Beta-Thalassaemia educational booklet - Hindi by Thalassaemia International Federation (TIF)

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This booklet titled "About Beta Thalassaemia" authored by Dr. Androulla Eleftheriou and Dr. Michael Angastiniotis is a publication of Thalassaemia International Federation. To create public awareness and increase understanding about Thalassaemia, this book has been translated in Hindi by Dr. V. K. Khanna who is a senior consultant pediatrician and incharge of Preeti Tuli Thalassemia Unit at Sir Ganga Ram Hospital, New Delhi, India. The printing of this book has been facilitated by Thalassemics India, A-9, Nizamuddin West, New Delhi, India.

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d s o SK k f u d ' k C n k o y h e sa d k sf M ax d su k e l st k u k t k r k g S) f y , f t E e n sk j t h u e as f d l h r j g d kn k " s k( d e h ) b u J [ a` k y k v k as d s m R i k n u e as d e h y kl d r kg S v k j S b l l s (carrier / trait) g a & F k Sy k l h f e ; k d k l o a k g d k u ssd h f L F k f r c u l d r h g A S v x j ; g n k " sk v f / d t h u k as i j v l j M k y r h g Sr k sa & J [ a` k y k d sm R i k n u e as v k j S d e h v k t k r h g S v k j S b l d k j . k O ; f D r F k y S k l h f e ; k l sx H ak h j : i l si H zk k f o r g k s l d r k g A S b l h i d z k j ] b & J [ `k y a k v k as ( b & X y k f sc u t h u ) d h t h u d k f sM x a e as n k " sk b & J [ a` k y k v k as d sm R i k n u e as d e h y k Ø k se k st +k sE l l d r k g S; k m R i k n u i j wh r j g c n a g k sl d r k g A S d k b sZO ; f D r b & F k Sy k l h f e ; k d k l o a k g d g Sv F k o k e è ; e ; k x H ak h j F k y S k l h f e ; k l si h f M r + j k x sh g ] Sb l d k i r k d j u k b l c k r i j f u H k j Z d j r k g Sf d b & J [ `k y a k d sm R i k n u e as d e h f d l L r j r d v k b Zg A S , l s sF k y S k l h f e ; k ] f t u e as X y k f sc u d k m R i k n u i H zk k f o r g k r sk g ] S d sB h d m y V d N q , l s h f L F k f r ; k ¡ g k r sh g ] aS f t u e as t h u e as n k " sk d si f j . k k e L o : i x y r i d z k j d si k zV sh u d k m R i k n u g k r sk g ] S f t U g as v l k e k U ; ; k l j ap u k R e d g h e k X sy k f sc u : i k r aj (structural haemoglobin variants) d g k t k r k g ] S v k j S f t u d h c u k o V v k j S d k ; Zl k e k U ; g h e k X sy k f sc u (HbA) d h c u k o V v k j S d k ; Zl sv y x g k r s sg A aS m u d h v u o q k f a' k d i k zf I r ( e k r k & f i r k l sf e y u k ) v k Sj f u n k u l sl ac af / r (clinical) i f j . k k e d k m Y y s[ k c qd y sV ~l 1 ] 2 v k j S 3 e as f d ; k x ; k g SA g h e k sX y k sf c u & l ac a/ h i ze q[ k c h e k f j ; k ¡g Sa% a & J `[ k ay k l ac a/ h c h e k f j ; k ¡ b & J `[ k ay k l ac a/ h c h e k f j ; k ¡ & F k Sy k l h f e ; k f l d y l sy f o d k j a HbH j k sx f l d y l sy , u sf e ; k ( j D r k Y i r k ) ( HbSS) a & F k Sy k l h f e ; k g k b ZM ªk I l f i Q V Sf y l HbS/b & F k Sy k l h f e ; k ( ¾ Hb c k V ~Z d k g k b ZM ªk WI l f i Q V Sf y l HbSC c h e k j h a & J `a[ k y k : i k ar j HbSD c h e k j h v U ; d e i k , t k u so k y sf l d f y ax f o d k j b & F k Sy k l h f e ; k x aH k h j b & F k Sy k l h f e ; k e è ; e b & F k Sy k l h f e ; k HbE/b & F k Sy k l h f e ; k v U ; d e g k su so k y h F k Sy k l h f e ; k b &FkSyklhfe;k 07

e k r k & f i r k d sv u o q k f a' k d ( t U e d sl k F k g h e k r k & f i r k l sc P p k as d k sf e y u k ) y { k . k k as d sv u l q k j f d l i d z k j c P p k as d k sc h V k & F k y S k l h f e ; k i k zI r g k r sk g ] S b l d h p p k Zb l c d q y V s e as d h t k , x h A v U ; n k sc d q y V sk as ( 2 v k j S 3 ) e as Ø e ' k % a & F k Sy k l h f e ; k v k j S f l d y l y s c h e k f j ; k sa i j p p k Zd h t k , x h A ; f n e k r k & f i r k n k u sk as e as b & X y k f sc u t h u l p q k : : i l sd k ; Z (functional) d j j g sg k A as v x j e k r k & f i r k n k u sk as d h b & X y k f sc u t h u v i H zk k f o r g k s; k i j wh r j g l sd k ; Zd j j g sg k ] sa r k s c P p k n k sv i H zk k f o r , o al p q k : : i l sd k ; j Zr b & X y k f sc u t h u k as d h v u o qk f a' k d i k zf I r d j x sk r F k k l H k h c P p k as e as l k e k U ; o ; L d g h e k X sy k f sc u (HbA) e k t S n w g k x sh A ; f n e k r k & f i r k e as l sd k b sZ, d ] , d i H zk k f o r b & X y k f sc u t h u d k o g u d j r k g k as ; k f u t c e k r k & f i r k e as l s, d b & F k y S k l h f e ; k d k l o ak g d g k sv k j S n l w j k n k sv i H zk k f o r b & X y k f sc u t h u k as d k o g u d j r k g k sr k s, l s se k r k & f i r k d si R z; d s c P p se as l o ak g d e k r k ; k f i r k l si H zk k f o r b & X y k f sc u t h u d h v u o qk f a' k d i k zf I r d h l H ak k o u k n k se as l s, d ( ; k f u 5 0 » ) g k x sh A ( v k d f `r & 1 ) v k i d k sl ao k g d f L F k f r c r k u so k y sv U ; u k e H k h f e y l d r sg Sa] t Sl s% ( i) b & F k Sy k l h f e ; k d sl ao k g d (Thalassemia traits) ( ii) b & F k Sy k l h f e ; k d sf y , g sV ªk st k b x l O ; f D r (Heterozygous) ( iii) y ? k qb & F k Sy k l h f e ; k l si h f M +r O ; f D r (Thalassemia minors)

b & FkSyklhfe;k ds laokgdks ds ckjs esa% & F k y S k l h f e ; k d sl o a k g d c h e k j h l si h f M r + u g h ag k r s sg A aS m u e as d k b sZ' k k j h f j d ; k e k u f l d y { k . k b u g h ag k r s sg aS v k j S m U g as f o ' k " sk [ k k u & i k u (diet)] f p f d R l h ; i j k e ' k Z; k b y k t d h t : j r u g h ag k r sh A x Sj & l ao k g d (non-carrier) O ; k f D r ; k sa d h r qy u k e sa m u e sa N k sV h y k y j D r d k sf ' k d k , ¡g k sr h g SA , sl k b l f y , g k sr k g Sf d f d l h l ao k g d d k s, d i zH k k f o r b & X y k sf c u t h u d h v u qo k af ' k d i zk f I r e k r k @ f i r k l sg qb Zg k sr h g St k so ; L d g h e k sX y k sf c u (HbA) d e e k = k k e sa ; k f c Y d qy u g h a c u k r k g SA b l i zd k j ] m l d h j D r d k sf ' k d k v k sa e sa l k e k U ; l sd e g h e k sX y k sf c u g k sr h g Sv k Sj b l d si Q y L o : i m u d k v k d k j x Sj & l ao k g d d k sf ' k d k v k sa d h r qy u k e sa N k sV k v k Sj d e y k y g k sr k g SA f i Q j H k h l ao k g d v f / d y k y d k sf ' k d k , ¡i Sn k d j d sb l d e h d k si j wk d j y r s sg aS v k j S b l i d z k j j D r e k u o t h o & l j ap u k d sf y , d k ; Zd j r k g A aS l o ak g d f L F k f r c k n e as f d l h c h e k j h e as r C n h y u g h ag k sl d r h A o k L r o e ] as t c r d b l d h [ k k l t k p ¡ u g h ad h t k , ] r c r d T ; k n k r j y k x sk as d k sb l d k i r k H k h u g h ap y r k g A Sg k y a k f d ] d N q l o ak g d k as d k sg Y d h j D r k Y i r k (anaemia) d k v u H qk o g k sl d r k g Sv k j S f t l d h i g p k u x y r h l sv k ; j u r Ù o d h d e h o k y k , u f se ; k (iron deficiency anaemia) d s: i e as d h t k l d r h g A S g k y ¡ k f d y c sk j sV sj h & t k p ¡ l s n k u sk as d sc h p v r aj d k i r k v k l k u h l sy x k ; k t k l d r k g A S &FkSyklhfe;k 08 b

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b & u r h t k ; g g Sf d F k Sy k l h f e ; k d sl ao k g d g k su sd k L o k L F ; ] t h o u d h v o f / ; k x q. k o Ù k k i j d k sb Zv l j u g h ag k sr k g SA l ao k g d x H k Zo r h e f g y k v k sa d sc k j se sa D ; k j k ; g S\ v U ; x H k Zo r h e f g y k v k sa d h r j g ] b & F k Sy k l h f e ; k d h l ao k g d e f g y k v k sa e sa H k h v k ; j u d h d e h g k sl d r h g Sv k Sj m U g sa v f r f j D r v k ; j u y su sd h t : j r i M +l d r h g SA b & F k Sy k l h f e ; k d k l ao k g d g k su sd sd k j . k x H k k Zo L F k k d sn k Sj k u F k k sM +h l h j D r k Y i r k H k h T ; k n k [ k r j u k d g k s ldrh gS vkSj dHkh&dHkh [kwu p<+kus dh Hkh ukScr vk ldrh gSA cPps ds tUe ds ckn j D r k Y i r k e sa l q/ k j g k st k r k g SA D ; k d k sb Z, sl k m i p k j g S] f t l l sO ; f D r d k sl ao k g d r k l sN qV d k j k f e y t k , \ b & F k Sy k l h f e ; k d sl k F k i Sn k g q, O ; f D r d k st h o u H k j b l d sl k F k j g u k g k sx k A D ; k b & F k Sy k l h f e ; k d sy { k . k c k n e sa f d l h m e ze sa , d O ; f D r l sn wl j sO ; f D r e sa l ap k f j r @ i zk I r f d , t k l d r sg Sa\ b & F k Sy k l h f e ; kd s y { k . ko k r k o j . k ] [ k wu p < +k u s (blood transfusion) ; kl aØ e . k (infection) d sf d U g h an wl j sl k / u k sa } k j k , d O ; f D r l sn wl j sO ; f D r e sa u g h at k l d r sA D ; k b & F k Sy k l h f e ; k d sl ao k g d j D r n k u d j l d r k g S\ ; f n b & F k Sy k l h f e ; k d sl ao k g d d sg h e k sX y k sf c u d k L r j j D r n k u l ac aè k h j k " V ªh ; e k i n aM i j [ k j k m r j r k g k s] r k so g j D r n k u d j l d r k g SA ; f n b & F k y S k l h f e ; k d k l o a k g d c P p k i n Sk d j u k p k g ] sr k sm l sD ; k d j u k p k f g , \ m U g as v i u si k V u Zj d k sc r k u k p k f g , f d l H ak o r % o g b & F k Sy k l h f e ; k d sl o a k g d g Sv k j S m U g as v i u s i k V u Zj l sH k h g h e k X sy k f sc u & l c a/ a h c h e k f j ; k as d k i r k d j u sd sf y , [ k u w d h t k p a d j k u sd sf y , d g u k p k f g , A , l s k x H k Z/ k j . k d j u sd si g y sg h d j u k p k f g , A ; f n m u d k i k V u Zj H k h b & F k Sy k l h f e ; k d k l o a k g d g ] Sr k sT ; k n k t k u d k j h d sf y , m U g as f d l h f o ' k " sk K l y k g d k j l sl y k g y u sh p k f g , A D ; k l o a k g d d k sv k j S H k h d N q d j u k p k f g , \ l o ak g d d k sb l c k j se as v i u sH k k b @ Zc g u d k sH k h c r k u k p k f g , v k j S m U g as g h e k sX y k sf c u & l ac a/ h c h e k f j ; k sa d k i r k d j u sd sf y , [ k wu d h t k ap d j k u sd h l y k g H k h n su h p k f g , A b & e k r k & f i r kn k su k sa i zH k k f o r X y k sf c u t h u d s l ao k g d & , d ¶ t k sf [ k e & i w. k Z¸ t k sM +k % & t Sl k f d m Q i j c r k ; k x ; k g Sf d b & F k Sy k l h f e ; k d k l ao k g d g k su k L o k L F ; i j d k sb Zf o i j h r v l j u g h aM k y r k ] v x j o g f d l h v U ; l ao k g d d sl k F k v i u k i f j o k j c l k u k p k g r k g S] r k si zR ; sd x H k Z/ k j . k d sl e ; f u E u f y f [ k r l aH k k o u k , a g Sa% & &FkSyklhfe;k 10 b

p k j e as l s, d ; k f u 2 5 » l H ak k o u kg S f d c P p ke t s j ( x H ak h j ) @ b V aj e h f M ; k ( e è ; e ) ] d k i Q h x H ak h j b & F k y S k l h f e ; kl s i h f M r + g k x sk A e t s j @ b V aj e h f M ; k ] b & F k y S k l h f e ; k d k se f sM V j sf su ; u , u f se ; k ; k d y w h t + (Cooley's) , u f se ; k d su k e l st k u k t k r k g Sr F k k j k x sh d k sb & F k y S k l h f e ; k l si h f M r + g k u ssd sd k j . k g k e s k t s k +b x l (homozygous) H k h d g k t k l d r k g A S n k se sa l s, d ; k f u 5 0 » l aH k k o u k g Sf d c P p k b & F k Sy k l h f e ; k d k l ao k g d g k sx k ] r F k k b l d sb y k t d h d k sb Zt : j r u g h ag k sx h A v k Sj p k j e sa , d ; k f u 2 5 » l aH k k o u k d k c P p k b l c h e k j h l si wj h r j g v i zH k k f o r g k sx k A ( v k d `f r 2 ) d b & F k Sy k l h f e ; k b l : i e sa] d v k Sj b n k su k sa g h t h u l qp k : : i l sd k ; Zu g h ad j j g h g k sr h g Sv k Sj ; g f L F k f r g & J a`[ k y k d sv f / d m R i k n u l sv k af ' k d : i l s( d qN g n r d ) l ar qf y r g k sr h g SA b & F k Sy k l h f e ; k d sl ao k g d d h r j g d b & F k Sy k l h f e ; k d k l ao k g d H k h L o L F k j g r k g S] v k Sj m l sf d l h H k h b y k t d st : j r u g h ag k sr h ] y sf d u i zH k k f o r t h u e k r k @ f i r k l sc P p k sa e sa i g qap l d r h g SA ; f n f d l h c P P k sd k sl ao k g d e k r k & f i r k e sa l si zR ; sd l s, d & , d d b t h u ; k e k r k & f i r k e sa l sf d l h , d l sd b t h u v k Sj n wl j sl sb & F k Sy k l h f e ; k t h u v u qo k af ' k d : i l s f e y r h g S] r k sc P p sd k sg h e k sX y k sf c u & l ac a/ h x aH k h j c h e k j h g k st k , x h r F k k b l d su r h t sv k Sj b y k t b & F k Sy k l h f e ; k e st j ; k b & F k Sy k l h f e ; k b aV j e h f M ; k t Sl sg h g k sax sA v U ; v l k e k U ; g h e k sX y k sf c u v k Sj g h e k sX y k sf c u l ac a/ h c h e k f j ; k ¡ d b Zv U ; v l k e k U ; i zd k j d h O ; L d g h e k sX y k sf c u k sa d h i g p k u d h x b Zg S] t k s c u k o V v k Sj j k sx & l ac a/ h i f j . k k e n k su k sa n `f " V d k s. k l s, d & n wl j sl sv y x g Sa] b U g sa lajpukRed gheksXyksfcu :ikarj ds :i esa Hkh tkuk tkrk gS vkSj blesa f l d y l sy c h e k j hd s f y , f t E e sn k j g h e k sX y k sf c u & , l ( HbS) ] g h e k sX y k sf c u & b Z( HbE) ] g h e k sX y k sf c u & l h ( HbC) ] g h e k sX y k sf c u & M h ( HbD) v k Sj g h e k sX y k sf c u y si k sj ( Hb Lepore) ' k k f e y g SaA ; g e k r k & f i r k l sc P p k sa d k sv u qo k af ' k d : i e sa B h d m l h si zd k j f e y r sg Sa] f t l i zd k j ] b l c qd y sV e sa i g y sd h x b Zp p k Zd se qr k f c d b & F k Sy k l h f e ; k i zk I r g k sr k g SA f i Q j H k h ] d so y m U g h ac P p k sa] t k se k r k & f i r k n k su k sa l sg h e k sX y k sf c u y si k sj ; k g h e k sX y k sf c u & , l i zk I r d j r sg S] e sa b l d sb y k t d h t : j r g k sr h g SA

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e k r k & f i r kn k su k sa l s v u qo k af ' k d : i l as i zk I r v U ; v l k e k U ; g h e k sX y k sf c u k sa (HbC ; kHbD ; k HbE) d k d k sb Ze g R o i w. k Zu r h t k u g h ag k sr k g Sv k Sj b l d sb y k t d h H k h t : j r u g h ag k sr h A g k ay k f d e k r k & f i r k e sa l sf d l h , d l s f o f H k U U k g h e k sX y k sf c u k sa ( Lepore, E, S) d h v k Sj n wl j sl sb & F k Sy k l h f e ; k d h v u qo k af ' k d : i l si zk f I r d si f j . k k e L o : i Hb Lepore@ b ] HbE@ b v k Sj HbS@ b t Sl h g h e k sX y k sf c u l e L ; k , ai Sn kg k st k r h g Sa] t k sb & F k Sy k l h f e ; k e st j @ b aV j e h f M ; k d h g h r j g e g R o i w. k Zj D r c h e k f j ; k ag k sr h g Sa v k Sj b u d s b y k t d h t : j r g k sr h g S] v l k e k U ; v k Sj l aj p u k R e d g h e k sX y k sf c u : i k ar j d s l ac a/ e sa v k Sj t k u d k j h u h p sn h x b Zg SA Hb Lepore/b & F k Sy k l h f e ; k bl :i esa b vkSj d thuksa dk nksckjk iquO;ZoLFkk (rearrangement) gksrk gS] ftlls g h e k sX y k sf c u y si k sj (Lepore) u k e d h , d v l k e k U ; g h e k sX y k sf c u c u r h g SA b & F k Sy k l h f e ; k d sl ao k g d d h r j g g h e k sX y k sf c u y si k sj d k l ao k g d H k h , d L o L F k O ; f D r g k sr k g Sv k Sj m l s f d l h b y k t d h t : j r u g h ag k sr h g SA g k ay k f d i zH k k f o r t h u e k r k & f i r k l sm u d sc P p k sa e sa v u qo ak f ' k d : i l sm l h i zd k j i g qยกp l d r h g S] f t l i zd k j b & F k Sy k l h f e ; kd se k e y se sa v u qo ak f ' k d i zk f I r g k sr h g SA v x j d k sb Z c P p ke k r k & f i r kn k su k sa l sv u qo k af ' k d : i l s Hb Lepore i zk I r d j r kg S v F k o ke k r k & f i r ke sa l s , d l s Hb Lepore v k Sj n wl j s l s b & F k Sy k l h f e ; kd s t h u i zk I r d j r kg S] r k s m l c P p s e sa b & F k Sy k l h f e ; ke st j d h r j g g h e k sX y k sf c u & l ac a/ h x aH k h j c h e k j h ( f o d k j ) i Sn k g k st k , x h v k Sj b l i zd k j ] m l sb l h r j g d s b y k t d h t : j r g k sx h ] t Sl h b & F k Sy k l h f e ; k d se k e y se sa g k sr h g SA g h e k sX y k sf c u & b Z (HbE)@ b & F k Sy k l h f e ; k g h e k sX y k sf c u & b Zl c l sT ; k n k i k , t k u so k y sv l k e k U ; g h e k sX y k sf c u e sa l s, d g S] [ k k l d j n f { k . k i wo Z, f ' k ; k e wy d sy k sx k sa e sa ; g T ; k n k i k b Zt k r h g SA b & F k Sy k l h f e ; k d sl ao k g d d h r j g g h e k X sy k f sc u & b Zd k l o a k g d , d L o L F k O ; f D r g k r sk g Sv k j S m l sf d l h b y k t d h t : j r u g h ag k r sh A g k y ak f d i zH k k f o r t h u e k r k & f i r k l sm u d sc P p k sa e sa v u qo k af ' k d : i l sm l h i zd k j i g qยกp l d r h g S] f t l i zd k j b & F k Sy k l h f e ; k d se k e y se sa ( b l d k m Y y s[ k i g y sf d ; k t k p qd k g S) g k sr h g SA v x j d k sb Zc P p k n k sg h e k sX y k sf c u & b Zt h u k sa ; k f u e k r k v k Sj f i r k n k su k sa l s, d & , d ] v u qo k af ' k d : i l si zk I r d j r k g S] r k sH k h o g L o L F k j g sx k v k Sj m l sf d l h b y k t d h t : j r u g h ag k sx h A g h e k sX y k sf c u & b Zr c e g R o i w. k Zg k st k r k g St c d k sb Zc P p k e k r k & f i r k e sa l sf d l h , d l s b & F k Sy k l h f e ; k d h r F k k n wl j sl sg h e k sX y k sf c u & b Zl ao k g d r k i zk I r d j r k g SA HbE/b & F k y S k l h f e ; k , d x H ak h j f o d k j g ] S f t l d sf u n k u d sy { k . k b & F k y S k l h f e ; k b aV j e h f M ; k t Sl sg k sr sg SaA y sf d u ; g b r u sx aH k h j g k sl d r sg Sa t Sl sd h F k Sy k l h f e ; k e st j e sa n s[ k st k l d r sg SaA b &FkSyklhfe;k 13

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dkbsZ O;fDr loakgd gS ;k ugh]a bldk irk dlSs pyxsk\ T ; k n k r j e k e y k sa e sa] v k l k u v k Sj [ k k l y sc k sj sV j h & t k ¡p } k j k i r k y x k ; k t k l d r k g Sf d f d l h O ; f D r e sa b & F k Sy k l h f e ; k ; k g h e k sX y k sf c u & l ac a/ h f d l h n wl j h c h e k j h d sy { k . k g Sa ; k u g h a] t k ¡p k sa d si g y sv k Sj c k n ] v u qo k af ' k d r k d sl ac a/ e sa f o ' k s" k K k sa l sl y k g ( t Sl s& e k U ; , o a u o h u r e t k u d k f j ; k av k Sj e k x Zn ' k Zu m i y C / d j k u k ) d j u sl sb l i j j k sd F k k e d sf u E u f y f [ k r e g Ù o i w. k Zi g y qv k sa d sc k j se sa t k u d k j h i zk I r g k sx h % t k ¡p d g k ad j k b Zt k u h p k f g , \ t k ¡p d si f j . k k e k sa l sD ; k i r k g k sr k g S\ l ao k g d g k su sd k D ; k e r y c g k sr k g S\ v x j i f r & i R u h n k su k sa l ao k g d g k sa] r k sd k Su & d k Su l sf o d Y i m i y C / g Sa\ b & F k Sy k l h f e ; k e st j d h i zÑ f r v k Sj m i p k j

b d k b sZO ; f D r & F k y S k l h f e ; k d k l o a k g d g S; k u g h ] a ; g t k u u sd s f y , y c s k j sV sj h & t k p ¡ F k y S k l h f e ; kd s f y , y c s k j sV sj h & t k p ¡ e as l k / k j . k(routine) [ k u w& t k p ¡ d h t k r h g ] S f t l s d E I y h V C y M d k m V a (CBC) d su k e l st k u k t k r k g A S b l t k p ¡ l s; g i r k y x k ; k t k r k g S f d y k y j D r d k f s' k d k v k as e as g h e k X sy k f sc u d h e k = k k f d r u h g Sv k j S y k y j D r d k f s' k d k v k as d k (MCH) , v k d k j , o ao k Y W; e w f d r u h g ] S f t U g as Ø e ' k % e h u d k i Wl Z D ; y w j g h e k X sy k f sc u o ae h u d k i Wl Z D ; y w j o k Y W; e w (MCV) d su k e l st k u k t k r k g A S b & F k y S k l h f e ; k d sl o a k g d O ; f D r ; k as e as b u n k u sk as d h e k = k k d e g k x sh A y k y j D r d k f s' k d k v k as d sv k d k j , o aL o : i d h t k p ¡ d j u sd sf y , b U g as e k b Ø k L sd k i s } k j k n [ s k k t k r k g A S b & F k y S k l h f e ; k l o a k g d O ; f D r ; k as d h y k y j D r d k f s' k d k , aF k k M s s+ d e y k y j x a d h g k x as h v k j S d b ZL o : i k as (poikilocytosis) e as g k x as h r F k k v k d k j e as N k V sh (microcytosis) g k x as h ] t c f d l k e k U ; y k y j D r d k f s' k d k , aL o : i e as x g j h y k y ] x k y s k d k j v k j S v o r y (concave) g k r sh g A aS b &FkSyklhfe;k 15

f d l h O ; f D r e as b & F k y S k l h f e ; k d sy { k . k k as d h e k t S n wx h d sc k j se as t k u u sd sf y , f d , t k u so k y h d N q t k p a k as e as l s, d y c sk j sV sj h & ç k l s l s g k r sk g ] Sf t l sg h e k X sy k f sc u b y D sV k ªi sQ k j sf sl l (Hb electrophoresis) ; L d g h e k X sy k f sc u d su k e l st k u k t k r k g A S b l i f zØ ; k l so d sØ e ' k % i e z[ qk v k j S N k V ss? k V d k ] as g h e k X sy k f sc u & , (HbA) v k j S g h e k X sy k f sc u & , 2 (HbA2) d h e k = k k d k i r k p y r k g A S o ; L d y k y d k f s' k d k v k as (adult red cells) e as e k t S n w v U ; g h e k X sy k f sc u ] t l S& as H k . wz k g h e k X sy k f sc au (HbF) v k j S g h e k X sy k f sc u & , l (HbS) ] dk irk Hkh byDsVkªisQkjsfsll }kjk yxk;k tk ldrk gAS T;knkrj e k e y k as e ] as d k b s ZO ; f D r l o a k g d g S; k u g h ] a b l d k i r k d j u sd sf y , m Q i j c r k b Zt k p a as d k i Q h g A aS HbA2 d h e k = k k t k sb F k Sy k l h f e ; k l ao k g d k as e sa v D l j c < +t k r h g S] e k i u sd k , d n wl j k ¶ g k b Zi zs' k j f y f D o M & Ø k se sV k sx zk i Q h ¸ ( HPLC) * uke ls tkuh tkus okyh ,d r j h d k [ k k l y sc k sj sV j h r d u h d (technology) g SA b l t k ¡p d k b L r se k y g h e k sX y k sf c u d s: i k ar j k sa ( f o f H k U u i zd k j d sg h e k sX y k sf c u ) d ki r kd j u sd sf y , H k h f d ; kt k r kg SA v i u h m P p l ao sn u ' k h y r k , o af o f ' k " V r k v k Sj [ k k l d j ] r st x f r l sn Sf u d i f j . k k e n su sd sd k j . k v k t d y b l sg h e k sX y k sf c u l sl ac af / r c h e k f j ; k sa d k i r k y x k u sd h l c l se g R o i w. k Zr d u h d e k u k t k r k g SA t c m i j k sD r t k ¡p k sa l sf u f ' p r r k Sj i j d qN i r k u g h ay x i k r k g Sv k Sj b u t k ¡p k sa d sv k / k j i j y sc k sj sV j h d so SK k f u d c h e k j h d h i qf " V d j u se sa l { k e u g h ag k sr sg aS] b l f y , v k Sj n wl j h [ k k l izdkj dh tk¡pas miyC/ gS]a tSls&a vuqoaf'kd tk¡pas ;kfu O;fDr ds Mh,u, ds ijh{k.k ij v k / k f j r t k ¡p saA b l t k ap e sa] f u f ' p r u r h t si j i g q¡p u sd sf y , i f j o k j d sv U ; l n L ; k sa d s[ k wu d h H k h t k ¡p d j u sd h v k o ' ; d r k g k sr h g SaA d b Z v o l j k sa i j ] O ; f D r e sa v k ; j u d h d e h d s d k j . kc h e k j h d h l g h < ax l s i g p k u (diagnosis) u g h ag k si k r h g SA , sl h f L F k f r e sa] O ; f D r d k sd qN v k ; j u d h x k sf y ; k ¡n su k v k Sj d e l sd e , d e g h u sc k n n k sc k j k t k ap d sf y , c qy k u k t : j h g k st k r k g SA b l d sc k n c h e k j h g k su sd k i r k p y l d r k g SA

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l ao k g d i f r & i R u h ( t k sM +k ) ] t k sv i u sc P p k sa d k sg k su so k y st k sf [ k e l s o k f d i Q ( i f j f p r ) g Sa] d si k l v k t d y d b Zm i k ; g SaA o g L o L F k c P p k sa d k st U e n su sv k Sj i zH k k f o r c P p k sa d sf y , m i y C / l c l sv P N h n s[ k H k k y d sf y , d b Zd n e m B k l d r sg SaA m u d sf o d Y i f u f ' p r : i l sv k l k u u g h ag SaA i zR ; sd t k sM +k v k Sj l k e k U ; u k x k f j d ] j k " V ªh ; L o k L F ; i zk f / d k j . k k sa v k Sj j k sf x ; k sa@ j k sf x ; k sa d s l g k ; d l e wg k sa l s f o ' o l u h ; ] f o L r `r v k Sj u b Z t k u d k f j ; k ¡ i zk I r d j l d r s g SaA l ao k g d e k r k & f i r k d k sv i u st k sf [ k e d sc k j se sa t Y n l st Y n i r k p y t k u k p k f g , r k f d v i u sc k j sa e sa l g h i Q Sl y k d j u sd sf y , m u d si k l d k i Q h l e ; g k sA L o k L F ; l so k m i y C / d j k u so k y k sa d h f u E u f y f [ k r f t E e sn k j h g k sr h g S% & g k b ZL d wy e sa ; k m l l si g y s; k u o & f o o k f g r k sa d sf y , x H k k Zo L F k k l si g y s; k x H k Z/ k j . k d j r sg h l ao k g d r k t k ap m i y C / d j k u k A * Bio-Red HPLC for example is considered by many as Gold Standard for Hb determaination

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l ao k g d k sa d k sm f p r t k u d k f j ; k ¡m i y C / d j k u k v k Sj m U g sa l y k g n su k f d m u d si k V Zu j ( ; k i f r ) d sf y , l ao k g d r k d h t k ¡p d j k u k f d r u k t : j h g A S l o a k g d t k M s k + as ( t k f s[ k e i . wk Zt k M s k +) as d k st k u d k j h n u sk & v x j i f r & i R u h n k u sk as b & F k y S k l h f e ; k d sl o ak g d g k ] as r k sg h e k X sy k f sc u & l c a/ a h c h e k f j ; k as d sf o ' k " sk K ( ; k , d f o ' k " sk K v u o q k f a' k d r k & l c a/ a h l y k g d k j (specialist genetic counselor)) l sf e y u sd h t : j r g k r sh g A S ; g f o ' k " sk K m U g as t k f s[ k e d h l g h f L F k f r v k j S m i y C / m i k ; k as d h t k u d k j h n x sk A i zl wf r f o ' k s" k K t U e & i o w Z i g p k u (antenatal diagnosis) l s l c af a/ r (obstetrician) H k h t k M s k as d k sm i y C / i f zØ ; k v k as d sc k j se as v k j S i H zk k f o r c P p k i n Sk d j u sl s c p u sd sg j l H ak o m i k ; k as d h f o L r r ` t k u d k j h n sl d r k g A S b l d sv y k o k ] v x j H k . wz k (fetus) d h t U e & i o w Zt k ¡p d si f j . k k e k as d k è ; k u f d , f c u k ] l H ak k f o r e k r k & f i r k c P p k i n Sk d j u sd s i Q l S y si j d k ; e j g u k p k g r sg ] Sr k sm U g as v k t d h r k j h [ k e as F k y S k l h f e ; k d sm i p k j @ n [ sk H k k y d s f y , f p f d R l k f o K k u } k j k m i y C / d j k , x , f o d Y i k as d h t k u d k j h v o ' ; n h t k u h p k f g , A

f d l h t k sf [ k e i w. k Zt k sM +s d sf y , m i y C / m i k ; & t c n k su k sa i k V Zu j b & F k Sy k l h f e ; k d sl ao k g d g k saA v x j n k s i k V Zu j d s c h p l ac a/ p y j g s g k sa v k Sj m U g sa b l c k r d h t k u d k j h g k s f d o g b & F k Sy k l h f e ; k d sl ao k g d g Sa] r k so sb l d h i j o k g f d , f c u k ' k k n h d j u s; k ' k k n h u g h ad j u s d k i Q Sl y k y sl d r sg Sa] v x j n k su k sa i k V Zu j b & F k Sy k l h f e ; k d sl ao k g d g Sa v k Sj i g y sl sg h ' k k n h ' k qn k g Sa] r k so sf u E u f y f [ k r c k r sa d j l d r sg Sa% & c P p si Sn k u d j sa v F k o k v i u k c P p k u g k su sn sA c P p k x k sn y saA f d l h x Sj & l ao k g d n k r k d so h ; Zl sd `f = k e : i l sx H k Z/ k j . k } k j k c P p si Sn k d j saA p k g st k sg k st k , ] c P p k i n Sk d j u k v k j S H k . wz k d h f L F k f r t k u sc x j S x H k / Z k j . k d j u k A ; g i r k p y t k u sd sc k n f d H k zw. k b & F k Sy k l h f e ; k e st j ; k g h e k X y k sf c u & l ac a/ h v U ; x aH k h j c h e k f j ; k sa l si zH k k f o r g S] x H k Zt k j h j [ k u k A H k zw. k d si zH k k f o r g k su sd h c k r i r k p y t k u si j x H k Z[ k R e d j n su k A b &FkSyklhfe;k 17

F k Sy k l h f e ; k f l aM ªk se v k Sj g h e k sX y k sf c u & l st qM +h v U ; c h e k f j ; k sa d s f y , H k zw. k d h t k ¡p b d k sb Zv t U e k ( t k sv H k h i sV e sa g h g S) c P p k & F k Sy k l h f e ; k e st j ; k b aV j e h f M ; k l si zH k k f o r g S ; k u g h a] ; g r ; d j u sd sf y , r h u i zd k j d h t k ¡p sa d h t k r h d h t k r h g Sa% & ( i) , f E u ; k sl saV sf V l (Amniocentesis) , f E u ; k sl saV sf V l x H k k Z/ k u d s1 5 l I r k g d sc k n x H k k Zo L F k k d h n wl j h f r e k g h e sa d h t k r h g SA v Y V ªk l k m aM d sf n ' k k & f u n sZ' k d k b L r se k y d j r sg q, d k sb Zi zf ' k f { k r i zl wf r f o ' k s" k K e k r k d si sV e sa , d i r y h l wb ZM k y d j F k k sM +k l k , e f u ; k sf V d n zo ] f t l e sa H k zw. k d h d k sf ' k d k , ¡ (fetal cells)g k sr h g Sa] c k g j f u d k y r k g SA b l d sc k n ] y sc k sj sV j h e sa b l d k i j h { k . k d j d s; g i r k f d ; k t k r k g Sf d H k zw. k b & F k Sy k l h f e ; k ( e st j ; k b aV j e h f M ; k ) l si zH k k f o r g S; k u g h aA b l t k ¡p d h o t g l se k r k ; k H k zw. k d k sg k su so k y st k sf [ k e [ k k l u g h ag SaA b l l sx H k Zi k r (miscarriage) dk FkksM+k tksf[ke , f E u ; k sl saV sf V l d k WM k sZl saV sf l l j g r k g St k s1 % 2 0 0 & 1 % 4 0 0 ( 0 5 » l s d e ) e k e y k sa e sa g h g k sr k g SA g k ay k f d i zl wf r f o ' k " sk K d sl k F k p p k Zd j d sb l t k ¡p d sl H k h i g y qv k sa d k sl e > k t k l d r k g SA ( ii) d k WM k sZl saV sf l l ( H k zw. k d s j D r d k u e wu k y su k ) (Cordocentesis) v Y V ªk l k m aM d k b L r se k y d j r sg q, H k zw. k d sv E c f y d y d k WM Z (umbilical cord) e sa] i sV d sj k L r sl s, d c k j h d l wb ZM k y h t k r h g S] f t l d se k è ; e l s[ k wu d h , d N k sV h e k = k k y h t k r h g SA H k zw. k d sj D r (fetal blood) d k sv y x d j d sy sc k sj sV j h e sa b l d k i j h { k . k f d ; k t k r k g SA T ; k n k r j e k e y k sa e sa d q' k y g k F k k sa } k j k i g y si z; k l e sa g h 1 0 0 i zf r ' k r ' k q¼ H k wz. k d k sf ' k d k , ¡i zk I r g k st k r h g SaA ' k q¼ H k zw. k j D r i zk I r d j u se sa f o i Q y r k d s d k j . k k sa v k Sj b l i zf Ø ; k d sn k Sj k u v U ; l aH k k f o r t k sf [ k e k sa d sc k j se sa t U e l si g y sd h t k ap e sa [ k k l ; k X s; r k j [ k u so k y si l z f wr f o ' k " sk K g h f o L r k j l sl e > k l d r sg A aS d k WM k sZl saV sf l l x H k k Zo L F k kd s 1 8 l I r k g c k n f d ; kt k r kg SA b l e sa x H k Zi k r (miscarriage)( 1 & 2 » ) ] j D r & L = k k o (bleeding)] l aØ e . k (infection) v k Sj , f E u ; k sf V d n zo d k f j l k o t Sl sa t k sf [ k e ' k k f e y g k sr sg SA e k Wf y D ; wy j r j h d k sa l s l e ; j g r s [ k k l i g p k u (diagnosis) u s y x H k x i wj h r j g d k WM k sZl sV saf l l d h t x g y sy h g SA d k WM k sl Z asV sf l l d h l y k g v k t d y d so y &FkSyklhfe;k 18 b

m u x H k Zo r h e f g y k v k sa d k sn h t k r h g Sa] f t U g k sau sn sj l sf j i k sZV d h g k s] ; k f t u d sl h o h , l ( u h p sn s[ k sa) d k d k sb Zf u f ' p r u r h t k u g h f u d y i k ; k g k s] r F k k b l d h l y k g r c H k h n h t k r h g S] t c t k sf [ k e i w. k Zt k sM +k sa d sc k j se sa d k sb Zf i N y h t k u d k j h m i y C / u g h ag k sA h o h , l ) (iii) d k Wf j v k sf u d f o y l l Sai f y ax (Chorionic villus sampling)(l l ho h, l H k zw. ke sa g h e k sX y k sf c u & l ac a/ h c h e k f j ; k sa d k i r k y x k u sd k , d r j h d k g SA ; g , f E u ; k sl saV sf l l l s i g y s x H k k Z/ k j . kd s y x H k x 1 0 & 1 1 g Ý r sc k n f d ; k t k l d r k g SA b l e sa i zl wf r & f o ' k s" k K v Y V ªk l k am M d h l g k ; r k l s¶ d k Wf j v k sf u d f o y k b Z¸ ; k f u H k zw. k d sl e k u g ht h u & l ac a/ ht k u d k j hj [ k u s o k y h d k s f ' k d k , ¡ t k s c k n e s a I y s l s a V k d k : i y s y s r h l h o h , l g Sa] d kF k k sM +kl ku e wu kc k g j f u d k y y sr h g S; g d k sf ' k d k , ¡ e k ¡ d s i sV d s j k L r s (transabdomial) , d i r y h l wb Z; k ; k sf u d sj k L r s (transcervical) , d i r y k d SF k sV j M k y d j c k g j f u d k y ht k r hg SaA b l d s c k n d k sf ' k d k v k sa d kf o ' y s" k . k (analysis) d j d si r k y x k ; k t k r k g SA i g y sc r k b Zx b Zi zf Ø ; k v k sa d se qd k c y sb l e sa x H k Zi k r d kF k k sM +k( 2 » r d ) t k sf [ k e j g r kg S v k Sj b l l s H k h F k k sM +kd e t k sf [ k e jDr&L=kko ;k laØe.k dk jgrk gSA blds vykok cgqr FkksM+k tksf[ke vax dk :i f c x M +u s (limb abnormalities) d k j g r k g S] g k ay k f d b l d h l aH k k o u k y x H k x u g h a d sc j k c j g k st k r h g S] v x j l h o h , l 1 0 l I r k g c k n f d ; k t k r k g SA t U e l si g y s c h e k j h d k i r k y x k u sd sv U ; r j h d k sa d h g h r j g ] b l i zf Ø ; k d k b L r se k y d j r sg q, i zl wf r f o ' k s" k K } k j k t k sM +k sd k sl aH k k f o r t k sf [ k e k sa v k Sj i Q k ; n k sa d h t k u d k j h n h t k r h g SA

m i j k sD r r j h d k sa l su e wu si zk I r d j u sd sc k n H k zw. k d h i g p k u d Sl s d h t k r h g S , f E u ; k sl saV sf l l v k Sj l h o h , l n k su k sa M h , u , i j v k / k f j r g Sa] f t U g sa v u qo k af ' k d (genetic) t k ap d s : i e sa H k h t k u kt k r kg S v k Sj b l e sa e k r k & f i r ke sa e k St wn v u qo k af ' k d i f j o r Zu (mutation) d h i g p k u d h t k r h g SaA b l r j g d h t k ¡p e sa e k r k & f i r k l sv u qo k af ' k d : i l s f e y h c h e k f j ; k sa d k i r k y x k u sd sf y , l c l sl V h d l k / u k sa d k b L r se k y g k sr k g SA f i Q j H k h ] t Sl k f d l h H k h t k ¡p d sl k F k g k sl d r k ] b l e sa F k k sM +h l aH k k o u k y sc k sj sV j h e sa vkerkSj ij uewus dk g qb ZH k wy d h H k h j g r h g S] y sf d u , sl k c g qr d e g h g k sr k g SA fo'ys"k.k djus esa yxHkx ,d lIrkg l h o h , l d se k e y se sa] e l y u i z; k sx ' k k y k & f o K k u h d k Wf j v k sf u d f o y k b Z d h d k sf ' k d k v k sa d sM h , u , e sa i k b Z t k u so k y h g h e k sX y k sf c u t h u k sa d k dk le; yxrk gSA v è ; ; u ; g t k u u sd sf y , d j r sg Sa f d c P p sd h t h u i j d k sb Zv l j r k s b &FkSyklhfe;k 19

u g h ai M +k g Sv k Sj o g L o L F k j g sx k v k Sj d g h ao g F k Sy k l h f e ; k d k l ao k g d r k su g h ag k sx k ; k d g h a m l d h g h e k sX y k sf c u t h u k sa i j v l j r k su g h ag k sx k v k Sj o g b & F k Sy k l h f e ; k l si h f M +r r k su g h ag k s t k , x k A v k e r k Sj i j u e wu sd k f o ' y s" k . k d j u se sa y x H k x , d l I r k g d k l e ; y x r k g SA v x j t k p ยก l s; g i r k p y r k g Sf d c P p k i H zk k f o r g Sr k se k r k & f i r k ; g l e > r sg , q f d m l d sf y , t h o u H k j m i p k j d h t : j r g k x sh ] x H k Zd k st k j h j [ k u sd k i Q l S y k y sl d r sg aS ; k i g y sd h x b Zp p k Z d sv u l q k j x H k Zd k sl e k I r d j u sd k i Q l S y k y sl d r sg A aS g k y a k f d v x j x H k Zd k sl e k I r d j u sd k i Q l S y k f y ; k x ; k g k sr k sx H k k Zo L F k k d h f L F k f r d k sn [ sk r sg , q , l s k n k sr j h d k as l sf d ; k t k l d r k g A S

x H k Zl e k I r d j u k i zk aj f H k d v o L F k k e sa x H k Zl e k I r d j u k vxj efgyk 14 lIrkg ls de dh xHkZorh gks] rks 'kq:vkrh nkSj esa xHkZikr djk;k tk ldrk gS] t k sM +s d k sb l l ac a/ e sa l H k h t k u d k f j ; k an h t k u h p k f g , v k Sj m u d sM j v k Sj f p ar k v k sa d k sm f p r i j k e ' k Zn sd j n wj d j u k p k f g , A m U g sa i zl wf r f o ' k s" k K ; k l y k g d k j (counselor) } k j k ; g c r k ; k t k u k p k f g , f d x H k Z[ k R e d j u sl sm u d sf i Q j c P p k i Sn k d j u sd h l aH k k o u k e sa d k sb Zd e h u g h av k , x h v k Sj v x j d k sb Zt k sf [ k e i w. k Zt k sM +k n k sc k j k x H k Z/ k j . k d j r k g Sr k si zH k k f o r c P p k i Sn k d j u sd h l aH k k o u k m r u h g h g k sx h A b l d sv y k o k ] v x j t k sM +k t k u u k p k g s] r k sl k i Q r k Sj l s; g c r k ; k t k u k p k f g , f d v x y h c k j x H k Zo r h g k su si j x H k Ze sa i y j g k c P p k F k Sy k l h f e ; k l si zH k k f o r g S; k u g h a] ; g t k u u sd sf y , t U e l si g y sn k sc k j k t k ยกp d h t : j r g k sx h v k Sj b l d sf y , f c Y d qy o g h i zf ร ; k , av i u k b Z t k , ax h r F k k i Q k ; n s, o at k sf [ k e H k h l e k u g k sx saA n sj l sx H k Zl e k I r d j u k 1 4 l I r k g l sv f / d d h x H k Zo r h e f g y k d sx H k Zd k s[ k R e d j u sd sf y , g k e k sZu ( i zk sL V X y Saf M u ) n sd j x H k Zo r h e f g y k e sa i zl o (delivery) d h f L F k f r i Sn k d h t k r h g SA ; g i zl o d b Z? k aV k sa r d p y l d r k g S] t k si zk j aH k e sa x H k Zl e k I r d j u sd h r j h d sd h r qy u k e sa] e f g y k d k sT ; k n k e k u f l d r d y h i Q n sr k g SA x H k Zl e k I r d j u se sa g qb Zn sj h l se f g y k d sn k sc k j k x H k Zo r h g k su sd h l aH k k o u k i j d k sb Zv l j u g h ai M +r k r F k k l H k h t k u d k f j ; k ยกv k Sj b l l sl ac af / r l o k y k sa d st o k c i zl wf r f o ' k s" k K l sy h t k l d r h g SaA v U ; r j h d as % t U e l si g y sg k su so k y h j k sx d h i g p k u (prenatal diagnosis) v k Sj x H k Zl e k I r d j u sd s r j h d sg j t k sf [ k e i w. k Zt k sM +s ; k d qN l e qn k ; k sa d k sv i u s/ k f e Zd , o al k aL d `f r d p y u k sa d sd k j . k L o h d k ; Zu g h aH k h g k sl d r sg SA &FkSyklhfe;k 20 b

c n f d L e r h l s] t U e & i wo Zt k ¡p v k Sj x H k Zd k sl e k I r f d , f c u k d so y l ao k g d k sa d h i g p k u v k Sj L Ø h f u ax d sl g k j sb l d h l i Q y v k Sj i zH k k o h j k sd F k k e u g h ad h t k l d r h A n [ k y v k Sj e u k so SK k f u d n c k o d k sd e d j u sv k Sj d qN l e qn k ; k sa , o a[ k k l t k sM +k sd sc h p b U g sa L o h d k ; Z cukus ds fy, dqN vU; rjhds fodflr fd, x, gSa] tcfd dqN rjhdksa ij vHkh 'kks/ (research) p y j g k g SA m n k j g . k d sf y , ] e k ¡d sj D r e sa ? k we u so k y h (circulating) H k zw. k & d k sf ' k d k v k sa d k f o ' y s" k . k , d , sl h t k ¡p g S] f t l i j f i N y sn ' k d e sa] e g R o i w. k Z ' k k s/ d j u si j è ; k u f n ; k x ; k g SA g k ay k f d b l d h v i u h l h e k , ¡g ] aS v k j S v k t d h r k j h [ k e ] as ; g H k j k l s e sn a t U e & i o w Zt k p ¡ d h t x g u g h ay sl d r k g A S i zh & b E I y k u V s' k u t su sf V d M k ; X u k sf l l (Pre-implantation genetic diagnosis) (ih th Mh) fiNys n'kd esa fodflr dh xbZ ,d vU; yscksjsVjh i zf Ø ; k g S] f t l e sa ' k q: v k r h H k zw. k l sy h x b Zd k sf ' k d k v k sa ; k l ao k g d e k r k l sf y , x , g h e k sX y k sf c u & l ac a/ h [ k j k f c ; k sa l se qD r v aM k . k qd k f o ' y s" k . k d j u sd sf y , V sL V V ~; wc e sa (invitro) f u " k sp u (fertilisation) d k b L r se k y f d ; k t k r k g SA b l d sd k i Q h e g ax h r d u h d g k su sv k Sj l i Q y x H k k Zo L F k k d sf y , v D l j d b Zi z; k l d j u sd h t : j r g k su sd sc k o t wn ] i h t h M h d k st U e & i wo Zi g p k u d h r y q u k e as y k x sk ] as [ k k l d j o g y k x s t k sx H k Zl e k I r d j u sd sf [ k y k i Q g ] aS u sT ; k n k L o h d k j f d ; k g A S

b &Fky sS klhfe;k dk gkeskt s kbxl gkusk ;k b &Fky S l s hfe;k et s j ¼efsMVjsfsu;u ,ufse;k ;k dy w ht+ ,ufse;k½ dk jkxsh gkuskA g k se k st +k b x l b & F k Sy k l h f e ; k l si h f M +r y k sx l k e k U ; < ax l sg h e k sX y k sf c u u g h ac u k i k r sg S] b l f y , o g l k e k U ; y k y j D r d k sf ' k d k , ¡H k h u g h c u k i k r sg SaA i zR ; sd y k y j D r d k sf ' k d k e sa l k e k U ; l sc g qr d e g h e k sX y k sf c u g k sr h g Sv k Sj b u d h l a[ ; k l k e k U ; l sc g qr d e g k sr h g SA b l l s[ k wu d h d e h g ak st k r h g S] t k sb & F k Sy k l h f e ; k l si h f M +r O ; f D r ; k as e sx aH k h j g k sr h g S] t c d h b & F k Sy k l h f e ; k b aV j e h f M ; k l si h f M +r O ; f D r ; k as e sb l d h x aH k h j r k d e g k sl d r h g SA b & F k Sy k l h f e ; k l si zH k k f o r c P p k t U e d sl e ; l k e k U ; g k sl d r k g S] y sf d u r h u e k g l s, d o " k Zd h m e zd sc h p m l e sx aH k h j [ k wu d h d e h f o d f l r g ak sl d r h g SA ; f n b l d k b y k t u f d ; k t k , r k sT ; k n k r j c P p k sa d h c g qr d e m e ze sa e `R ; qg k st k r h g SA T ; k n k r j e k e y k sa e sa b & F k Sy k l h f e ; k l sd e x aH k h j l e > h t k u so k y h b & F k Sy k l h f e ; k b aV j e h f M ; k l si h f M +r j k sx h x aH k h j [ k wu d h d e h l si h f M +r g ak su sd sc k o t wn ] d e l sd e t h o u d s' k q# v k r h o " k k s± e sf u ; f e r : i l s[ k wu & p < +k , (blood transfusion) f c u k H k h d k e p y k l d r sg SaA g k ay k f d m e zc < +u s d sl k F k & l k F k [ k wu d h d e h d h f L F k f r v k Sj [ k j k c g k sr h t k r h g S] r F k k c p i u d sv k f [ k j h l k y k sa e sa ; k o ; L d g k su si j f u ; f e r : i l s[ k wu y su sd h t : j r i M +l d r h g SA b & F k Sy k l h f e ; k e st j b &FkSyklhfe;k 21

b aV j e h f M ; k d sj k sf x ; k sa e sa x aH k h j , u sf e ; k ( [ k wu d h d e h ) d sv y k o k v U ; v k Sj b & F k Sy k l h f e ; k d b Zr j g d h c h e k f j ; k ¡i Sn k g k st k r h g Sa v k Sj b u d sf y , d b Zr j g d sb y k t k sa d h t : j r g k sr h g SA

b & F k Sy k l h f e ; k e st j d k b y k t D ; k g S\ b l d k c qf u ; k n h b y k t l k e k U ; r % g j p k j l I r k g c k n f u ; f e r : i l s[ k wu p < +k u k g SA f t u c P p k sa d k sl g h r j g l s[ k wu p < +k ; k t k r k g S] o g v P N h r j g c < +r sg Sa v k Sj l k e k U ; t h o u t h r s g SaA f i Q j H k h 3 0 o " k Zl sv f / d t h u sd sf y , b & F k Sy k l h f e ; k e st j d sj k sf x ; k sa e sa c p i u l sg h v k o ' ; d r k l sv f / d v k ; j u (iron, y k Sg )d sg V k u sd sf y , [ k k l b y k t d h H k h t : j r i M +r h g SA v k ; j u d h ; g v f / d r k p < +k b Zx b Z (transfused) y k y j D r d k sf ' k d k v k sa] t k s y x k r k j V wV r h j g r h g Sa] d h g h e k sX y k sf c u l se qD r g k su so k y sv R ; f / d v k ; j u d sd k j . k g k sr h g SA y x k r k j v k ; j u c < +r sj g u sd sd k j . k ' k j h j e sa b l d h e k = k k c g qr T ; k n k g k st k r h g S] t k sv ar r % f n y (heart)] f y o j (liver) v k j S v r a% L = k k o h x f zF k ; k as (endocrine glands) t l S s' k j h j d s e g R o i . wk Zv x ak as d k s[ k j k c d j n r sk g A S] v k o ' ; d r k l sv f / d v k ; j u d k s[ k k l n o k b ; k sa] f t U g sa v k ; j u d h y sf V U x , t saV ~l d g k t k r k g S] } k j k c k g j f d ; k t k l d r k g SA ; g n o k b ; k ¡v k ; j u d k si s' k k c @ i k [ k k u sd sj k L r sc k g j f u d k y n sr h g SA l qj f { k r , o a i zH k k o h < ax l s b L r se k y g k su s o k y ki g y kv k ; j u d h y sf V U x , t saV f M l i Q sf j v k WD l k e h u ; k M sl i Q sj y (Desferal) g SA b l n o k d k b at sD ' k u , d N k sV si ai d k b L r se k y d j r sg q, d b Z? k aV k sa r d ] y x H k x g j j k r d k sR o p k e sa y x k ; k t k r k g SA c g qr e qf ' d y v k Sj n q[ k n k ; d d k e g k su sd sc k o t wn ] ; g m i p k j t h o u & j { k d l k f c r g qv k g SA g k y ak f d e q¡g l sf y , t k u so k y su , v k ; j u d h y sf V U x , t saV d sm i y C / g k st k u sl sf L F k f r e sa / h j s& / h j sl q/ k j g k sj g k g SA e q¡g l sy h t k u so k y h n k s n o k b ; k ¡] f M i Q sj h i zk su (Deferipone L1) v k Sj f M i Q sj k f l j k WD l (Exjade) i g y sl sg h d b Zn s' k k sa e sa v k Si p k f j d r k Sj i j j f t L V M Zg Sa v k Sj n qf u ; k H k j e sa g t k j k sa j k sf x ; k sa } k j k b u d k b L r se k y f d ; k t k j g k g SA F k Sy k l h f e ; k d sl k F k i Sn k g q, c P p k sa l sv k t m E e h n d h t k l d r h g S f d o st h o u d h l k e k U ; v o f / r d t h f o r j g sa c ' k r sZ o g b l d sf y , t : j h l k j sb y k t f u ; f e r : i l sl g h < a+x l sd j k r sj g saA [ k wu p < +k ; st k u sv k Sj v k ; j u d h y s' k u i j f u H k Zj r k d k sv k f [ k j d k j d e d j l d u so k y si Q k e k ZL ; wf V d y i n k F k k s± d se k è ; e l sF k Sy k l h f e ; k e st j d sb y k t e sa v k Sj l q/ k j y k u sd sf y , d k i Q h ' k k s/ p y j g k g SA v k t d h r k j h [ ke sa b l c h e k j h l sd so y v f L F ke T t kd si R z; k i k j s. k (bone marrow transplant) ( c h , e V h ) } k j k g h N V qd k j k i k ; k t k l d r k g Sc ' k r ±s j k x sh l sf c Y d y q e p S d j u so k y sm l d sf d l h H k k b Z; k c g u d h i g p k u &FkSyklhfe;k 22 b

f L F k f r v P N h g k sv k Sj c p i u l sg h o g f u ; f e r : i l sm i p k j y sj g k d h t k , ] j k sx h d sj k sx d h g k sA g k ay k f d v i s{ k k d `r c g qr d e j k sf x ; k sa ( y x H k x 2 0 » ) d k sg h i wj h r j g l se Sp d j r k g qv k n k r k f e y i k , x k A l k F k g h ] v f L F k & e T t k i zR ; k j k si . k v H k h H k h , d e ag x h i zf Ø ; k g Sv k Sj b l d h d k e ; k c h d k L r j m i ; qD r n k r k (donor) v k Sj j k sx h d h c h e k j h d h v o L F k k r F k k c h , e V h d saU n zd sv u qH k o i j f u H k Zj d j r k g SA m i ; qD r n k r k r y k ' k d j u se sa g k su so k y h f n D d r k sa d sf y , , d l aH k o m i k ; ; g g Sf d e k r k & f i r k , d v k Sj c P p k i Sn k d j sa] t k st h f o r , o ai zH k k f o r c P p sd sv u qd wy (compatible) g k sA g k y k af d ; g l qf u f ' p r d j u sd sf y , f d ; g u ; k c P p k , d v u qd wy n k r k g k s] g k y d so " k k s± e sa ¶ i zh & b aI y k aV s' k u t su sf V d M k ; X u k sf l l ¸ & HLA(PGD-HLA) u k e d h , d i zf Ø ; k f o d f l r d h x b Zg SA b l e sa e f g y k d sx H k Ze sa v k j k sf i r f d , t k u so k y sH k zw. k Zd k p ; u y sc k sj sV sj h e sa fd;k tkrk gSa vkSj bl ckr dk è;ku j[kk tkrk gS fd fiNys i`"Bksa esa mYysf[kr PGD r d u h d d h g h r j g u d so y ; g F k Sy k l h f e ; k l se qD r g k s] c f Y d b l d sm Q r d k sa (tissues) d sy { k . k , o ax q. k (HLA type) f c Y d qy o Sl sg h g k s] t Sl si zH k k f o r v k Sj t h f o r c P p sd k g SA b l d sf y , ] t U e d sl e ; ¶ v E c f y d y d k M Z¸ l s[ k wu y sd j b l d h d k sf ' k d k v k sa d k b L r e sk y j k sx h H k k b Z@ c g u e sa v k j k sf i r (transplant) d j u se sa f d ; k t k , x k A n wl j s' k C n k sa e sa] i zH k k f o r H k k b Z@ c g u d sf y , v k n ' k Zn k r k c u k u sd sf y , u o t k r c P p sd k p u qk o ' k : q e as g h d j f y ; k t k r k g A S ; g , d e qf ' d y i zf Ø ; k g S] f t l su Sf r d : i l sl H k h y k sx L o h d k j u g h ad j r sg Sa] y sf d u f i Q j H k h ; g i zf Ø ; k b & F k Sy k l h f e ; k e st j v k Sj v U ; x aH k h j v u qo af ' k d c h e k f j ; k sa l sf u t k r f n y k u sd s f y , e Sp d j r k g qv k n k r k m i y C / d j k u se sa c sg r j g SA thu Fksjih (gene F k Sy k l h f e ; k d k l i Q y (final) b y k t , d v U ; o SK k f u d i zf Ø ; k ] therapy) l sf e y u sd h v k ' k k g S] b l i j v H k h H k h i z; k sx p y j g k g S] b l i zf Ø ; k e sa] j k sx h d h d k sf ' k d k v k sa e as f Ø ; k ' k h y t h u k as d k s' k j h j l sc k g j y c sk j sV sj h e as j [ k k t k r k g Sv k j S b l d sc k n m U g as j k x sh d sj D r c u k u so k y sm Q r d k as e as L F k k f i r d j f n ; k t k r k g ] S f t l l so sl k e k U ; g h e k X sy k f sc u d sl k F k u b Z j D r d k f s' k d k v k as d k f u e k . Zk d j u se as l { k e g k st k r sg A aS j k x sh b l ' k k / s & d k ; ] Zf t l e as n k r k i j f u H k j Z j g u s d h c t k ; j k x sh d h v i u h d k f s' k d k v k as d k g h b L r e s k y f d ; k t k r k g ] S d h l i Q y r k d h v k j s c M h + v k ' k k H k j h u t j k as l sn [ sk j g sg A aS b l l c a/ a e ] as b l i f zØ ; k d h l i Q y r k c h , e V h d h r j g l h f e r u g h a g A S v k ' k k g Sf d f o K k u t Y n g h d b Zr d u h d h d f B u k b ; Z k as i j f o t ; i k y x sk v k j S l c d k sb l c h e k j h l sf u t k r f n y k u sd k l i u k o k L r f o d r k e as c n y t k , x k A

b & FkSyklhfe;k estj gksus dk irk dSls pyrk gS\ b & F k Sy k l h f e ; k d sl k F k i Sn k g q, c P p se sa d k sb ZL i " V y { k . k u g h af n [ k k b Zn sr sg SaA ; g k ¡r d f d y sc k sj sV j h t k ¡p l sH k h b l d k i r k u g h ap y i k r k g S[ k k l d j r c ] t c e k r k & f i r k d h d k sb Z t U e & i wo Z t k ¡p u d h x b Z g k s] v k Sj i f j o k j t k ¡p u g h a d h x b Z g k s] e sad k sb Zv U ; i zH k k f o r c P p ku g k sA c g qr ' k q: v k r h m e z e sa b & F k Sy k l h f e ; ke st j d h i g p k u d j u k b &FkSyklhfe;k 23

b &FkSyklhfe;k dh igpku igys dqN eghuksa vkSj nks o"kZ ls igys dh tk ldrh gSA

d o s y t u sf sV d t k p ¡ k as } k j k g h l H ak o g A S b u t k p ¡ k as e as e k r k & f i r k e as l si R z; d s l s c P p sd k sv u o q k f a' k d : i l sf e y h g h e k X y k f sc u t h u k as d h i g p k u d h t k r h g A S c n f d L e r h l s] t g k ¡b l c h e k j h d k i r k y x k u sd sf y , u o t k r L Ø h f u ax d k ; ZØ e v k ; k sf t r f d , t k r sg Sa] o g k ¡H k h g k su so k y h f u ; f e r (routine) t k ¡p k sa l sb r u h d e m e ze sa b & F k Sy k l h f e ; k d h i g p k u u g h ag k si k r h g SA f i Q j H k h ] b l v o L F k k e sa L Ø h f u ax d j u k g h e k sX y k sf c u l ac a/ h [ k j k f c ; k sa d s v U ; : i k sa] t Sl sa g h e k sX y k sf c u & b Z; k g h e k sX y k sf c u & , l d h e k St wn x h d k i r k y x k u se sa m i ; k sx h l k f c r g k sl d r k g SA T ; k n k r j e k e y k sa e sa] b & F k Sy k l h f e ; k d h i g p k u i g y s d qN e g h u k sa v k Sj n k so " k Zl si g y sd h t k l d r h g SA g k y k af d b & F k Sy k l h f e ; k b aV j e h f M ; k ] t k sv D l j b & F k Sy k l h f e ; k e st j l sd e x aH k h j g k sr h g S] d k i r k d k i Q h y ac sl e ; r d u g h ay x k ; k t k l d r k g SA

& F k Sy k l h f e ; k y sc k sj sV j h e sa b e st j d k i r k y x k u sd sf y , v k e r k Sj i j b L r se k y f d , t k u so k y sr j h d s ( i) j D r & f o K k u l ac a/ h r k f y d k , ¡e k i u k & y k y j D r d k sf ' k d k d k m aV j (red blood cell counter)] t k s, d b y sD V ªk f u d m i d j . k g S] y k y j D r d k sf ' k d k v k sa d sv k d k j v k j S o k WY ; we (volume) r F k k m u e as e k t S n w g h e k X sy k f sc u d h e k = k k d k se k i r k g A S v x j y k y j D r d k f s' k d k v k as d k v k d k j , o ao k WY ; we v k j S m u e as e k t S n w g h e k X sy k f sc u d h e k = k k d k i Q h d e ( g h e k X sy k f sc u d k L r j 2 & 6 g/dl. d sc h p g k ) s g k r sh g ] S r k sF k y S k l h f e ; k l si h f M r + g k u s sd k i r k p y t k r k g A S F k y S k l h f e ; k d sj k f sx ; k as e as v k e r k j S i j i k b Zt k u sc k y h d N q j D r f o K k u l c a/ a h r k f y d k , ¡ (haematological indices) u h p sn h x b Zg S% & g h e k sX y k sf c u (Hb) g/dl 6.8 (j ast 3.9 - 9.3) v k Sl r (mean) : f / j k . k q (corpuscular) g h e k sX y k sf c u (MCH) pg 20.9 (j ast 15-26) v k Sl r d k sf ' k d k o k WY ; we (MCV) FL 65.8 (j ast 57-75) v k Sl r : f / j k . k qg h e k X sy k f sc u e k = k k & MCHC- g/dl. 30.9 (j t sa 26-34) (ii) j D r f i Q Y e (blood film) v k Sj y k y j D r d k sf ' k d kv k Ñ f r f o K k u & e k b Ø k sL d k si l s n s[ k u s i j y k y j D r d k sf ' k d k , a l k e k U ; l s T ; k n ki h y h (hypochromic) v k j S N k V sh (microcytic) e k y e w i M r +h g ] aS v k j S [ k k l d j f d m u e as l s T ; k n k r j d sv k d k j , o aL o : i & , f u l k l s k b V k f sl l v k j S i k b sd k b y k l s k b V k f sl l & v l k e k U ; g k r s sg ] aS t k sl o ak g d e as n [ sk st k u so k y si f j o r u Zk as l sT ; k n k L i " V g k r s sg A aS (iii) bZysDVªksiQksjsfll }kjk gheksXyksfcu dk fo'ys"k.k& ;g ,d ,slh izfd;k gS] tks g h e k sX y k sf c u v . k q] ; k f u g h e k sX y k sf c u & , (HbA)] g h e k sX y k sf c u & , 2 (HbA2) v k Sj g h e k sX y k sf c u & , i Q (HbF) c u k u s o k y s f o f H k U u i zk sV h u k sa d k s v y x d j r h g SA v x j &FkSyklhfe;k 24 b

d k L r j l k e k U ; l sv f / d g k sr k g S] t k s2 0 & 9 0 i zf r ' k r d sc h p g k s g h e k sX y k sf c u & , i Q l d r k g S] r k sF k Sy k l h f e ; k d sM k ; X u k sf l l d h l y k g n h t k r h g SA g h e k sX y k sf c u & , 2 ] t k s v D l j l k e k U ; o ; L d g h e k sX y k sf c u d s3 i zf r ' k r r d g k sr k g S] d h e k = k k ' k wU ; ] d e ] l k e k U ; ; k F k k sM +h c < +h g qb Zg k sl d r h g SA (iv) e k Wf y D ; wy j (molecular) r j h d k sa } k j k % i g p k u d h i qf " V (confirmation) ; k f d l h i g p k u d sn k Sj k u v k Sj v f / d [ k k l t k u d k j h g k f l y d j u sd sf y , ; sd qN [ k k l r j h d sg S] f t u e sa j k sx d k j d t h u l ac a/ h c n y k o (mutation) d h i g p k u d j u sd sf y , M h , u , t k ¡p d k b L r se k y d j r sg q, ] i g p k u d h i qf " V d sv y k o k ] c h e k j h d h x aH k h j r k d s c k j se sa H k h t k u d k j h f e y l d r h g SA g k y k af d b & F k Sy k l h f e ; k e st j d h i g p k u v D l j B h d g k sr h g S] y sf d u f o d k l ' k h y n s' k k sa e sa] t g k ¡e y sf j ; k t Sl h c h e k f j ; k sa d h v f / d r k i g p k u d k sd f B u c u k l d r h g Sa] i g p k u d j u se sa e qf ' d y g k sl d r h g SA e l y u ] e y sf j ; k d sd k j . k , u sf e ; k v k Sj L I y h u k se x sy h (splenomegaly) g k sl d r h g Sa] v k Sj g k y k af d j D r & l ac a/ h i z; k sx ' k k y k d su r h t sf c Y d qy v y x d k sl d r sg Sa] b l f y , j k sx h d h g k y r v k Sj i g p k u d k n k sc k j k e wY ; k ad u d j u sl si g y sj k sx h d k m i p k j e y sf j ; k & j k s/ h n o k b ; k sa l sd j u k i M +l d r k g SA v U ; f L F k f r ; k ¡H k h , u sf e ; k v k Sj L I y h u k se x sy h v k Sj c < +s g q, g h e k sX y k sf c u & , i Q L r j d k d k j . k g k s l d r hg Sa v k Sj v f r f j D r f u n k u v k Sj y sc k sj sV j ht k ¡p k sa d s l k F k, d v ar j l ac U / h (differential) i g p k u t : j h g k st k r k g SA m i p k j ' k q: d j u sl si g y sF k Sy k l h f e ; k d h l V h d i g p k u d j y su k c g qr e g R o i w. k Zg SA

D;k FkSyklhfe;k estj ds jksxh cPps iSnk dj ldrs gS\ g k ¡] d b Zo ; L d j k sx h v c ' k k n h ' k qn k g Sa v k Sj e k r k & f i r k c u p qd sg SaA m u d st h u k sa d k t U e d sl k F k c P p k sa e sa t k u sd h f u E u f y f [ k r l aH k k o u k , ¡g Sa % & (i) v x j e k r k & f i r k e sa l s, d b & F k Sy k l h f e ; k e st j @ b aV j e h f M ; k l si h f M +r g Sv k Sj n wl j sd h b & X y k sf c u t h u sa ( HbA) i wj h r j g f Ø ; k ' k h y g Sa] r k sm u d s v k d f `r & 5 ) l H k h c P p sb & F k y S k l h f e ; k d sL o L F k l o a k g d g k x as A s ( (ii) v x j e k r k & f i r k e sa l s, d b & F k Sy k l h f e ; k d k l ao k g d g Sv k Sj n wl j k b & F k Sy k l h f e ; ke st j d kj k sx h g S] r k s n k s e sa , d ; k f u 5 0 » c P p sa b & F k Sy k l h f e ; k d sl ao k g d g k sax sv k Sj n k se sa , d ; k f u 5 0 » c P p sb & F k Sy k l h f e ; k e st j d sj k sx h ( v k d `f r & 6 ) g k sax sA (iii) v x j e k r k & f i r k n k su k as b & F k Sy k l h f e ; k e st j d sj k sx h g Sa] r k sl H k h c P p sH k h b & F k Sy k l h f e ; k ( v k d `f r & 7 ) e st j d sj k sx h g k sax sA b &FkSyklhfe;k 25

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b &FkSyklhfe;k vkSj vU; gheksXyksfcu&laca/kh chekfj;k¡ fdu txgksa ij ikbZ tkrh gSa\ ' k q: e sa F k Sy k l h f e ; k d k sH k w& e è ; l k x j h ; b y k d sr d l h f e r c h e k j h l e > k t k r k F k k A b l f y , b l d k u k e i M +k e sf M V sj sf u ; u , u sf e ; k v k Sj F k Sy k l h f e ; k ] t k sF k Sy k l k ( l k x j ) v k Sj , u sf e ; k ( [ k wu d h d e h ) l sf u d y k , d f e f J r x zh d (Greek) ' k C n g S] y sf d u v c i r k p y k g Sf d g h e k sX y k sf c u & l ac aè k h c h e k f j ; k sa l sn qf u ; k d sd b Zf g L l k sa d sy k sx i h f M +r g SaA ; g c h e k j h n f { k . k ; wj k si e sa i qr Zx k y l sy sd j L i su ] b V y h v k Sj x zh l ] d b Zi wo h Z; wj k si d sn s' k k sa] i wj se è ; & i wo Zl sb Zj k u ] i k f d L r k u ] H k k j r ] c k aX y k n s' k ] F k k b Zy SaM ] e y sf ' k ; k ] b aM k su sf ' k ; kv k Sj n f { k . k h p h u r d r F k k v Ú h d k d sm Ù k j h r V d sn s' k k sa v k Sj n f { k . k v e sf j d k r d i Q Sy h g qb Zg SA F k Sy k l h f e ; k [ k k l d j m u { k s= k k sa e sa T ; k n k g S] t g k ¡e y sf j ; k , d f o ' k s" k { k s= k h ; c h e k j h g S; k d H k h F k h A , sl k e k u k t k r k g Sf d n qf u ; k d sb u { k s= k k sa e sa e k u o d s t h u k sa e sa F k k sM +kc n y k o v kx ; k& , d thu&laca/h lek;kstu ;k tho&foKku ds 'kCnksa e sa m R i f j o r Zu (mutation)A b l c n y k o d s d k j . ky k y j D r d k sf ' k d k v k sa d s o k r k o j . ke sa e g R o i w. k Z i f j o r Zu g q, ]f t l u s e y sf j ; kd s i j t h f o ; k sa (parasites) d k sc < +u sv k Sj i u i u sl s e y sf j ; k j k sd f n ; kv k Sj b l i zd k j b u y k sx k sa d k s m u d s e qd k c y s] f t u e sa t h u & l ac a/ h ; g i f j o r Zu u g h a f u ; a= k . k d k ; ZØ e L F k k f i r g k su sl s g q v k ] f t a n k j g u s d k i Q k ; n k (suvival i g y se y sf j ; k l si zH k k f o r n s' k advantage) f e y x ; k A b l f y , , sl k e k u k t k r k g Sf d F k Sy k l h f e ; k ( a v k Sj b ) d sl ao k g d k sa v k Sj f l d y l sy t Sl h g h e k sX y k sf c u l s t qM +h v U ; [ k j k f c ; k sa d sl ao k g d k sa e sa e y sf j ; k l sv i u k c p k o d j u s d h { k e r kL o L F kO ; f D r ; k sa l s T ; k n kF k h A b l f y , n qf u ; kd s e y sf j ; kl s f o ' k s" k: i l s i zH k k f o r { k s= k k sa e sa L o L F k O ; f D r ; k sa d h c M +h l a[ ; k e sa g h e k sX y k sf c u & l ac aè k h c h e k f j ; k sa d k e k Sr g k su sd sd k j . k l ao k g d k sa d h l a[ ; k e sa d qN f o ' o O ; k i h u D ' k k l k y k sa e sa d k i Q h c < +k sr j h g qb ZA "Guidlines to the clinical Management of Thalassaemia" y k sx k sa d s , d n s' kl s n wl j s n s' kt k u s v k Sj 2000 v y x & v y x t k f r ; k sa d s c h p f o o k g d s d k j . k F k Sy k l h f e ; k m Ù k j h ; wj k si v k Sj v U ; n s' k k sa] t g k ¡i g y sF k Sy k l h f e ; k u g h aF k h ] l f g r n qf u ; k d s y x H k x l H k h n s' k k sa e sa i Q Sy p qd h g SA b &FkSyklhfe;k 29

g k y d h e g k e k j h & l ac a/ h t k u d k f j ; k sa d se qr k f c d f o ' o & t u l a[ ; kd ky x H k x 7 i zf r ' k r t u l a[ ; k i zH k k f o r g h e k sX y k sf c u t h u d k l ao k g d g Sa v k Sj i zf r o " k Z3 ] 0 0 ] 0 0 0 l s5 ] 0 0 ] 0 0 0 i zH k k f o r c P p st U e y sj g sg SaA b u e sa l s8 0 i zf r ' k r l sT ; k n k n qf u ; k d sf o d k l ' k h y { k s= k k sa e sa t U e y sr sv k Sj j g r sg SaA b u e sa 7 0 i zf r ' k r e sa f l d y [ k j k c h g S] t c f d c k d h F k Sy k l h f e ; k l ay { k . k l e ; j g r sc h e k j h d ki r ku g h ay x u s; kx y r i g p k u (syndomes) l si h f M +r g SaA (misdiagnosed) d sd k j . k m f p r b Zy k t d sv H k k o e sa ; k c x Sj b y k t d sj g t k u sd h o t g l sv k t H k h f o d k l ' k h y n s' k k sa e sa b l c h e k j h l si zH k k f o r c P p k sa d h c M +h r k n k n e sa e `R ; q g k sj g h g SA ("World Bank 2006, report of a joint WHO - March of Dimes meeting 2006)

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FkSyklhfe;k varjkZ"VÂŞh; QsMjs'ku ds izdk'ku 1. "Blood Safety Kit" (1999) [ In English] 2. "Guidelines to the clinical Management of Thalassaemia" 2000 [ Translated into 6 languages ] 3. "Compliance to Iron Chelation therapy with Desferrioxamine" 2000 reprinted 2005 [ Translated into 4 languages ] 4. "About Thalassaemia" -2003 [ Translated into 11 languages ] 5. "Prevention of Thalassaemias and other Haemoglobinopathies" Volume I (2003) [ Translated into 2 languages ] 6. "Prevention of Thalassaemias and other Haemoglobinopathies" Volume II (2005) [ Translated into English ] 7. "Patients' Rights" 2007 [ In English ] 8. "A guide to the establishment and promotion of non-government patients/parents' organization" 2007 [ In English ] 9. Update version of the book "Guidelines to the Clinical Management of thalassaemia" May 2007 [ In English ] 10. Children's dialogue : "Thalassaemia and Me" 2007 [ In English ] 11. Booklet One: About b -thalassaemia 2007 12. Booklet Two: About a -thalassaemia 2007 13. Booklet Three: "About Sickle Cell Disease" 2007 14. TIF's Educational Folder 2007

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Thalassaemia International Faderation P.O. Box 28807, 2083 Nicosia, Cyprus Tel.: 00357 22319129/ 22319134 Fax: 00357 22314552 E-mail: thalassaemia@cytanet.com.cy Web-site: www.thalassaemia.org.cy

Published by the:

Thalassaemia International Faderation No. 11 ISBN: 978-9963-623-46-4

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