkkñúgépÞkMBugvivtþkMueGayrlUt. manTisedAkarBarCMgW. saMueTAnwgkarBüa)al b¤ mineqøIytbcMeBaHrMejac. Gg;hSIúmepSg²Edlkat;pþac; DNA eTACabMENk²Rtg; TItaMgBiess² enAxagkñúgm:UelKul ehIyeKrEmgeRbIvaCa sMPar³sMrab;eFIVkarviPaKm:UelKul. RkumvIrusEdlman RNA ¬dUcCa HIV ¦ EdlqøgeTAekasika edayeFIVeGayekasikaenaH cMlgyk RNA rbs;vIrusenaH edIm,IbegáItCa DNA. fñaMRbqaMgvIrus )anmkedaykarsMeyaK.

Ribonucleic acid

(RNA)

Saline

Edlman b¤ pÞúkCatiGMbil.

Plasma

Platelet Polymorphism Prion

Progesterone

Prophylactic Refractory Restriction enzyme

Retrovirus

Secondary amenorrhoea Sepsis Serum

kar)at;rdUvelI®sþI EdlBImunmkFøab;manrdUvFmμta. sßanPaBBul EdlCalT§plénkarratt,atrbs;)ak;etrI Epñkénvtßúravrbs;QamstV bnÞab;BIQamenaHkk. 175

Stem cell

Streptococcus

Testosterone

Thrombocyte Thrombophilia

Thrombus

Thyroid gland

TRALI

Urea

Uric acid

176

vamanBN’ragelOg ekIteLIgbnÞab;BIktþaQamkk RtUv)aneRbI Gs;edaykarbegáItkMNkQam. eKehAfa Blood serum. ekasikaedImdMbUg BMumanlkçN³kMNt;Biess Edlpþl;kMeNIt eTACaekasikaepSg² xusKña ¬haemotopoietic stem cell kñúgxYrq¥wg ¦ . BYk)ak;etrIEdlCaPñak;garbgáeraKdl;mnusS nig stVciBa©wm tampÞH. G½rm:UnbursbegáItedayBgsVas EdlTTYlxusRtUvkñúgkareFIV eGayekItman nig EfrkSalkçN³sMKas;man criklkçN³CaePTburs. emIl Platelet karkMNt;TukCamunEdlmanCatMNBUC b¤ eTIbnwgekItman edaybegáItCadMukMNkQamkñúgsésQam ¬Thrombosis¦. dMukMNkQamEdlekItkñúgsésQam ehIys¥itCab;enAnwg kEnøgEdlvaekIt ¬cUreRbobeFobnwgBakü Embolus¦. RkeBjbeBa©jkñúgenAEpñk)atxagmuxénk EdlbegáItG½rm:Un Thyroxine nig Triiodothyronine. Transfusion Related Acute Lung Injury

¬bBaðasYtekItmanPøambNþalmkBIkarbBa©ÚlQam¦. smasFaturwgd¾cMbgEdlmankñúgTwkenaménBYkstVciBa©wm kUnedayTwkedaH ehIyvaCalT§plcugeRkayénkar EbkFatuRbUetGIun. kaksMNl;énkarkat;pþac; Nucleic acids kñúgekasika sarBagÁkay. mYycMENktUcrbs; Uric acids )anmkeday karrMlayGaharsMbUr Nucleic acid dUcCa eføIm tMrgenam. Uric acids PaKeRcInRtUvqøgkat;tMrgenam ehIybeBa©jecal eTAtamTwkenam.

Vitamin D

Zinc (Zn)

Zygote

CavItamInrlaykñúgxøaj;/ tamlkçN³KImIsaRsþ vaCaRbePT Steroid ehIyvamansarsMxan;cMeBaHrcnasm<½n§Fmμta rbs;eFμj nig q¥wg. elahFatu BN’sragexovbnþic manv:aLg;BIr. vaCamIRkU sarCaticMruHd¾caM)ac;rbs;rukçCati nig stV. ekasikaEdlRtUv)anbdisn§ieLIgeBlemCIvitbursPBRbsBV nwgemCIvit®sþI.

¬sTÞanuRkmenH Ep¥keTAtamniymn½ykñúgeKhTMB½r Merriam-Webmaster’s Medical Desk Dictionary, Merriam-Webmaster, Springfield MA, 1996¦

177

Bt’mansMxan;² cMNIGahar nig CMgWtaLasesmIu kat;bnßyCatiEdkRsUbBIcMNIGahar kñúgCMgWtaLasesmIu eTaHCaPaBelIsbnÞúkCatiEdkPaKeRcInbNþalmkBIkar bBa©ÚlQamk¾eday karekIneLIgénkarRsUbCatiEdkBIcMNIGahar k¾CabBaða sMxan;mYyEdr. CaFmμta mankarRsUbCatiEdkBIcMNIGahareTAkñúgxøÜnrbs; eyIgkñúgbrimaNtictYcb:ueNÑaH. karRsUbCatiEdkekIneLIgeBlNaeGmU:køÚb‘Ín ¬Hb¦ kñúgQamcuHfy. dUecñHGñkmaneGmU:køÚb‘ÍncuHfy dUcCa GñkCMgW taLasesmIukMritmFüm b¤ GñkCMgW taLasesmIukMritF¶n;EdlbBa©ÚlQam min)aneTogTat; KYrsMrbsMrYlcMNIGahar[smRsb edIm,I[brimaNCatiEdk srubkñúgcMNIGahar nig kñúgsarBagÁkayrbs;xøÜnmantictYc. CatiEdk)an BIcMNIGaharmanBIrEbb KW CatiEdkEdlmankñúgsac;BN’Rkhm ¬CatiEdkkñúgGaharsac;¦ nig CatiEdkEdlmankñúgcMNIGaharepSg² ¬CatiEdkeRkABIGaharsac;¦. CatiEdkkñúgGaharsac; CatiEdkmankñúgsac;RkhmmandUcCa sac;eKa sac;ecom sac;RCUk nig sac;man;exμA k¾dUcCacMNIGaharsmuRT dUcCa RtIsaDIn xüg RKM. eføImKWCa RbPBsMbUrCatiEdk. Büayamkat;bnßycMNIGaharTaMgenH ehIyCMnYsGahar sac;TaMgenHedayRbUetGIun)anBIsENþkesog. ya:gNak¾eday k¾minCakarl¥ EdrEdleCosvagTaMgRsugnUvsac;man; nig sac;RtI BIcMNIGaharrbs;Gñk eRBaHGaharTaMgenHvapÞúknUvGaharUbtßmÖcaM)ac;epSg²eTot CaBiesssMrab; kumar. cUreRCIserIsjaMusac;man;Epñks CaCagsac;Rkhm eRBaHvaman CatiEdktic. 178

CamFümCatiEdkRbmaN 35° )anRsUbcUlkñúgsarBagÁkayrbs;eyIgeRkay BIjaMuGaharmansac;Rkhm. EtcMnYnenHGacERbRbYlcenøaH 10 - 40° sMxan;vaGaRs½yeTAelIGaharmanpÞúkTwkedaHeKa b¤ plitplBITwkedaHeKa. Catikal;süÚmEdlmankñúgTwkedaHeKa/ QIs_ ¬Cheese = RhVÚm:as;¦/ y:aGY ¬Yoghurt¦ nig kñúgTWkedaHeKaxab; vabnßykarRsUbCatiEdk Edlmankñúg Gaharsac;. BüayamjaMuTwkedaHeKamYyEkv eBljaMuGaharmanCatisac; ehIyeRbITwkedaHeKakñúgkardaMsø. ]TahrN_l¥² mandUcCa TwkRClk;QIs_ kñúgmðÚbEbb)araMgeQμaHfa Lasagne, Pasticcio, Mousaka nig Cannelloni edaydak;QIs_[eRcInkñúg Spaghetti bolognaise ehIyeRbIy:aGY nig TwkedaH eKaedIm,IeFVIsmøeRKOgkarIu. kñúgmYyéf¶jaMuTwkedaHeKaya:gehacNas;[)anRbEhlknøHlIRt eRBaHfava CYykarBareraKBuKq¥wgpgEdr. ebIsin)armÖxøacFat; eRbITwkedaHeKaKμanCati ERkm b¤ manCatiERkmtictYc EtvasMbUrCatikal;süÚm dUcTwkedaHeKa FmμtaEdr. CatiEdkeRkABIGaharsac; eRkABIGaharsac; CatiEdkmankñúgcMNIGaharepSg² dUcCa s‘ut sUkULa RKab;FBaØCati bEnø EpøeQI emIm ¬dMLÚg/ éqfav¦ nig sENþk. enAcRkPB Gg;eKøs manmðÚbGaharmYycMnYneKbEnßmCatiEdk dUcCa RKab;FBaØCatijaMuCa GahareBlRBwk emSARsUvsalI nig nMb½ug. EtRbeTsepSg²eTotRbEhl mineFVIEbbenHeT. GaRs½yeTAelIsmasPaBGahar karRsUbykCatiEdkEdlmanRbPBeRkABI Gaharsac;cUlkñúgsarBagÁkay manbrimaNticCagRbPBBIGaharsac; RbEhlCag 20dg. cMNIGaharEdlbnßykarRsUbcUl mandUcCa ³ (i) RKab; FBaØCati nig (ii) plitplBITwkedaHeKa/ ÉGaharEdlbegáInkarRsUbCatiEdk vij mandUcCa ³ (i) EpøeQI nig bEnøEdlsMbUrvItamInes/ (ii) sac;stV RtI ex©A xüg egov RKM nig bkSI/ nig (iii) RCk; ¬dUcCaRCk;Rtsk; RCk;és<ekþab>>>¦ 179

TwksIuGIuveFVIBIsENþkesog TwkexμH nig sura. karecos[putBICatiEdkEdlmanRbPBeRkABIGaharsac; CakarBi)ak eRBaHfa EdkmanenAkñúgesÞIrRKb;GaharTaMgGs;. b:uEnþeKGacpøas;bþÚrrebobhUbcuk edayjaMu[)aneRcInnUvcMNIGaharNaEdlbnßykarRsUbCatiEdk ehIykat; bnßyGaharNaEdlbegáInkarRsUbCatiEdk. GaharbnßykarRsUbCatiEdkEdlmanRbPBeRkABIGaharsac; 1> RKab;FBaØCati emSARsUvsalI eBat Ggár sENþkdI sENþkesog suT§EtbnßykarRsUb CatiEdkcUlkñúgsarBagÁkayrbs;eyIg ehIyRbqaMgnwg\T§iBlvItamInes. GaharsMbUrvItamInes begáInkarRsUbCatiEdk. ykl¥RtUvjaMuGaharEdlCa RKab;FBaØCati[)aneRcIn ehIyRtUvecosvagGaharsMbUredayvItamInes dUcCa TwkRkUcCaedIm. BüayamjaMuTwkedaHeKaCamYyRKab;FBaØCati ¬dUcCa nMb½ugdak; QIs_ nMb½ugecondak;bWr QIs_ma:karUnI RKab;FBaØCati nig TwkedaHeKa¦ . enAcRkPBGg;eKøs eRkABIemSARsUvsalIsuT§ RKb;emSARsUvsalIEkéqñTaMgGs; RtUvc,ab;tMrUveGaybEnßmCatiEdk. cMeBaHemSAFBaØCatisMrab;GahareBlRBwk vij manxøHeKbEnßmCatiEdk ehIyxøHeTotKμanbEnßmeT. dUecñH CakarRbesIr RtUveRCIserIsykemSARsUvsalIsuT§ b¤ nMb½ugeFVIBIRsUvsalIsuT§KμanbEnßm CatiEdk ehIyRtUvGansøaksMKal;sresrelIkBa©b;edayykcitþTukdak; munnwgTijmYyNaykmkeFVICaGahareBlRBwk. GaharEbbenHmandUcCa bbrRsUvsalI nig RKab;FBaØCatikBa©b;mYycMnYn Edllk;tamhagTMnijEpñk suxPaB EtRtUvemIlsøaksMKal;[c,as;edIm,IeRCIserIsykRbePTKμanbEnßm CatiEdk. enARbeTsepSg²eTot emSA nig RKab;FBaØCatijaMuCaGahareBlRBwk GacKμan bEnßmCatiEdkeT. 180

RbUetGIunsENþkesog GacCYybnßybrimaNCatiEdkRsUbcUlkñúg sarBagÁkay. RbUetGIunsENþkesog GacCYydl;rsCatimðÚbGaharCaeRcIn ¬]TahrN_ Spaghetti bolognaise, smø/ econbMBg¦ ehIyrsCatiGac kan;EtRbesIr ebImanbEnßmeRKOgeTspg. 2> TwkEt kaehV nig eRKOgeTs TwkEt kaehV nig eRKOgeTsmYycMnYn ¬]TahrN_ CIrnagvg CIv:an;s‘uy søwkéRK>>>¦ CYybnßykarRsUbCatiEdk. cUrjaMuTwkEt nigkaehV[)anerogral; éf¶ CaBiesseBljaMu)ay. ritEtRbesIrEfmeTot ebIjaMuCamYyTwkedaHeKa. TwkEt KWCaRbPBd¾sMbUrsarFatukMcat;CatiBulGuksIut ehAfa Gg;TIGuksIudg; ¬Antioxidants¦ EdlelIkmkBiPakSaxageRkam. 3> plitpleFVIBITwkedaHeKa TwkedaH QIs_ y:aGY bnßykarRsUbCatiEdkcUlkñúgsarBagÁkay. kal;süÚm k¾sMxan;Nas;EdrsMrab;karBareraKBukq¥wg dUecñHCakarRbesIrGñkRtUvbBa©Úl plitpleFVIBITwkedaHeKa[)aneRcIneTAkñúgcMNIGaharjaMu tamlT§PaBEdl GaceFVI)an. RbePTTwkedaHeKamanxøaj;tic ¬TwkedaHeKaKμanCatiERkm b¤ man CatiERkmbnþicbnþÜc¦ nig QIs_ suT§EtsMbUrkal;süÚm ehIyGacCakareBjcitþ sMrab;Gñkmincg;eLIgTMgn;. Caerogral;éf¶ ya:gehacNas;jaMuTwkedaHeKa [)anRbEhlknøHlIRt. GaharbegáInkarRsUbCatiEdkEdlmanRbPBeRkABIGaharsac; 1> vItamInes ¬Vitamin C¦ vItamInes mankñúgEpøeQI/ TwkEpøeQI nig bEnø. CakarRbesIrEdlRtUv ecosvagBisarTwkEpøeQI dUcCa TwkRkUcRc)ac; eBljaMuGahar b¤ eBljaMu ePsC¢³eBlRBwk. cUrjaMuTwkEt b¤ kaehV CMnYsvijeRBaHvaGacTb;karRsUb CatiEdk. b¤k¾jaMuTwkedaHeKamYyEkvvij ¡ RsaebobegáInkarRsUbCatiEdk dUecñHeBlBisaGahar ecosvagjaMuvajwkjyeBk b:uEnþGñkGacjaMuvaEtÉg 181

CamYyRKab;FBaØCatiepSg ]TahrN_ sENþkdIlIg. ya:gNak¾eday EpøeQI nigTwkEpøeQI KWRbPBsMbUrsarFatukMcat;CatiBul ehIyKYrjaMuvaEtÉg kMulay LMCamYyGaharepSg. bEnøRsuH b¤ es¶armanCativItamInestictYc eRBaHva RCabecjBIbEnøeTAkñúgTwk. 2> sac;stV sac;bkSI RtI nig GaharsmuRT sac;stV sac;stVsøab RtI nig GaharsmuRT minRKan;EtsMbUrCatiEdkb:ueNÑaH eT EfmTaMgCYyCMrujkarRsUbCatiEdkBIcMNIGaharEfmeTot. y:agNak¾ eday vaminCakarl¥eTEdlGñkRtUvtmGaharTaMgenHTaMgRsugEtmþg eRBaHva manpÞúkGaharrUbtßmÖcaM)ac;epSg²eTot BiesssMrab;kumar nig ekμgCMTg;. 3> RCk; ¬RCk;Rtsk; RCk;és<ekþab>>>¦ TwkexμHeFVIBIsENþkesog TwkRClk;manCatisura RCk;és<ekþab RCk;xÞwm)araMg éqfav nig kar:ut RBmTaMgplitplsENþkesog p¥ab; ¬]TahrN_ TwksIuGIuv¦ CYybegáInkarRsUbCatiEdk. brimaNCatiEdk RsUbrwtEtx<s; eBleKjaMu)ay b¤ nMb½ugCamYyRCk;bEnøTaMgenH. CaTUeTAGaharmanCatiEdktic CaGahareFVIBIRKab;FBaØCati ¬eBat emSARsUv salIsuT§ sENþk¦ nig emImbEnø ehIyminsUvmansac;stV RtI b¤ minsMbUr vItamInes. GaharmanCatiEdkmFüm CaGahareFVIBIRKab;FBaØCati emImbEnø Etmansac;eRcIn nig sMbUrvItamInes. GaharmanCatiEdkx<s; CaGahar sMbUrsac;stV nig RtI kñúgbrimaNeRcIn. GaharmanCatiEdkx<s; k¾manpÞúk pgnUvkMritvItamInesx<s; dUcCa EpøRkUcqμar nig bEnømYycMnYn. Gaharman CatiEdkx<s; GacbnßymkCaGaharmanCatiEdkmFüm)an edayjaMu[)an eTogTat;nUvGaharEdlbnßykarRsUbCatiEdkcUlkñúgsarBagÁkay dUcCa plitpleFVIBITwkedaHeKa RKab;FBaØCati sENþk Et nig kaehV. Gg;TIGuksIudg; kñúgGahar Gg;TIGuksIudg; ¬Antioxidants¦ kñúgGaharmansar³sMxan;Nas; dUcGtßn½y 182

énBaküdUecñHEdr. vakarBarkarxUcxatedayCatiBul RbePTGuksIutkñúg sarBagÁkay. kñúgn½yenHvaedIrtYnaTIsMxan;kñúgkarBarCMgW dUcCa CMgWsrés QamebHdUg nig CMgWmharIk. kñúgCMgWtaLasesmIu edaysarmanCatiEdkeRcInelIslb;kñúgsarBagÁkay vamaneRKaHfñak;x<s; bgá[mankarxUcxatedayGuksIut. kñúgGtßbTenH GñkniBn§nwgepþateTAelI Gg;TIGuksIudg; sMxan;² 4 RbePT KW vItamInGW ¬Vitamin E¦/ vItamInes ¬Vitamin C¦/ sarFatuBN’elOg ¬Carotenoids¦ nig sarFatukøin ¬Flavonoids¦. 1> vItamInGW ¬Vitamin E¦ vItamInGW CaGg;TIGuksIudg;kñúgcMNIGahard¾sMxan;CageKbMput. karsikSamYy cMnYn)anrkeXIjfa manGñkCMgWtaLasesmIuCaeRcInmankMrit vItamInGW kñúg QamticCagGñkKμanCMgW. enHGacmkBIGñkCMgWTaMgenH min)ancMNIGaharman vItamInGWeRcIn b¤ k¾mkBIGñkCMgWmantMrUvkarvItamInGWx<s;. kñúgkarsikSaCaeRcIn )anbgðajfa eBleKpþl;vItamInGWbEnßm enaHkMritvItamInGW kñúgQam)an RbesIreLIg. fVIebI evC¢bNÐit b¤ GñkCMnajGaharUbtßmÖ ENnaM[Bisar vItamInGWbEnßmk¾eday RtUvcaMfavItamInNak¾eday vacUlkñúgsarBagÁkay rbs;elakGñk Kwtamry³cMNIGahar. vItamInGW varlaykñúgxøaj; )anesckþIfavamanvtþmankñúgGaharEdlman xøaj;eRcIn. RbPBvItamInGWl¥bMput KweRbgrukçCati ¬eRbgGUlIv eRbgQUkr½tñ eRbgdUg nig eRbgsENþk¦. l¥CageK KW eRbgGUlIv eRBaHRbePTxøaj;kñúg eRbgenHGacCYykarBarCMgWebHdUg. enAbNþaRbeTstMbn;emDIETra:eN ¬RbeTsRkic B½rTuyhÁal; eGs,aj nig GIutalI¦ EdleRbIR)as;eRbgGUlIveRcIn CMgWebHdUgekItmanticCagenAGWr:ubxageCIg. b:uEnþcUrcgcaMfaBYkvItamIn RtUv bMpøajbnþicmþg²edaykarqa b¤ econ. dUcenHviFIl¥bMput edIm,ITTYl)anvItamIn eRcInbMputBIeRbgGUlIv KwbEnßmeRbgenHeTAelImðÚbenAcugbBa©b;karcMGin b¤ 183

eRkayBIcMGinrYcral;. eRbgGUlIvlayCamYyRkUcqμar CaeRKOgbEnßmelI sac;RtI sac;man; bEnøRsuH nig jaMPøa eGaykan;EtmanrsCatiq¶aj;. cUreRCIserIsykeRbgGUlIvsuT§ ebIsincg;)anxøinl¥ ehIyeRbIvaCaTwkjaMPøa b¤k¾sakl,gykeRbgGUlIvRbePTcMraj; ebIsincg;eRbIvasMrab;cMGinmðÚb b¤ eFVInMCaedIm.l. b½r\NðaeQμaHfa hÁI ¬Ghee¦ k¾manpÞúkvItamInGW Edr EtedayeRbgGUlIvmanRbeyaCn_dl;suxPaBl¥Cag GñkGaceBjcitþsakl,g eRbIva kñúgkarcMGinGahar. RbPBepSgeTotrbs;vItamInGW manplitplBITwkedaHeKa RKab;FBaØCati s‘ut nig sac;. plitplBITwkedaHeKamanPaBl¥viesskñúgkarykmkeFVICacMNI Gahar eRBaHfaminRKan;EtvamanvItamInGW b:ueNÑaHeT EtvaTb;karRsUbCatiEdk BIGaharcUlkñúgsarBagÁkay ehIyvamanCatikal;süÚmeRcIn EdlCYykarBar eraKBukq¥wgEfmeTotpg. GñkGacsakl,geRbITwkedaHeKakñúgkareFVIGahar b¤ k¾jaMuTwkedaHeKamYyEkv eBljaMuGahar. TwkedaHeKaKμanCatiERkm mankMrit vItamInGW ticCagTwkedaHeKamanCatiERkm EtbrimaNkal;süÚmmandUcKña. 2> vItamInes ¬Vitamin C¦ vItamInes begáInkarRsUbCatiEdkEdlmanRbPBeRkABIGaharsac; dUecñH vItamInes KWCaGg;TIGuksIudg;d¾manfamBlmYy kareRbIGaharEdlman pÞúkvItamInes layCamYyGaharmanCatiEdkeRcIn RtUvmankMrit. EbbenH vamansar³sMxan;BiessedayELksMrab;GñkCMgWtaLasesmIukMritmFüm EdlbBa©ÚlQammin)aneTogTat;. cUrcgcaMfa CatiEdkmanRbPBBIcMNIGaharCaeRcIn kñúgs‘ut sUkULa RKab;FBaØCati bEnø EpøeQI emIm ¬dMLÚg/ éqfav¦ nig sENþk. enAcRkPB Gg;eKøs manmðÚbGaharmYycMnYneKbEnßmCatiEdk dUcCa RKab;FBaØCatijaMuCa GahareBlRBwk emSARsUvsalI nig nMb½ug. EtRbeTsepSg²eTot RbEhlmin eFVIEbbenHeT. eKeXIjvItamInes eRcInmankñúgEpøeQI TwkEpøeQI nig bEnø. vaGacCakar 184

RbesIrEdlRtUvjaMuEpøeQI b¤ TwkEpøeQImYyEkvdac;EtÉg KwcenøaHeBl)ay ehIyminGMLúgeBljaMu b¤ eRkayBIjaMu)ayrYcPøameT. GñkCMnajEpñksuxPaB ENnaMRbCaBlrdæ[hUbbEnø nig EpøeQI[)an 5 dgerogral;éf¶. ]TahrN_ jauMmþgmandUcCa TwkEpøeQImYyEkv EpøeQImYycMNit ¬dUcCaEpøe)a:m EpøB½r eck RkUceBaF×sat; RkUcføúgknøHExøb b¤ eb:ge)a:HmYyEpø¦ bEnø ¬dUcCa kar:ut Rtsk; sENþk)araMg b¤ saLat;¦. vItamInes rlaykñúg Twk dUecñHebIykbEnøeTAes¶ar b¤ RsuH vItamInesnwgrlaycUlkñúgTwkGs;. karcMhuybEnøkñúgkMritlμm nwgrkSa)anRbesIrnUvvItamInkñúgbEnø. bEnøcMGin CamYyeRbgGUlIv nig RkUcqμar GaceFVIGahard¾manrs;Cati. vItamInGW nig vItamInes eBlBYkvaenACamYyKña skmμPaBBYkvaritEtl¥ dUecñHsUmkMuePøc bEnßmfamBldl;bEnø rbs;elakGñkedayeRbIeRbgGUlIv ¡ 3> sarFatuBN’elOg ¬Carotenoids¦ CaTUeTA RbPBcMNIGaharEdlmansarFatuBN’elOg mandUcCa kar:ut el<A sac;elOg eBatelOg eb:ge)a:H lðúg RkUceBaFisat; nig bEnøsøwkébtgcas;. GaharTaMgenHPaKeRcInmanvItamInesx<s; dUecñHeyIgRtUvRbugRby½tñ dUcbBa¢ak; xagelI. eyIgsUmbBa¢ak;CUnfa karRsUbyksarFatuBN’elOgBIcMNIGahar mankMritekInx<s; eBlcMNIGaharenaHmanCatixøaj; b¤ eRbg dUecñHkMuePøcbEnßm eRbgGUlIv. sarFatuBN’elOgGacRtUvbMpøajenAsItuNðPaBx<s; dUecñHsUm daMsø enAsItuNðPaBTab nig ry³eBlxøIRbsinebIGaceFVIeTA)an. 4> sarFatukøin ¬Flavonoids¦ eXIjmankñúg TwkEt RsaRkhm EpøeQI nig bEnø. enHCaelsmYyGac[ eyIgjaMuRsaRkhmmYyEkv eBlBisarGahar ¡ EtebI»kasminGnuBaØatieT cUrjaMuTwkEtmYyEBgvij. TwkEtminRtwmEtpþl;Gg;TIGuksIudg; CaeRcInb:ueNÑaH eT vaEfmTaMgTb;karRsUbCatiEdkBIcMNIGahar CaBiesseBljaMuvaCamYy TwkedaHeKa. cUrsakl,gBisaTwkEt CaRbcaM[)anbIeTAbYnEkvCaerogral;éf¶. cUrcgcaMfa mnusSeyIgRtUvkarTwkRbEhl 8 EkvkñúgmYyéf¶ edIm,I[manCati TwkRKb;RKan;kñúgxøÜn.

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esckþIsegçb vItamInGW ¬Vitamin E¦sMbUrmankñúgeRbgrukçCati dUcCaeRbgGUlIv nig eRbgpáaQUkrt½ñ. l¥CageK KweRbgGUlIv eRBaHvaGacCYykarBarCMgW ebHdUg. cUrbEnßmeRbgGUlIvenAcugbBa©b;énkarcMGinGahar eRkayeBl Gaharq¥in b¤ EfmeTAelIbEnøeqA eRBaHkMedAGacbMpøajvItamIn. vItamInes ¬Vitamin C¦ mankñúgEpøeQI nig bEnø . RbesIrbMputkMujauM EpøeQI nig bEnøTaMgenaHCamYyGaharEdlsMbUrCatiEdk RbsinebIelak GñkmanCMgWtaLasesmIukMritmFüm ehIyKμankarbBa©ÚlQam. cUrGñk hUmEpøeQI nig bEnøenAcenøaHeBljaMuGahar. cUrbEnßmeRbgGUlIv dl;bEnørbs;Gñk eRBaHvItamInGW nig vItamInes eBlBYkvaenACamYyKña skmμPaBBYkvaritEtl¥. sarFatuBN’elOg ¬Carotenoids¦ mankñúgkar:ut el<Asac;elOg eBatelOg eb:ge)a:H lúðg RkUceBaF×sat; nig bEnøsøwkébtgcas;. edaysarGaharTaMgenHsMbUrvItamInes dUcenHRtUvRbugRby½tñdUc )anerobrab;xagelI. CafμImþgeTot cUrkMuePøceRbgGUlIv. Et nig RsaRkhm mansarFatukøin ¬Flavonoids¦ ehIyk¾Ca Gg;TIGuksIudg;Edr. elIsBIenH TwkEtTb;karRsUbCatiEdk.

GtßbTenH)anykbBa©ÚlkñúgesovePAenHedaymankarGnuBaØatiBIGñkniBn§ ³ Dr. Dona Hileti-Telfer Senior Dietician Great Ormond St. Hospital for Sick Children London

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eRbI T2* sMrab;vas;kMritbnÞúkCatiEdkkñúgebHdUg T2* KWCakarvas;EvgefrevlamYyEdlGacqøúHbBa©aMgbnÞúkCatiEdkkñúgCalika. karvas;Evg T2* enHTTYlrUbPaBbgðajelIma:sIun MRI sþg;dar ¬Standard Magnetic Resonance Image¦. ma:sIun MRI eRbIrlkviTüú nigma:ej:Tik d¾xøaMg edIm,IbgðajrUbPaBCalikasarBagÁkay. ma:sIun MRI kMNt;PaBxus²Kña énKuNPaBma:ej:TikkñúgsarBagÁkay ehIyERbkøayPaBxus²KñaTaMgenHeTA CarUbPaBEdlRKUeBTüGaceRbIedIm,IeFVIeraKvinicä½yCMgWepSg². karelIsbnÞúk CatiEdkbgá[mankarERbRbYllkçN³ma:ej:TikkñúgCalika KWeFVI[ T2* ¬T2* value¦ mantMéltUcCagFmμta. karvas;Evg T2* énebHdUg mansar³sMxan; Nas; eRBaHeK)anrkeXIjfa ]TahrN_ebIGñkCMgWman T2* énebHdUgman tMélticCag 20 milliseconds ¬1 millisecond = 1¼1000 énvinaTI¦ enaHeRKaHfñak;cMeBaHebHdUgmankMritx<s; edaysarelIsbnÞúkCatiEdkCag T2* elIsBI 20 milliseconds. kareFVIetsþ T2* CakarBinitümYymanefrevla 45 naTIkñúgma:sIun MRI biTCit. eK[GñkCMgWedkelIturMkilmYy ehIyeKdak;G½kSGKÁisnIBiess mYycMnYnP¢ab;nwgRTUgGñkCMgWedIm,ItamdancgVak;ebHdUg. GñkCMgWRtUvBak; nwgk,alnUv]bkrN_mü:ag manmIRkUhVÚnnwgRtecok (Headset with a microphone) edIm,IeFVITMnak;TMngCamYyGñkbec©keTs. tuGñkCMgWRtUvruj cUlyWt² eTAkñúgma:sIun MRI edIm,I[xøÜnGñkCMgWTaMgmUlsßitkñúgRbehagma:sIun ¬ma:sIun MRI manrUbragÁRsedognMkgdUNat;d¾FMmYy manRbehageRCA¦. kñúgGMLúgeBlkMBugft/ GñkCMgWRtUvedk[es¶óm sþab;karENnaM[Gt;degðImmYy ry³eBlxøI edIm,IeKvas; T2*. kñúgxN³eBlkMBugRbEmRbmUlkarvas;Evg/ ma:sIun MRI beBa©jsMelgxÞ½r. ]bkrN_EdlGñkCMgWBak;nwgk,al nwgkarBar RtecokBIsMelgxÞ½renH. etsþenHmanlkçN³minb:HBal; ehIyminbgákar QWcukcab;eLIy. 187

elIsBIenHeTot edayma:sIun MRI pþl;rUbPaBebHdUgkMBugkRnþak;xN³kMBug eFVIkarvas; T2* eKGacvaytMél)anpgEdr BImuxgarrbs;ebHdUg. karvas; muxgarrbs;ebHdUgeFVIeLIgtamry³ma:sIun MRI eKKitfamanlkçN³BitR)akd Cagma:sIuneGkU ¬Ultrasound¦ vas;ebHdUgFmμtaeTAeTot. MRI GaceFVIkar vas;EvgpgEdrnUvTMhM nig dMeNIrRbRBwtþieTAénftebHdUgeRkamxagsþaM EdlCa ftrujQamcUleTAsYt. GñkCMgWtaLasesmIu gaymaneRKaHfñak;ekItCMgW sMBaFQamekInkñúgsYt EdlGacbgá[ftebHdUgeRkamxagsþaMrIk. sPaBenH GacbnßyRbsiT§iPaBkñúgkarrujRcanQamrbs;ftebHdUgenH. bNþaGñkviTüasaRsþEdleK[Gnuvtþkic©karenH bc©úb,nñkMBugEteFVIkarbBa¢ak; GHGagnUvBt’manxagelIenHEdlTTYl)anBIkarBinitüGegátEpñkKøInik ehIy TIF RBmTaMg NIH enAshrdæGaemrikkMBugKaMRTkic©RbwgERbgenH.

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mIlIlIRt ¬m>l = ml¦ 1¼1000 énmYylIRt Rkam kñúgmYyedsIulIRt ¬Rkam¼d>l = g/dl¦ edsIulIRt = 1¼100 énmYylIRt KILÚRkam ¬KRk = kg¦ mIlIRkam ¬mRk = mg¦ ¬DFO, L1¦ 1¼1000 énRkam mIRkURkam¼l ¬μg/L¦ 1¼10 000 énRkam kñúgmYylIRt

BakübMRbYjCaskl Parvovirus B-19 :

B19

Epstein-Barr Virus :

EBV

Deferioxamine :

DFO

Iron

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Hepatitis B Virus :

HBV

Hepatitis C Virus :

HCV

Human Immunodeficiency Virus :

HIV

Acquired Iimmunodeficiency Syndrome :

AIDS

Serum Alanine Aminotransferase :

sALT-Liver Function Test

Serum Asparate Aminotransferase :

sAST-Liver Function Test

¬eGm:UkøÚb‘Ín¦ : Hb HAV ¬vIrusrlakeføImRbePT Ga¦ : Hepatitis A Virus Hemoglobin

Magnetic Resonance Image : MRI

198

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http://www.thalassaemia.org.cy

kñúgenH rYmman ³ 1> 2> 3> 4> 5>

Gñkpþl;RbwkSaviTüasaRsþ shkarI-rinI viTüasaRsþ smaKmCatiCMgWtaLasesmuInana sMPar³sMrab;Gb;rM RBwtþikarN_epSg²

Bt’manTak;TgnwgkareFVIdMeNIr sUmGanesovePA {kareFVIdMeNIreTAbreTs nig suxPaB 2002} erobcMeday GgÁkarsuxPaBBiPBelak (WHO)

http://www.who.int/ith

email: cdsdoc@who.int

WHO

³ GgÁkarsuxPaBBiPBelak

WHO Geneva headquarters

http:/www.who.ch

WHO Africa Regional Office

www.whoafr.org

WHO Europe Regional Office

www.who.dk

WHO Southeast Asia Regional Office

www.paho.org

199

WHO Eastern Mediterranean Regional Office

www.who.sci.eg

WHO Western Pacific Regional Office

www.who.org.ph

HIV

nig karrlakefIĂ¸m

www.hivandhepatitis.com

HIV

www.who.int/hiv_aids/first.html

ICBS : International Consortium of Blood Safety

www.icbs.com/about/htm

ISBT : International Society for Blood Transfusion

isbt@eurocongress.com

Blood Safety

www.int/health_topics/blood_safety/en

Interactive Groups for Discussions

http:/groups.msn.com/thalassemiapatientsandfriends/home.htm

Product Information prepared by WHO

200

www.inf/vaccines_documents/

Gas½ydæanTMnak;TMngsMxan;² nig Bt’man WHO Headquarter World Health Organisation Headquarters Avenue Appia 20, 1211 Geneva 27, Switzerland Telephone : (41 22) 791-21-11 / Fax : (41 22) 791-0746 Cable : UNISANTE GENEVA / Telex : 415 416 Email : info@who.int / Website : http:/www.who.ch/

AFRICA WHO Regional Office Dr. Naomi Nhwathiva, Chief DCP, Regional Office for Africa, World Health Organisation, Parirenyatwa Hospital, P.O Box BE 773, Harare, Zimbabwe Telephone : (263) 407-69-51 or (263) 470-74-93 Fax : (263) 479-01-46 or (263) 479-12-14 Telex : 5217 or 5364 UNISANTE / Cable : UNISANTE BRAZZAVILLE Email : ragafro@whoafra.org Website : http:/www.whoafra.org/

EUROPE WHO Regional Office J.E. Asvall, Regional Director, Regional Office for Europe Accident Prevention Programme, World Health Organisation, 8, Scherfigsvej, DK-2100 Copenhagen 0, Denmark Telephone : (45) 39-17-17-17 / Fax : (45) 39-17-18-18 Telex : 15348 or 15390 / Cable : UNISANTE COPENHAGEN Email : postmaster@who.dk Website : http:/www.who.dk/

201

AMERICAS WHO Regional Office George A.O. Alleyne, Regional Director, Regional Office for the Americas, Emergency Preparedness & Disaster Relief Coordination, World Health Organisation, Pan American Sanitary Bureau, 525 3rd Street NW, Washington, DC 20037, USA Telephone : (202) 974-3000 / Fax : (202) 974-3663 Telex : 248338-440057-64152-892744 Cable : OFSANPAN WASHINGTON Email : postmaster@paho.org / Website : http:/www.paho.org/

SOUTHEAST ASIA WHO Regional Office Mrs Harsaran Bir Kaur Pandey, IO Regional Office for South East Asia World Health Organisation, World Health House Indraprastha Estate, Mahatma Gandhi Rd, New Delhi, 110002 India Telephone : (91) 11-331-7804 or (91) 11-331-7823 Fax : (91) 11-331-8607 or (91) 11-332-7972 Telex : 3165095 or 3165031 / Cable : WHO NEW DELHI Email : postmaster@whosea.org Website : http:/www.tron.um.u-tokyo.ac.jp/

EASTERN MEDITERRANEAN WHO Regional Office Hussen A.Gezairy, MD., F.R.C.S., Regional Director, Regional Office for the Eastern Mediterranean World Health Organisation, PO Box 1517, Alexandria, 21511 Egypt Telephone : (203) 48-202-23 or (203) 48-202-24 or (203) 48-300-90 Fax : (203) 48-389-16 or (203) 48-243-29 Telex : 54028 or 54684 / Cable : UNISANTE ALEXANDRIA Email : emro@who.sci.eg / Website : http:/www.who.sci.eg

202

WESTERN PACIFIC WHO Regional Office Dr. Shigeru Omi, Regional Director, Regional Office for the Western Pacific Health Services, Development & Planning World Health Organisation PO Box 2932, 1099 Manila, Philippines Telephone : (632) 538-80-01 / Fax : (632) 521-10-36 or (603) 536-02-79 Telex : 27652-63260-40365 / Cable : UNISANTE MANILA Email : postmaster@who.org.ph Website : http:/www.who.org.ph/

Council of Europe (CoE) Mr. Karl â&#x20AC;&#x201C; Friedrich Bopp, Administrative Officer, Health & Social Affairs Directorate General III Social Cohesion 67075 Strasbourg, France Tel : (33) 388412214 / Fax : (33) 388412726 Email : karl-friedrich.bop@coe.int

European Commission Dr. Lieve Fransen, Principal Administrator, Health & Family Planning, AIDS Unit, Rue de Geneve/Genevestraat 12, B1140, Bruxelles Tel : +32-2 2963698/2969117 Fax : 2963697

Food and Drug Administration (FDA), USA Dr Jay Epstein, Director, Office of Blood Research and Review, Center for Biologics Evaluation and Research, Food and Drug Administration, HFM-300,1401 Rockville Pike, Rockville, MD 20852 Tel : +13018273518 / Fax : +13018273533 Email : epsteinj@cber.fda.gov

203

International Consortium for Blood Safety (ICBS) Dr Mohamed El-Nageh, Executive Director, New York Blood Center, 310E 67th Street, New York, NY 10021, USA Tel : +12125703319 / Fax : +12125703320 Email : elnagehmm@aol.com

International Federation of Blood Donor Organisations (FIODS) Mr Niels Mikkelsen, Secretary General, Bloddonorerne, Denmark, Vesterbrogade 191, 1800 Frederiksberg, Denmark Tel : +4570137014 / Fax : +4570127010 Email : mikkelsen@bloddonor.dk

International Federation of Red Cross and Red Crescent Societies (IFRCRCS) Mr Peter Carolan, Senior Officer, Case Postale 372, 1211 Geneva 19, Switzerland Tel : +41227304222 / Fax : +41227330395 Email : carolan@ifrc.org

International Society of Blood Transfusion (ISBT) Dr Paul Strengers, Secretary General, PO Box 9190, NL 1006 AD Amsterdam, The Netherlands Tel : 31205123212 / Fax : 31205123560 Email : p_strengers@clb.nl

204

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Cover text.qxp

1/12/2008

9:48 AM

Page 1

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ISBN : 9963-623-40-9

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