Seated from Left to Right: Dr. Chaminda Garusinghe (Editor), Dr. Dimuthu Weerasooriya(Chairperson academic activities), Dr. Wasantha P. Diassanayake (Vice President), Dr. P.H. Pradeep Silva (President), Dr. Hemantha Gamage (Editor), Dr. Eshantha Perera(Treasurer), Dr. Hemantha Rajapakse (Vice President) Standing from Left to Right: Dr. Vinodani Wanigasekara(Chairperson Social Activities), Dr. P. Sumudu N. Muthukumarana (Secretary), Dr. Sanjeewa Bandara (Secretary), Dr. Jagath Karunarathna, Dr. W.M. P.B. Prageeth Bandara Wasala, Dr. T.M Yajithangani K. Thennakoon, Dr. Niranjala Kumarihamy Absent: Dr. S. P. D.Upul Somathilaka, Dr. G.A. Daminda Dinesh Bandara
KURUNEGALA MEDICAL JOURNAL Volume 5 November 2013
Chronic Kidney Disease of Uncertain aetiology in Sri Lanka....................................... 1 - 8 Dr S Mathu, Consultant Nephrologist
Diagnostic Assessment of First Episode Psychosis ..................................................... 9 - 11 H.P.I. Jayasekera ,MD(Psy) Consultant Psychiatrist, Teaching Hospital, Kurunrgala
Emerging Field Of Health Informatics in Sri Lanka..................................................... 12-15 W M A WijekoonMBBS, MSc (Biomedical Informatics), Dip Diplomacy and World Affairs Provincial Health Informatician, Provincial Department of Health Services - North Western Province, Sri Lanka P E K B RanatungaMBBS, MSc (Biomedical Informatics)
Medical Officer – Health Information, Teaching Hospital - Kurunegala, Sri Lanka
Practical Problems Reported in Relation to Postmortem Examinations in the Kurunegala District......................................................................................... 16 - 17 DR.S.M.H.M.K.Senanayake MBBS (Peradeniya), DLM, MD, DMJ (London),DFM (Glasgow) Consultant Judicial Medical Officer Colombo
Rhinoplasty (Unilateral Cleft Rhinoplasty) – Clinical Audit and Review of literature...............................................................................................................17 - 21 M.H.M.Ashraf, T.Sabesan Registrar, Oro-Maxillo-Facial Surgery. Consultant Oro-Maxillo-Facial Surgeon.
Fatty Liver.......................................................................................................21 - 25 Dr. Dileep Karunarathna Consultant Radiologist, TH Kurunegala
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Undiagnosed Bilateral C3-C4 Dislocation Causing Cervical Spine Instability .............26 - 31
Herath H.M.S, Medical Officer Anaesthesia and ICU Teaching Hospital, Kurunegala
H.M.N.D.H. Menike, Medical Officer ICU, Teaching Hospital, Kurunegala.
Common Oral Ulcerations.............................................................32 - 35 Dr. Manori Jayasinghe Consultant in Restorative Dentistry, General Hospital, Kurunegala Effectiveness of minimal access treatment of varicose veins with foam sclerotherapy. An audit in a peripheral surgical centre..................................................................36 Sirisean RCK, Manoagaran S, Abeyrathna PGPL,Tirichelvan B, General Hospital, Trincomalee, Sri Lanka
Effectiveness of minimal access treatment of appendicitis. An audit in a peripheral surgical centre............................................................................................37 Sirisean RCK, Abeyrathna PGPL, Tirichelvan B, B, Malwatta K, Thangavel N, Goonetilleke CU, Kekulthotuwa KDPD, Weerasena R. General Hospital, Trincomalee,Base Hospital Kuliyapitiya,Sri Lanka.
Scleredema but not Sceroderma...........................................................................38 - 39 D.M.Munasinghe Consultants Dermatologist – T.H. Kurunagala
A herbal plant in near fatal uremia.......................................................................40 - 41 K.L.R.Kalupahana, W.M.P.G.R.Dissanayake, I.M.H.W.Bandara, H.M.A.N.Herath Medical unit B- Teaching Hospital Kurunegala
Mycetoma (Madura foot).....................................................................................42 - 45 Munasinghe CS, Perera MDS Senior Registrar in Radiology and Consultant Radiologist ,Teaching hospital, Kurunegala
Paget's disease involving the entire breast with underlying ductal malignancy- A rare presentation of breast cancer.........................................46 - 47 Bandara I.M.H.W1, Somathilaka S.P.D.U1, Gajanthan K1, Udayakumara E.A.D1 1.Surgical Unit-A, Teaching Hospital Kurunegala
Congenital pseudarthrosis of radius. ................................................47 - 50 D. Tennakoon , D. Dissanayaka
Consultant Orthopaedic Surgeon** Senior House OfficersOrthopaedic Unit A Teaching Hospital Kurunegala
Incarcerated massive parambilical hernia in a morbidly super-super-obese patient...................................................................51 - 52 Bandara I.M.H.W1, Subasinghe D1, Gajanthan K1,Udayakumara E.A.D1
Is Right Hemicolectomy too much for Caecal Leak After Certain Appendicectomy.........................................................................53 - 54 Rajapaksha D.C1, Wickramarathna S. J.D1 1Teaching Hospital Kurunegala, General Surgical unit
Hepatocellular carcinoma: Experience in a general surgical unit at KGTH.....................................................55 Rajapaksha D.C 1 Bogamuwa M.M.M.P.2 1 Consultant General Surgeon Kurunegala Teaching hospital 2 Intern Medical officer Kurunegala Teaching hospital
Kurunegala Medical Journal, 2013 Leading Article
Chronic Kidney Disease of Uncertain aetiology in Sri Lanka Dr S Mathu, MD Consultant Nephrologist
“The only true wisdom is in knowing that you know nothing� - Socrates Chronic Kidney Disease of unknown etiology known as CKDuis an insiduous onset slowly progressive interstitial nephritis leading toend stage renal disease.First reported from Padaviya (1985),CKDu is a baffling health problem with serious socioeconomic implication, eminently in some parts of the North Central region(NCR)like Madawachiciya, Horawapathana, Padaviya, Kabithigollawa, Rambawa, Pollonaruwa, Dehiyattikandiya, Madirigiriya etc.,in the Uva province places like Girandukotte, and some parts of Vavuniya andAmpara Districts. The prevalance of CKDu is steadily increasing over the last three decades
A WHO-MOH expert panel has given a case definition for CKDu as, a state of CKD (as described above) with, 1.No past history of glomerulonephritis orpyelonephritis,(absence of active urinary sediment) 2.Abscence of structural lesion ultrasonically 3.Not on treatment for diabetes/ normal glycosylated haemoglobin (HbA1c; <6.5%) 4.If on treatment for hypertension, blood pressurebelow <140/90 mmHg; if not on treatment forhypertension, blood pressure below <160/100 mmHg.
What is CKDu ? Kidney is an excretory(water & waste) organ with multiple functions, which includes reguationof blood pressure, Erythropoitin synthesis, Vitamine D hydroylation and regulation of water, acid/base and electrolite balance. Each kidney weighs about 150gram and represents 0.5% of body mass but uses about 10% of resting body energy expenditure. In generl kidney can be considered as having three funtiono-structural units glomerulus, tubulointerstitium and vasculature. CKD is defined as Clinical condition lasting more than 3 months, associated with kidney damage- assessed by blood or urine analysis or imaging tests- or reduction of Glomerular Filtration Rate (GFR) to less than 60ml/min/1.73m2. Ingeneral the common causes for CKD are Diabetes mellitus, Hypertension, Chronic glomerularnephritis and hereditary disease which make up abbout 80-90 % of the cases. This is not the case in NCR and majority of those with CKD do not have an identifiable cause.
5.Resident inthe region for >5years What is the burden/prevalance? Based on populationbased study (4) (WHO-MOHMOH) in which S Creatinine with estimatedGFR and Urine albumin to creatinine ratio was used as screening tools the prevalance of CKDu ranges between 15.1-22.9% in Anuradhapura, Polannaruwa and Badulla. In advanced disease, i.e.stages 3 and above tha male to femae ratio is about 3:1. Prevalance increase with age and highest incidence is seen between 30- 60 ages. A positive family history is seen in about 20-30% of the families indicating a common exposure with or without genetic predisposition. As indicated by the medical statistics of theAnuradhapura General Hospital, which is the mainhospital in the north central region, there was a 227%increase in live discharge patients with endstage CKDu, whereas the death rate increased by 354%during the last few years.
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The Nephrology Unit of the Teaching Hospital in Kandy treats 3,000–3,500patients annually, out of which 50–69% of the treatedpatients do not have any identifiable cause andinterestingly most of them are from the north centralregion of the country.Hospital admissions due to diseases of the genitourinary systemhave doubled during the period between 1990 and 2007. During the same period, hospital deaths due todiseases of the genitourinary system rose from 2.6 to 9.1 per 100,000 populations. CKD has beenthepredominant contributor to this rise in in hospital morbidity and mortality.Diabetes andhypertension are considered to be the chief causes of CKD in areas outside the CKDu provinces.
Some cases present with tubular dysfunction such as renal tubular acidosis (with or without hypokalaemic paralysis) and salt loosing nephropathy (well before renal failure set in). Hypocalcaemic seizures have also noted as one of its clinical featue in few patients. Anaemia is present and is often severe than anticipated from the degree of kidney disease. Severe bone mieral disease of CKD is also a prominant clinical feature in estabilished CKDu. CKDu is a relentlessly progressive disaese and peripheral oedema and hypertenion are late features. In majority of the cases urinary sedimant is unremarkable. However RBCuria and WBCuria are noted. In our seriesa significant amount of leucocytes were seen in about 19.2% and significant RBC were demonstrable in 9.6% of patients (unpublished data). One of the feature of CKDu is intermittent low grade proteinuria. With our series, it is notable that despite a diagnosis of CKDu 25.6% of patients had undetectable proteinuria and 28.8% had trace proteinuria as detected by a urinary dipstick on first visit. Howver on serial testing some of them showed presence of proteinuria. Hence dipstic proteinuria is not a screening test to detect CKDu. Evidence of tubular injury is seen very early in CKDU (1). In this atudy it was shown that Urinary excretion of á1-microglobulin (tubular protein) was elevated even in the earliest stage of CKDu compared with its levels in unaffected relative controls. Urinary excretion of N-acetyl-â-D: -glucosaminidase was elevated only in stage 5. Ultrasonically kidneys are shrunken and some show irregular outline. Intrestingly few cases of CKDu only had unltrasonically incresed cortical echogenicity even before the development of proteinuria and or fall of eGFR. Microscopically, Extensive interstiitial fibrosis with tubular atrophy and loss (proximal) and interstitial infilammation with mononuclear cells were the predominant lesions. Glomerulosclerosis is seen as secondary to tubular atrophyand interstitial fibrosis (Chronic interstitial nephritis and fibrosis).Arteriolar hyalinosis indicating hypertensive nephropathy was also observed only a limited number of cases, WHOMOH had advanced diseasee with hypertension.
What is the geographical distribution of CKDu? The affected area coversapproximately 17,000 km2 and with a population ofabout 2.5 million in which more than 95% live in rural areas. Endemic occurrence of the kidney disease was i itially recognized in the North Central Province,situated in the dry zone of Sri Lanka, and this has been increasing steadily.New cases are detectedb in the adjoining districts of North Western province, Uva province, Eastern province, Central province and the Northern province. The endemic foci are scattered in a mosaicpattern, with endemic CKD regions located within fewkilometers from non-endemic villages. Even within anendemic village, certain households may have had thedisease, while neighboring households have had norecorded cases. In some families multiple individuals are effected. The CKDu in Sri Lanka has been compared to the Balkan Endemic Nephropathy (BEN), which in 1950'swas described as a chronic tubulointerstitial kidney disease. The most remarkable characteristic of BENas that it affected only certain endemic rural foci along tributaries of the Danube River in the Balkannations of Bosnia, Bulgaria, Croatia, Romania and Serbia. BEN stands out from Sri Lanka's CKDu in that the geographical distribution is so stable that 50 years after the original description, no new endemicareas have been reported and no endemic areas became free from BEN Clinico Pathology of CKDu(figure 1) Initial presentation of CKDu is usually silent and the renal failure is discovered by routine blood, urine or radiologic investigation. 2
What is/are the causative factors? CKDu is a tubular interstitial disease.Toxins and chronic ischaemia are genearraly the cause for tubular interstitial diseasesand glomerulus is susceptible for immune injury. Toxic injury to the kidney could occur following exposure to an increasing number of chemicals and drugs. Potential agents include substances in the food and ambient environment as well as drugs or diagnostic agents used in medical practice. As kidney reaceive about 20-15% of cardiac output, have a very high rate of oxygen consumptionand normally concentrate potentially tocic substance in the tubular lumen it is vulnerable for tooxic injury. Since the kidney can only respond to injury by a limited number of ways, the underlying pathology and clinical appearences of toxic nephropathy tends to overlp with other forms of kidney disease. Appreciation of spectrum of effects that may result from toxicinjury may help in recognising a specific aetiological agentt The exact aetiology ofCKDu is not yetconfirmed. Number of researchers are investigating and we shall look into the available evidence. In order to prove causation enequvocally agent, source, media, route, suscebtibility, health effect and prevention of disease by nonexposure should be shown. Which is a daunting task. Model linking the sources of causative agent, carrier and the effects in CKDu
Current hypotheses on etiology of CKDu are listed below (not complete and some of them disproved). 1.Cadmium poisoning through use of contaminated groundwater with cadmium containing pesticides and fertilizers and direct exposure 2.Arenic poisoning through the use of arsenic contaminated groundwater of hardness, with arsenic containing fertilizers and pesticides under conditions of heat stress and dehydration 3.Toxic compounds generated by groundwater fluoride content with aluminum species released from low-quality aluminum utensils used by the inhabitants in CKDu affected areas 4.Use of water contaminated with cynobacterial toxins 5.Ochratoxin A 6.nephrotoxic agrochemicals 7.Use of herbal medicine containing Aristolochic acid (from a medicinal plant, Sapsanda) 8.Multifactorial etiology where combination of the above factors contribute to occurrence of CKDu In addition fallowing are hypothesized as contributing factors 1.Dehydration/ malnutrition of farmers and their families 2.Consumption of water with hardness and varying ratio of sodium: calcium 3.Selenium deficiency4.Genetic predisposition Cadmium(3,4,6,7) Cadmium is a well recognised nephrotoxin. It occurs in rocks of the earth crust in and tends to accumulate in soils from fertilizers. The concentration of cadmium reported in the endemic area in the WHOMOH report is double that of the non-endemic area. High concentrations of cadmium are also reported in soils from vegetable plots in low country and up country. This suggests probable accumulation of cadmium perhaps from agrochemicals. Cadmium has also been observed in association with Triple Superphosphate (TSP) fertilizers obtained from rocks rich with this resource. 3
The values presented in the WHO-MOH report and observed by others are in line with what is reported elsewhere as global figures. Cadmium cannot be avoided totally in fertilizer anywhere in the world, as TSP is a major component in fertilizers. Concentrations of cadmium in all types of drinking water is within WHO-MOH limits. Data from a study carried out by Water Resource Board and Chandrajith et al also agrees with this finding. The values are reported on Cd content in rice in WHOMOH report to be below Codex limits. The manyfold high values observedon the Cd content in vegetables from the endemic area in the WHOMOH report suggest contamination of cadmium. High levels of cadmium are also reported in a separate study on leafy vegetables in low country and upcountry vegetables. Its presence in vegetables, but not in rice is probably associated with excessive and frequent spraying of vegetable plots with pesticides as a probable cause. Frequency of spraying rice fields with pesticides is much less. This activity could very well be exposing the vegetable farmers directly to cadmium. WHOMOHreport indicate presence of pesticide residues in CKDU patients further suggesting direct exposure to pesticides. Presence of cadmium in urine Urinary Cd excretion is an indicator of chronic exposure of the element as half life of Cd in the body is 20-30 years.WHO-MOH standards stipulate a urinay excretion of less than 2 microg/g Cr as normal while 10 micg/g Cr would indicate irreversible situation in chronic exposure and potential renal dysfunction. US Occupational safety and Heath administration considers less than 3micg/gCr as safe. However one US (NHANES) observational data showed higher association of albuminuria (potential marker of kidney injury) with urinary Cd excretion above 1 micg/g Cr. Table 1. Urinary Cd (micg/g Cr) patients and controls in Sri Lanka. Data from from Chandrajith et al, Bandaraet al and WHO-MOH report
The data reveals that in subjects with CKDu, urine Cd excretion was significantly higher (mean 1.039, median 0.695,min 0.005, max 8.93 µg/g) compared to healthy subjects in the endemic area (mean 0.646,median 0.18, min 0.005, max 5.13 pg/g) (p < 0.05). This indicates that low dose exposure to Cd could be a risk factor for CKDu in the region. WHOMOH study also revealed aa dose–effect relationship between the concentration of cadmium in urine and the stage of CKDu. In coontrast to Bandara et aland WHO-MOH report Chandrajith et al repoerted lower lwvwls of Cd in CKDu individuals. Urinary excretion of Cd in those living in the endemic area (mean 0.464,median 0.48, min 0.005, max 5.13 µg/g) is higher than those in non endemic area (mean 0.345, median 0.265, min .005,max 2.079 µg/g) (p value <0.001). The WHO-MOH report suggest accumulation of cadmium in nails of CKDU persons in endemic area when comparing those with non-endemic area. High Cd exposure causes bone diseae (called itai itai disease) which is not seen in CKDu. Arsenic(3,4,8,9,10) Soils may contain heavy metals originating from parent rocks during natural weathering, or added during agricultural activities through fertilizer or pesticides. The values for arsenic in soils reported in the WHO-MOHreport are of the same order as those reported by other studies. Fertilizer contains three major elements nitrogen, phosphorus and potassium. TSP is a major component in fertilizer, obtained from rocks rich with phosphorus. All rocks containing TSP carry arsenic as an impurity. Eppawela appetite is reported to contain up to five fold high levels of arsenic than elsewhere. Arsenic cannot be avoided totally in fertilizer anywhere in the world, as TSP is a major component in fertilizers. The levels reported in the WTO report are of the same order as what is generally available globally. The WHO-MOH reported values of arsenic are between 0-94 µg/g in pesticides. In a separate report by the pesticide registrar, it was revealed that 3 out of 22 samples tested contained arsenic(2012). Concentrations of arsenic in all types of drinking water are within WHO-MOH limits.These findings agree with the validated work reported earlier by researchers in Sri Lanka.
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The WHO-MOH report show that theAs values in rice are below Codex limits. Other publications on rice from Sri Lanka, which showed similarly low values between 2005 –2011. A publication of Food and Drugs Administration (USA) in Jan 2013 showed mean of 0.174 µg/g arsenic in rice from USA and India, whereas values reported in Sri Lanka is of the order of 0.030-0.060 µg/g].There are no studies indicating arsenic in vegetables. Presence of Arsenic in urine Urinay As excretion indicates recent As exposure as the half life of As is 2- 4 days in the body. Individuals excreting As <100 micg/L in urine is considered as unexpossed individuals by the United states Department of Health and Human Services Public Health Service Agency for Toxic Substance and Disease Registry.However, over the years threshold levels for safe exposure of toxic metals are being gradualy lowered by competent authorities. In the presence of dermatological features charecterised by Chronic Arsenicosis and/or noncarcinomatous manifestation of chronic arsenicosis (in which CKD is notabaly not one criteria as CKD is a rare complication when compared with others) and /or cencers attributable to chronic Arsenicosis authorities consider a urine As ecretion of >50 micg/L as attributable to As toxicity. However a heavy seafood consumption can exceed this limit and seafood must be exempted from food for 48 hours before the measurment of urine As levels (Seafood contain organic As which is not toxic).One Taiwanese study found in the presence of low lycopene (an anti oxident),urinary As excrretion above a level of 20.75micg/g Cr showed a dose response corelation with CKD. (Please note to convert Arsenic (ìg As/g creat) value to As micg/L value or vice versa, urine creatinine concentration need to be known)
Table 2. Urine As concetration in Bangladesh population exposed to high As levels in water
Here even in the lowest quartile there were individuals excreting more than 100micg/L As in urine. Data available in Sri Lankan scenario is described below Table 3 urinary As (micg/g Cr) patients and controls. Data from from WHO-MOH report
The WHO-MOH study reveals that mean urinary As in CKDu individuals are lower than the healthy controls in the area. Therefore As exposure alone is not a likely cause for CKDu in the region. However the higher mean urinary As in control subjects in the endemic area compared to controls from the nonendemic area indicates exposure to As in the CKDu endemic region. Therefore, the available data indicates that Sri Lankan subjects have a higher exposure than the developed world. However , it is lower than that of countries with patients with well documented chronic As toxicity. As As exposure in Sri Lanka is higher than the West and there are individuals both in enedemic and nonendemic areas with urine excretion levels higher than 100micg/L (50micg/L), measures have to be taken to reduce exposure.
Average background concentrations of As in urine are generally below 10 micg/L in Europe. It is 50 micg/L in Japan.Median urine Total and inorganic As concentration of selected American population is10.5 and 7.2 micg/g Cr.
It is also important to note that co exposureof As is recognised to aggravate the effect of Cd alone on the kidney. Hence when both are found together in an individual in significant levels the threshold to injure the kidney may be at lower levels of each. Further evidence is needed in this regard. New evidence are emerging with low to moderate levels of As exposure in communities, increasing urine arsenic concentrations areassociated with increased albuminuria (marker of kidney injury).
However the landmark study which described the Classic Chronic As toxicity epidemic in Bangladesh showed very highlevel of urine As excretion as shown in the table (includes both cases and controls from endemic region)
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Discussion on lead (4) While fertilizer and pesticides appear to carry lead in recognizable quantities, there is no evidence of lead as a problem in the in test results for urine of CKDU patients against others. However elevated levels of lead has been observed in soils associated vegetables and other crops, but not rice. Mitochondrial swelling and pathognomonic lead inclusionbodies in proximal tubular cells of lead nephropathy are not described in CKDu. Haematological manifestation of lead poisoning is also not reported. In consideration of the carriers into the human body, currentlyavailable data does not support to conclude that Drinking water and rice are risk carriers for three elements referred above. However the vegetables were found to be a risk carrier for Cd as the availability of Cd in vegetables in the endemic area was higher than that of non endemic area.
a positive role in enhancing fluoride intake to the body, the fluoride enhances the leaching of aluminum in cooking utensils and it was hypothesised hhat Aluminofluoride complexes may play a significant role in causing ckd. However Al is not a known nephrotoxin, it is commonly used by the population in sri lanka as a utensil and does not explain the geographical distribution of CKDu patients since it is used in other parts of the country as well. Cyanobacterial toxin Dhammika M Dissanayake of the University of Peradeniya, in a paper in 2011, hasproposed the theory of cyanobacterial toxins. All the high prevalent areas are clustered aroundreservoirs/tanks of the irrigation system. Low prevalence of the disease was noted in communities WHO-MOHconsumed water from natural springs. The analysis of water from natural springs revealed absence ofalgae and cyanobacteria while the analysis of water samples from reservoirs showed the presence of blooms of cyanobacteria. The presence of large numbers of waterreservoirs within a given area in the North Central Region of Sri Lanka could explain the incidence of high prevalence of CKDu.However cyanobacterial toxin is a known cause of actetubular injury, ie, acute kidnney injury, it is not a known cause of CKD. AKI and CKD have entirely defferent pathogenesis and pathology. unless a new cyanobacterial toxin is proven to cause experimentel chronic renal injury this hypothesis cannot explain CKDu. Pesticides(4,11,12) WHO-MOH-MOH study detected pesticide residues in the urine from individuals with CKDu. The f r e q u e n c y o f d e t e c t i o n o f 2 , 4 - D, 3 , 5 , 6 trichloropyridinol, p-nitrophenol, 1-naphthol,2naphthol, glyphosate, AMPA was 33%, 70%, 58%, 100%,100%, 65% and 28% respectively). Isopropoxyphenol,2,4,5-trichlorphenol and pentachlorphenol were below detectionlimitsin that study. Chlopyrifos, Diazynon and propanil are shown in animal studies to cause renal injury Ayurvedic medicines Aristolochic acid nephropathy, first described in Belgium, in women taking a type of slimmimg pill containing Aristolochic acid and ened up in end stage renal disease. This is a well estabilished diseaase dueto chinese herbal medicine containing aritolochic acid.Some researchers implicate Aristolochic acid in the pathogenesis of BEN.
Fluoride in water The link between fluoride geochemistry in water in an area and theincidence of dental and skeletal fluorosis is well established. As in the case of some essential trace elements, the optimum range of fluoride varies within a narrow range and this causes fluoride imbalances, very often in large populations, mostly in developing countries of the tropical belt.In several regions of the dry zone of Sri Lanka, excessive quantities of fluoride in groundwater haveaffected the water quality significantly. According to a 2010 study, high fluoride levels were found in theendemic region as well as the non-endemic regions.This does not explain the geographical distribution of the CKDue cases. Though there are not enough studies on the exposure to natural fluorides and its nephrotoxiceffects on ckd, animal experiments have shown kidney damage with fluoride exposure for along period of time. Flouride is not known to cause direct human renal toxicity. Combination of fluoride in drinking water and aluminum pots According to 2009 study by OAIlleperuma, people affected by CKDu, consumed fluoride rich water and almost exclusively used substandardaluminum pots for cooking and storing water. It was shown that while aluminum plays (especially in acidic cooking condition)
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Aristolochiaindica(Sapsanda) is an ingredient in ayurvedic medicine in Sri lanka. Further research is needed to assessextent of use of Aristolochia plant in Sri Lanka Ochratoxin A (2) Is a naturally occuring mycotoxin with nephrotoxicity that has the potential to contaminate food produts. Wanigasuriya et al assessed 98 randomly selected food samples in NCP and found levels of Ochratoxin A found in these food commodities were below the recommended statutory maximum and are unlikely to be a potential risk factor for CKDu.
Conclusion CKDU is due to an environmental toxin. Theobservation in relation to cadmium as cause of CKDU is that the cadmium makes probably a recognizable contribution to CKDU, perhaps in association with other agents. The observation in relation to arsenic as cause of CKDU is, that the datas does not support scientifically the view that arsenic is the main cause of CKDU, while it may have some contributory effect on the main source.Similarly nephrotoxic pesticides may have a contrbutary role. At present there is no evidence to suggest Pb/Ochratoxin A as a contributory cause to CKDu. Likewise Cyanobacterial toxinis unlikely to be thecausation of CKDu
Selenium deficiency(4) Selenium has been shown to protect the kidney fromoxidative stress. A selenium concentration of 80â&#x20AC;&#x201C;95 ĂŹg/l is needed to maximise the activity of the antioxidantenzyme glutathione peroxidase and selenoproteinsin plasma. In the WHO-MOH-MOH study, about two-thirds(63%) of CKDu subjects had selenium levels below this cut-offvalue. Low seleniumlevels may have been a contributory factor increasingthe vulnerability of the kidneys to oxidative damage caused by heavy metals and metalloids Multifactorial A combination of two or more of above factors and its synergistic effect could be responsible for CKDue. eg Co-exposureto cadmium and arsenic is known to produce additive effects on the kidney that are more pronounced than exposureto either metal alone.
So far most ofthe studies have not implicated water/rice as a causation. High flauride level in the water is a concern for health of an individual but is not directly implicated in ckdu. A number of other potential nephrotoxins(Uranium, Mercury, Gold etc) have not been evaluated adequately. Genetic predispositionn and Selenium deficiency need further investigation to prove ther role Like in the case of BEN, as yet, there have been no definitive answers for the cause of CKDue, Figure (1) Histological apprearance of CKDu under light microscope (H & E stain). Tubular atrophy and interstitial f ibrosis with mononuclear cell inf iltration
It also postulated that naturally found flauride could alter the properties of other trace elements and chemicals making them more potent in toxicity.. Genetic predispostion could explain the familial clustering and also the resistance to CKDu in non affected individuals of the region. All these are hypothesis and need further evaluation. Treatment Primary prevention is prevention of causative factor, which is not yetclear however avaoiding over exposure of Cd/As/pesticides is recommended Secondary prevention or prevention of progression of disease is as for any CKD. 1.BP control (<130/80 mmHg) 2.Control of proteinuria by Renin-AngiotensinAldesterone System inhibition 3.Control of lipids 4.Protein intake moderation 5.Avoidance of tobacco 7
References 1. Nanayakkara S, Komiya T, Ratnatunga N, Senevirathna ST, Harada KH, HitomiT, Gobe G, Muso E, Abeysekera T, Koizumi A: Tubulointerstitial damage asthe major pathological lesion in endemic chronic kidney disease amongfarmers in North Central Province of Sri Lanka. Environ Health Prev Med2012, 17:213–221.
6. Bandara et al Chronic renal failure in cascade irrigation system in Sri Lanka associated with elevated dieteray Cd levels. rice and fresh water fish Environ Geochem and Health 2008, 30:465-478 7. Wanigasuriya KP, Peiris-John RJ, Wickremasinghe R: Chronic kidney diseaseof unknown aetiology in Sri Lanka: is Cd a likely cause? BMC Nephrol2011, 12:32.
2. Wanigasuriya KP, Peiris H, Ileperuma N, Peiris-John RJ, Wickremasinghe R:Could ochratoxin A in food commodities be the cause of chronic kidneydisease in Sri Lanka? Trans R Soc Trop Med Hyg 2008, 102:726–728.
8. Hsueh et al Urenary Arsenic species and CKD in Taiwanese population: A case control studyAm J Kidney Dis 54:859-870. 9. Huang M, Choi SJ, Kim DW, Kim NY, Park CH, Yu SD, Kim DS, Park KS, SongJS, Kim H, Choi BS, Yu IJ, Park JD: Risk assessment of low-level Cd and Ason the kidney. J Toxicol Environ Health A 2009, 72:1493–1498.
3. Chandrajith et al CKDue in Sri lanka:geographic distribution and environmental implication Environmen Gepchem Health 2011,33(3):267-278 4. Jayatilake et al. Chronic kidney disease of uncertain aetiology:prevalence and causative factors in a developingcountryBMC Nephrology 2013, 14:180
10. Hsueh YM, Chung CJ, Shiue HS, Chen JB, Chiang SS, Yang MH, Tai CW, SuCT: Urinary As species and CKD in a Taiwanese population: a case–control study. Am J Kidney Dis 2009, 54:859–870.
5. Ferraro et al Low level exposure to Cd increases the risk of CKD: Analysis of NHANES 1999=2006 BMC Public Health 2010, 10:304 11. Tripathi S, Srivastav AK: Nephrotoxicity induced by long-term oraladministration of different doses of chlorpyrifos. ToxicolInd Health 2010,26(7):439–447 12. Shah MD, Iqbal M: Diazinon-induced oxidative stress and renaldysfunction in rats. Food ChemToxicol 2010, 48(12):3345–3353
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Kurunegala Medical Journal, 2013 Article
Diagnostic Assessment of First Episode Psychosis H.P.I. Jayasekera, MD(Psy) Consultant Psychiatrist, Teaching Hospital, Kurunrgala
Abstract -
“Crude exogenous organic damage of the most varying kind can produce acute psychotic clinical pictures of a basically uniform kind.”(1)discusses the classification, symptoms, investigations and principals of epilepsy management.
AIM: To assist clinicians in carrying out a comprehensive diagnostic assessment in patients who present with a first episode psychosis prior to psychiatric referral.
Symptoms and Signs: Psychosis can be attributed to primary psychiatric and secondary causes. The latter includes delirium (toxic psychosis), neuropsychiatric disorders, medical illnesses, and substance related psychotic disorders. There are no pathognomonic signs or symptoms that unequivocally point clinicians either way. While certain symptoms suggest a medicaltoxic etiology (visual hallucinations, lack of Schneider first rank symptoms), some acute, primary psychiatric presentations can include confusion and perplexity, seemingly implicating a toxic psychosis.
METHOD: A literature review with emphasis on the diagnostic goals of excluding treatable medical causes of psychosis. RESULTS: The guidelines for initial medical workup of first-episode psychosis has no agreed upon standard. Excluding secondary causes of psychosis is based on the likelihood of disease, performance of relevant laboratory tests and evaluation of the relevance of positive test results. CONCLUSIONS: A diagnostic work-up that combines broad screening with medical baseline measures followed by exclusion of specific diseases informed by epidemiology, treatability and clinical relevance appears to be the most cost-effective strategy.
Clinicians, therefore, have to rely on typicality of the clinical picture with regard to age of onset, symptoms, treatment response, and course as well as temporality and biological plausibility to judge whether a medical condition is causally related to psychosis. The overall clinical and epidemiological situation is of utmost importance in narrowing down the broad differential diagnosis of psychosis to keep the diagnostic workup manageable. For example, any new-onset psychosis in a hospitalized, elderly patient following surgery is most likely a toxic psychosis (delirium), whereas an antisocial patient with poly-substance dependence who presents at the emergency department is more likely to be suffering from a drug-induced psychosis.(2)
IntroductionMany medical, toxic and neuropsychiatric disorders can present with psychotic symptoms such as delusions and hallucinations; therefore, a thorough differential diagnosis of possible causes of psychosis is necessary to avoid the mistaken attribution of psychosis to a primary psychiatric disorder. Karl Bonhoeffer, one of the fathers of “organic psychiatry,” recognized a century ago that the psychiatric clinical picture produced by a medical condition was nonspecific, regardless of etiology. 9
â&#x20AC;&#x153;First Episode Psychosisâ&#x20AC;?
In selecting appropriate diagnostic tests, test characteristics (sensitivity and specificity) as well as the prevalence of the disease are key considerations. A positive finding on an examination or a positive laboratory test result alone (a urine drug test positive for cannabis) does not establish causality.
It would be useful to have an operational definition of first episode psychosis in guiding the diagnostic workup. This is complicated by the fact that patients may first present for treatment at varying stages after the onset of illness. Prodromal symptoms may appear many years before the onset of frank psychosis. Keshavan and Scooter (1992) have listed six distinct clinical events that need to be considered in identifying the onset of a first episode psychosis: a) decline in social functioning b) onset of general behavioral symptoms c) onset of positive symptoms d) onset of negative symptoms e) first treatment and f) first hospital admission. (3) It may prove difficult to determine the exact time of onset by relying on clinical history alone. It would therefore be prudent to exclude treatable causes of psychosis in all patients who present with psychosis for the first time, regardless of the duration of their illness.
The extent of the laboratory workup to complement the history and physical examination is a matter of debate. One possible medical workup is a compromise between broad-based screening (ESR, ANA, complete blood count, blood chemistry , glucose, liver function tests) and exclusion of some specific conditions that are treatable if diagnosed ( HIV infection, syphilis, thyroid disease, vitamin B12 and folate deficiency, ceruloplasmin levels). If there is clinical concern, further investigations may be indicated, such as EEGs, lumbar punctures, chest radiography, arterial blood gases, blood and urine cultures, serum cortisol levels, drug screening, toxin search and genetic testing (6).
The diagnostic workup A thorough history and physical examination are the cornerstones for the initial approach to first episode psychosis. The absence of a family history of major mental illness, an acute onset, an age of onset beyond the mid- thirties, and psychosis occurring in a patient being treated in a medical, intensive care or emergency setting would increase the likelihood of a secondary cause of psychosis(4).
Use of Brain Imaging in First Episode Psychosis: Of note, there is no consensus regarding the need for routine brain imaging in first-episode psychosis. Clinically unsuspected, usually neurodevelopmental brain lesions of etiological relevance may occur in 6-10% of schizophrenic patients (7). In a study of 13 adolescents with first episode psychosis, abnormalities of CT scans were detected in two patients, but in neither case did the positive result lead to a diagnosis of brain-lesion related psychosis, nor did it affect the clinical care of the patient.(8). In a systematic review of 8 papers with 625 CT and 214 MRI scans, potentially causal brain abnormalities were 0.8% with CT and 3.3% with MRI scans. The authors concluded that there is no evidence in support of performing routine CT scans in first episode psychosis (9).
A thorough physical examination of all systems with an emphasis on neurological and cognitive assessment is mandatory in all patients presenting with first episode psychosis. Fluctuations in the mental state is suggestive of a toxic psychosis or delirium. To detect fluctuations in mental status typical for a toxic psychosis, repeated visits with bedside testing of cognition may be necessary. If there is a strong clinical suspicion for a disease, its diagnosis must be actively pursued with collateral history, repeated physical examinations and serial tests (serial electroencephalograms or epilepsy)(5).
10
4.Positive test results can be incidental findings or false positives.
On the other hand, many psychiatrists have informally reported cases of first episode psychosis that have later been determined to result from an intracranial lesion. CT or MRI may thus be reserved for patients with an atypical clinical presentation, neurological findings, an unusual or treatment-refractory course and in late onset psychosis in people aged fifty years and older. A normal baseline CT or MRI scan, however,is reassuring and can help patients and families accept that medical and neurological causes of illness have been excluded (10).
5.Routine CT or MRI scans are of little benefit and should be reserved for situations where history or examination suggests a neurological causation. 6.Long-term follow-up is the best safeguard against missing treatable medical conditions.
References 1.Neumärker KJ. Karl Bonhoeffer and the concept of symptomatic psychoses.
Errors in diagnosis
Hist Psychiatry. 2001;12(46, pt 2):213-226. 2.Freudenreich O, Differential Diagnosis of Psychotic
Some medical diagnoses are difficult to make. Clinicians may be unfamiliar with a disease that is rare or they might not recognize a common disease if it presents in an atypical manner. Freudenreich et al have listed six common diagnostic errors: a) missing a toxic psychosis (delirium), b) not obtaining a thorough medical history, c) indiscriminate screening, d) attribution of causality to incidental findings, e) premature diagnostic closure and f) not revisiting the initial impression of a primary psychiatric disorder. Long-term follow-up with attention to new or atypical signs or symptoms is the best safeguard against missing potentially treatable medical conditions (2).
Symptoms: Medical Mimics, Psychiatric Times, Dec 2010, p5661 3.Keshavan MS and Schooler NR. First episode studies in Schizophrenia: criteria and characterization. Schizophrenia Bulletin, 1992; 18(3): 491-513. 4.Sheitman B, Lee H,Strauss R, Lieberman JA. The evaluation and treatment of first-episode psychosis. Schizophrenia Bulletin, 2006: April; 32(2)401. 5.Freudenreich O , Schulz SC, Goff DC. Initial medical work-up of first-episode psychosis: a conceptual review. Early Intervention Psychiatry.2009; 31:10-18. 6.Freudenreich O, Holt DJ, Cather C, et al. The evaluation and management of patients with first-episode schizophrenia: a selective, clinical review of diagnosis, treatment, and prognosis. Harv Rev Psychiatry. 2007;15:189-211.
Summary Ruling out secondary causes of psychosis is important because the causation of psychosis by a medical disorder or substance can dramatically change management and prognosis. Key clinical points to consider when evaluating a patient with new-onset psychosis of unknown etiology are:
9."Patients with first-episode psychosis should not be scanned
1.Psychopathology is not helpful in distinguishing secondary from primary psychosis.
Bjørn Hylsebeck, et al. Ugeskrift for laeger 173.7 (2011): 484. (
7.Differential diagnosis in acute psychotic episode. Falkai P. International Clinical Psychopharmacology, 1996 May; 11 supplement 2:13-17 8.What investigations are ordered in patients with first episode psychosis? Shefrin A, Puddester D, Greenham S, Bisnaire L, Gandy H. Jefferson Journal of Psychiatry, 2006; Volume 20, Number 1 routinely." Ebdrup BH, Lublin H, Akeson P, Glenthoj B. Ebdrup, English Version, PUBMED – indexed for MEDLINE) 10.Use of brain imaging (computed tomography and magnetic
2.History and physical examination that includes vital signs and serial mental status examinations with emphasis on cognition are critical to detecting a toxic psychosis.
resonance imaging) in first-episode psychosis: review and retrospective study. Goulet K, Deschamps B, Evoy F, Trudel JF. Canadian Journal of Psychiatry, 2009 July; 54(7):493-501
3.The extent of the medical workup for psychosis should be determined by prior probabilities with an emphasis on treatable conditions.
11
Kurunegala Medical Journal, 2013 Article
Emerging Field Of Health Informatics in Sri Lanka W M A WijekoonMBBS, MSc (Biomedical Informatics), Dip Diplomacy and World Affairs Provincial Health Informatician, Provincial Department of Health Services - North Western Province, Sri Lanka
P E K BRanatungaMBBS, MSc (Biomedical Informatics) Medical Officer â&#x20AC;&#x201C; Health Information, Teaching Hospital - Kurunegala, Sri Lanka
Complexity of the healthcare system, ethical concerns,limitation of resources and not adhering to proper system development procedureare most significant among many identified reasons for such failures. This has stimulatedthe emergence of the discipline Health Informatics.
Information in Healthcare Healthcareis a sector thatmany decisions are being made routinely and speedily, which is havinga considerable influence on the care receivers, which involves uncertainties and compromises. (1)Therefore informed decision making is vital for the healthcare sector. Hence healthcare is an information intensive field.Maternal and child health in Sri Lanka is one of the best examples in the world for excellent achievements using an optimised information system in the 20th century.
Health Informatics Health Informatics is a discipline at the intersection of Information Science, Statistics, Computer Science, Medicine and Healthcare.Hence it is a broad field with complexity and is multidisciplinary nature
Despite its importance,most of the healthcare organisations and professional at large, has not given the due priority and attention to the information systems. 21st Century has given many new challenges to the healthcare sector anywhere in the world 1.Increasing expectations of care recipients, 2.Concern on safety and quality needs, 3.Limitation of resources, 4.Population growth and aging of the population, and 5.Concern on need to ensureprivacy and confidentiality.
Figure : Health Informatics and related fields Health Informatics has now been developed in to several specialisations 1.Healthcare Informatics 2.Clinical Informatics (A) 3.Community Health Informatics(B) 4.Clinical Bioinformatics Š
Information in Healthcare -
5.Informatics for Education in Health and Medicine (D)
Information systemsin the healthcare sector are needed to be modified accordingly to address these challenges. Healthcare sector is in need of utilising Information science and computer science to have better information systems. There were many attempts worldwide to modernise the information systems in healthcare using Information and communication technology, but most were not successful or could not be sustained.
Figure : Health informatics and related subspecialties (Adapted from Figure Appendix 3 Biomedical and Health Informatics and related fields)(2)
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Clinical Decision Support Systems
Community Health Informatics (Public Health Informatics)
Clinical Decision-Support Systems (CDSS) are computer applications designed to assist clinicians in making diagnostic and therapeutic decisions in patient care utilising current medical knowledge and to analyse patient data to generate case specific assistance at the time of patient encounter. (4) CDSS help clinicians to make better decisions by,
Community Health Informatics (CHI) is thediscipline involved in application of health informatics to practice, research and learning in community health. The primary focuses of community health informatics are on health of populations or communities rather than individuals and concentrate on disease surveillance and prevention.Unlike in clinical informatics, CHI mainly deals with aggregated data.(7)
1.Simplifying access to data needed to make decisions, e.g.: graphical display of laboratory result 2.Provide reminders and prompts,
Clinical Bioinformatics
3.Assist in order entry, e.g. dialog boxalerting the clinician to drug sensitivity or allergy, drug-drug interaction
In bioinformatics biological data is stored, retrieved, organized and analysed using mathematical, statistical and computing methods.This includes analysis of whole genomes and protein structure. Clinical bioinformatics offers the technical infrastructure, tools and knowledge to sanction individualised healthcare, popularly known as personalised medicine.(8)
4.Assist in diagnosis and as a 2nd opinion, 5.Review new clinical data and alert when new patters recognised, e.g.: alert when conflict occurs between newly available sensitivity results and already prescribed antibiotics 6.Minimising errors, e.g.: drug dosage calculated to weight.
ClinicalDecision Support Systems Concerned with how medical images,ranging from xraysand ultra sound scans to MRI and CT scans, are using digital technology to;
Informatics for Education in Healthcareand Medicine Need of continuous professional development a recognised need for the medical professional. Evan for other staff such as paramedics nurses and midwives; regular in-service training is mandatory to keep the staff in par with changing dynamics of the sector. Informatics for education in healthcare and medicine supports Education in Healthcare and Medicine using learner appropriate educational methodologies and technologies for individual or group learning.
1.Provide access to medical images when required, 2.Image processing to enhance the usability of images, and 3.Enable chronological comparison of medical images.
ElectronicMedical Record (EMR or eBHT) An EMR is a digital solution for managing and delivering data required for patient care. Rather than being a mere electronic version of the paper-based record or a database, it presents many functions though integrated CDSS. It usually interface with other systems such as pharmacy, radiology, laboratory, etc.(5) The major advantages of an EMR over a paper-based record are,
International Trends on Health Informatics World Health Organisation (WHO) WHO adopted a resolution in 2005 to establish an eHealth strategy and established the Global Observatory for eHealth. It urges member states to initiate appropriate eHealth services. As WHO has identified that eHealth is transforming the delivery of health services around the world, it is playing a significant role specially in low- and middle-income countries in influencing and monitoring eHealth progress.(9)
1.Retrieval of history when required, 2.Minimise unnecessary of investigations, and 3.Ease in shared care.
ElectronicMedical Record (EMR or eBHT) Clinical audit is a process of quality improvement meant to improve healthcare delivery and outcomes through systematic review of care and services against evidence based standards and effecting changes.(6)
13
United Kingdom (UK)
Some of the most notable Health Information related activities in Sri Lanka are described below.
National Health Service of UK has identified that a major change is needed to deliver the required productivity and efficiency targets and have identified Health Informatics as the key to it.(10)
Master of Science degree in Biomedical Informatics This course enables medical and dental professionals in application of Information Science, medical ethics, Computer Sciences and modern technology in communication to deal with information systems in the fields of healthcare, bioinformatics, medical and health professions education, research, resource optimisation and change management. This course is conducted by the Postgraduate Institute of Medicine (PGIM) of the University of Colombo in collaboration with the Department of Informatics,
India Information technology is used in healthcare sector in India, though its use is very low. Even though large pharmaceutical companies, corporate hospitals and other private health sector institutions use information technology widely, public health sector is far behind in its use. It is understood that India need to develop policy, standards or guidelines to maintain its quality.(11)
of University of Oslo, Norway and conducted as a two year fulltime course.Following successful completion of the MSc in Biomedical Informatics at PGIM, the medical officers and dental surgeons are posted as Health Informaticians. Currently they are appointed to priority stations, namely National Programmes, Provincial Departments of Health Services, Teaching Hospitals and Provincial General Hospitals. Ministry of health has officially requested for a Doctor of Medicine course form PGIM to cater the future need of the country.
Sri Lankan There are many health informatics initiatives and projects that are being carried out in the country. The WHO global survey on eHealth in 2005/6 states thatthe e-Sri Lanka initiative is s the most effective action in building an enabling environment for the use of ICT including the healthcare sector.(9)
Figure : Enabling environment for ICT in the health sector: actions taken or planned within 2 years and their effectiveness rating
14
National eHealth Strategic Plan
References-
National eHealth Strategic Plan will be the strategic master plan directing the state healthcare sector of Sri Lanka towards using electronic systems appropriately, effectively, universally and in a coordinated manner. This was drafted by the eHealth National Technical Committee. Many components of this has been included under the thematic area 1 (Health Sector Modernisation) of the Second Health Sector Development Project 2013 -2016. (14)
1. Hunink MGM, Erasmus M, Siegel JE, et al. Decision Making in Health and Medicine: Integrating Evidence and Values. 1st ed. Cambridge: Cambridge University Press; 2001. 2. Australian Health Informatics Education Council. [Online].; 2011 [cited 2013 October 12. . 3. Gardner RM, Overhage JM, Steen EB, et al. Core Content for the Subspecialty of Clinical Informatics. Journal of the American Medical Informatics Association. 2009 March / April; 16(2).
Healthcare Identification Number (HIN) Computerised systems in the healthcare sector of Sri Lanka shall
4. Payne TH. Computer Decision Support Systems. CHEST Journal. 2000 August; 118(2_suppl). 5. Luo JS. Electronic Medical Records. Primary Psychiatry. 2006; 13(2): p. 20-23.
use the Healthcare Identification Number (HIN) as patients need to be linked to their appropriate medical records. This a unique number issued to each care recipient, which differs from the existing or any future citizen identification number due to severalreasons including the ethical norm of respecting the
6. Healthcare Quality Improvement Partnership. [Online].; 2009 [cited 2013 October 14. . 7. Kukafka R. Public Health Informatics: The Nature of the Field and Its Relevance to Health Promotion Practice. Health Promotion Practice. 2005 January ; 6(1). 8. Chang PL. Clinical Bioinformatics. Chang Gung Med Journal. 2005; 28(4): p. 201-11.
patients'right of privacy and confidentiality. Existing information systems will be updated to support HIN with a reasonable time frame and all new systems are expected to design with support for HIN.
Electronic Indoor Morbidity Mortality Report (eIMMR) As a digitised solution to paper based Indoor Morbidity Mortality Report (IMMR), a web-based eIMMRhas been implemented. It was launched after a period of piloting at selected hospitals. This will enable timely analysis of indoor morbidity and mortality statistics and the timely production of the Annual Health Bulletin.
9. Global Observatory for eHealth. [Online].; 2006 [cited 2013 October 14. 10. Preparing theNHS for an information revolution, BCS report on the NHS Information Revolution consultation on proposals. Swindon: British Informatics Society Limited; 2011. Report No.: ISBN 978-1-78017098-5.
Suwasรกriya Suwasรกriyais a web based health information sharing service, from which any person can access and seek the necessary information according to their need. This programme is aimed at serving Sri Lankans by Sri Lankan Doctors. (15)
11. Kalpa S. Health IT in Indian Healthcare System: A New Initiative. Research Journal of Recent Sciences. 2012 June; 1(6).
Human Resource Management Information system (HRMIS) HRMIS is the system developed for collecting, storing and managing workforce data of state healthcare sector of Sri Lanka. This enables streamlining and increasing the efficacy of day to day and recurrent administrative activities (e.g.: transfers, promotions and training) and enable informed decision making (e.g.: recruitment, deployment and staff development). (16) 15
Kurunegala Medical Journal, 2013 Article
Practical Problems Reported in Relation to Postmortem Examinations in the Kurunegala District DR.S.M.H.M.K.Senanayake MBBS (Peradeniya), DLM, MD, DMJ (London),DFM (Glasgow) Consultant Judicial Medical Officer Colombo
IntroductionAutopsy means the dissection of a dead body to find the cause of death. â&#x20AC;&#x153;Autopsyâ&#x20AC;? word is now replaced by postmortem examination (PME) which includes judicial order, collection of back ground information, scene visit, external examination of the body, dissection and internal examination of the body, investigations and opinion with comments. Not only the cause of death but several Medicolegal issues also are addressed such as identification of unidentified persons, mode of death, manner of death, reconstruction of the fatal event, volitional activities, time since death and injury severity. Various practical problems were seen in every step of the PME during my service in Kurunegala teaching hospital. Different practical problems reported in Kurunegala districtA-Order from the inquirer1- Judicial autopsy is carried out under the order of coroner or magistrate1. Magistrate conducts the inquest on suspected murder cases, road traffic accidents, deaths in custody, rehabilitation centers or psychiatry wards. After the scene the visit, order for a PME is issued. Delay of obtaining the order causes delay of postmortem examination. Kind explanations to relatives can prevent misunderstanding about doctors. 2- Sometimes doctors request inquests and then cancel it. Police and inquirer in to sudden deaths (ISD or Coroner) are harassed due to this unethical practice. Public thinks that doctors change the decisions due to external influences. 3- After receiving the post mortem order from the inquirer, some doctors had issued the death certificate without doing the autopsy. It is contempt of the court.
4- For unnatural deaths (accidents, suicides, homicides) doctors have issued death certificates without requesting an inquest. This mistake had caused severe harassments for the relations, police, Medicolegal practitioners and coroners. 4- When a dead body is transferred from a district hospital to nearest consultant Judicial Medical Officer, new postmortem order should be obtained for the official designation (Judicial medical officer or consultant judicial medical officer depend on the complexity of the case). 5- When an order for a postmortem of a complicated case is issued doctors can educate coroner or magistrate by a letter and request to refer the case to nearest consultant. Putrefied bodies, infant deaths, maternal deaths, skeletons, alleged medical negligence cases, torture cases, high risk autopsy (HIV) and complicated murder2cases are common examples need for referral to consultants. B- Back ground information- Police and relations supply back ground information. In hospitalized cases bed head ticket (BHT) is a compulsory document. When a dead body is transferred, a certified photocopy of BHT also should be sent. Some doctors refuse to send a copy of BHT because it is a confidential document. A copy of the BHT can be sent with an officer of the hospital. Otherwise doctor will be summoned to appear before the inquirer. C- Scene visit is performed by a group consists of inquirer, police and medical officer. Participation to a scene visit is a duty of the nearest Medico-legal practitioner (DMO or JMO). If the body is already moved to the mortuary, retrospective scene visit can be performed when necessary. Police should provide the details of the scene visit to the doctor before the PME.
16 8
D- External examination- clothes, external injuries, postmortem changers and features of illnesses should be noted in the postmortem report. In the court trail, maximum questions are asked on external examination. Reconstruction of the events mainly depends on findings of external examinations. E- Internal examination1- Proper dissection of all the organs are very important to ascertain the cause of death (COD). Some exhumed dead bodies had shown non dissected organs. 2- Postmortem artifacts3 were considered as significant in lot of cases. Some examples are common. Resuscitation artifacts such as sternal fractures and rib fractures were recognized as due to assaults. Reddish brown discoloration of sclera of eyes due to dryness (tachynoire) was considered as a sign of asphyxia. Hypostasis of organs had been recognized as contusions or inflammation. Putrefaction of pancreas was recognized as hemorrhagic pancreatitis. Postmortem autolysis of stomach was considered as rupture of stomach. F- Investigations- Collection of sufficient amount of samples in to correct containers is the first step. Labeling and the preservation is the second step. Dispatch with a proper seal is the third step4. If proper instructions are not given to police, they will not come to transport the samples to laboratories. In doubt full cases collection of all necessary samples, for investigations when necessary is the best option. g- Opinion- Cause of death should be issued as immediate cause , underlying cause and antecedent cause. If there are ante mortem injuries, the pattern of injuries should be commented as suicidal, homicidal or accidental when possible. Relations history should not be written in the postmortem report. Inclusion of relations history and expressing the opinion as â&#x20AC;&#x153; injuries are compatible with autopsy findingsâ&#x20AC;? had caused unjust for innocent people. All the comments should be limited to medical facts found in the postmortem examination and medicolegal opinion. Unlike in Medicolegal report, there is no place to consider the history in a postmortem report.
References1-Chapters II, III, XI and sections 369-373 of the Code of Criminal
ConclusionPractical problems can arise in any step of the PME. Obtaining the advice from nearest consultant can provide the justice to affected parties and also prevent unnecessary appearances in the courts and exhumations.
Procedure (No. 15 of 1979) 2-Knight B. The obscure autopsy. Forensic science international 1980; 16:237-240 3-Alwis LBL. Lecture notes in forensic medicine- volume two; 1st edition 2008.Primal printers, Colombo. pp-114-124 4-Health circular- 545
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Kurunegala Medical Journal, 2013, Review Article
Rhinoplasty (Unilateral Cleft Rhinoplasty) â&#x20AC;&#x201C; Clinical Audit and Review of literature M.H.M.Ashraf, T.Sabesan Registrar, Oro-Maxillo-Facial Surgery. Consultant Oro-Maxillo-Facial Surgeon.
IntroductionRhinoplasty is a nose job, is a plastic surgery procedure for correcting and reconstructing the form, restoring the functions and aesthetically enhancing the nose, by resolving nasal trauma, congenital defect, and respiratory impediment.
Cleft rhinoplasty: Nose surgery procedures that involves repair of secondary deformities of the nose due to congenital clefting Following pictures would simply explain context of the terminology of rhinoplasty
Rhinoplasty was first developed in ancient India, by the ayurvedic physician Sushruta (c. 800 BC), who described reconstruction of the nose in the Sushruta samhita. Rhinoplasty has been performed for different purposes. Cosmetic rhinoplasty: Cosmetic rhinoplasty Nose cosmetic surgery improves the appearance and proportion of nose, enhancing facial harmony and self confidence Traumatic and Post-traumatic rhinoplasty: Nose surgery procedures that involve reconstructing the different elements of Post-traumatic deformities of the nose such the bones, the septum, cartilages, skin and dermal elements
Post-traumatic rhinoplasty
Cleft rhinoplasty
18
Unilateral Cleft Rhinoplasty
Characteristics of unilateral cleft nose deformities
The basic cleft nose deformity is characteristic and
* A nasolabial fistula may be present
dependent upon the original extent of clefting.
*The premaxilla and maxillary segments are
However the secondary nasal defect varies greatly
displaced on the noncleft side
and is a result of 1) original malformation, 2) Any interim surgery performed, and 3) growth of the
* The nasal pyramid is tilted to the cleft side; the
nose and face.
turbinate on the cleft side is hypertrophied
In addition to anatomical and aesthetic problems
* The curved nasal septum and hypertrophied
associated with secondary cleft nose deformities, a
turbinate result in airway obstruction
degree of functional problem also exist in the cleft
* The columella is short on the cleft side and its base
nose. Air flow obstruction, abnormal speech is the
is deviated to the noncleft side
significant functional problems in CLP. Cleft nose
* The lateral crux of the lower lateral cartilage, on
remains 30% smaller than that of patients without
the cleft side, is longer. Its base is attached backwards
cleft lip deformity (Warren et al).
and downwards * The dome of the lower lateral cartilage is displaced
Primary aesthetic and functional deferments of cleft
lower and to the cleft side, which results in bifid tip
nose correlate with severity of CLP. While mild CLP
and excessive skin on the dome of lower lateral
produces least deficiency, severe CLP produces great
cartilage on cleft side
degree of deficiency. Also external nasio labial
* Larger ala forms S-shaped curve or is flat
abnormalities contribute to the diversity
* The nasal tip is asymmetrical
presentation.
* The nostril sill may be small or wide * The nostril is smaller or larger on cleft side
With regard to nose deformity, to analyze the
* The entire nostril may be retro positioned
secondary nose deformity in cleft patients it can be
* The whole nose on the cleft side may be longer as
considered as 2 different entities due to its unique
measured from Radix to the alar margin
micro anatomical abnormality. 1.Secondary nose deformity in unilateral cleft lip and palate (UCLP)
The characteristics of Secondary nose
2.Secondary nose deformity in bilateral cleft lip and
deformities described in various literatures are
palate (BCLP)
compatible with our studies performed in General hospital Badulla and kurunagale in the period of
Secondary nose deformity in patient with cleft lip
January 2009 to December 2011 in 21 unilateral cleft
without palate in both unilateral and bilateral clefts
lip and palate patients who were underwent cleft
simulates their counterparts but the degree of
rhinoplasty
deformity probably less.
19
The following finding were observed: Broad tip 77%, Splayed alar genua â&#x20AC;&#x201C; 65%, Defects of the lower lateral cartilage - 93%, Short columella - 68%, Concave dorsum on cleft side â&#x20AC;&#x201C; 78%
Procedure for correction of cleft lip nasal deformity fall in to 1) Correction of Cartilaginous septum, tip and columella as one unit, 2) correction of ala and alar soft tissue as one unit
Few views
Cartilaginous septum Submucous resection of septal cartilage may be adequate to correct mild septal deviation in most patients. In some patients other methods of septal alterations may be required: 1) Scoring of septal cartilage on
appropriate side,
(2) Excision of caudal part of septum that protruding into the nostril (3) Placing the caudal septum on the nasal spine if it is dislocated. (4) Septoplasty to reduce septal bulk and to correct deflection. (5) Spreader grafting to straiten the septum. Many grafts used for spreader grafting septal cartilage, costal cartilage, calvarial bone, Alloplasts (Medpor)
Patients with facial malformations suffer significant disadvantages in their social lives. Previous studies found higher levels of behavioral inhibition in patients exhibiting a deformed appearance and a higher prevalence of depression and anxiety (Thompson and Kent, 2001). Individuals with a CLP have been found to be affected by social isolation and poor self-esteem (Lockhart, 2003).
We used septal cartilage for spreader grafting where it was necessary in all. Nasal tip Following methods may be necessary to address problems related to tip 1). Repositioning of lower lateral cartilage to correct the nostril level, size and shape 2). The dome on the cleft side must be elevated to the normal side and sutured in the elevated position. 3). To support the hypo plastic weak lower lateral cartilage on lays, in folding of existing cartilage may be necessary 4). To give more definition to the tip, cephalic trimming, a tip graft with a columella strut support may be necessary. Columella The columella may need lengthening using many methods: 1) A simple V-Y plasty from the tip might suffice. 2) A short columella on the cleft side is difficult to correct. Therefore radix adjustment and tip projection is much dependent on soft tissue envelop available for columella lengthening. Nostril sill The alar base excision may be necessary for alar flare.
The optimal timing for the reconstruction of the cleft lip nasal deformity was once unresolved (Clin Plast Surg. Jan 1988.) multiple long-term studies published by experienced cleft surgeons now show that early nasal reconstruction has no adverse effects on growth. (Plast Reconstr Surg. Oct 2007.) The current standard of care is to reconstruct the nose at the time of primary cleft lip repair however; several of the patients still require correction of cleft lip nose deformity later in life. Most surgeons now recognize the broad utility of both endonasal and external approaches. The open rhinoplasty is far from new and particularly used for more complex repairs. In Secondary cleft nose deformity where the deformity is complex in nature which needs good exposure of the nose for repair. (May H. The Rethi incision in rhinoplasty. Plast Reconstr Surg 1951; 8:123). 20
7.LaRossa D, Donath G. Primary nasoplasty in unilateral and bilateral cleft nasal deformity. Clin Plast Surg. Oct 1993; 20(4):781-91. [Medline].
1) To widen the nasal floor, a flap from the lateral aspect of the alar base may need to be transposed to the nostril sill. 2) Conversely, to narrow the wide nasal floor a flap from the nasal floor may be transposed to the lateral aspect of the alar base.
8.Madorsky SJ, Wang TD.Unilateral cleft rhinoplasty: a review.Oto laryngol Clin North Am 1999;32:669-682
Bifid nasal tip Usually it is a feature of bilateral cleft nose deformity. The open rhinoplasty technique is best suited for correcting a bifid tip. The domes should be sutured together and the tip graft should be added if tip projection must be improved. The caudal part of the septum may protrude in one of the nostrils, which may require resection.
9.Mazzola RF. Secondary unilateral cleft lip nose: the external approach. Facial Plast Surg. Oct 1996; 12(4):367-78. [Medline]. 10.Millard DR,Morovic CG.Primary unilateral cleft correction: a 10 year follow up. Plas Reconstr Sur 1998;102:1331-1338 11.O'Connor GB, McGregor MW, Tolleth H. The management of nasal deformities associated with cleft lips.Pac Med Surg. Sep-Oct 1965; 73(5):279-85. [Medline]
The surgical techniques we used in our study patients were Spreader grafting – 87%, Reinforcement of lower lateral cartilage - 83%, Alar repositioning – 92%, Columella lengthening -76% We assesed the the surgical outcome with aesthetic visual analog scale the rsults were.
12.Ortiz-Monasterio F, Olmedo A. Corrective rhinoplasty before puberty: a long-term follow-up. Plast Reconstr Surg. Sep 1981; 68(3):381-91. [Medline].
12 patients scored AVA (8), 7 patients scored AVA (7), 2 patients scored AVA (5) at 12th month review.
13.Rifley W, Thaller SR. The residual cleft lip nasal deformity. An anatomic approach. Clin Plast Surg. Jan 1996; 23(1):81-92. [Medline].
Conclusion The cleft lip nose deformity is a difficult surgical problem to correct. It is necessary to individualize a treatment plan to achieve optimum results. A spate of articles on this subject in the latter part of last century reveals that there is no simple surgical procedure to obtain a harmonious relation between lip and nose. References 1.Anderl H, Hussl H, Ninkovic M. Primary simultaneous lip and nose repair in the unilateral cleft lip and palate. Plast Reconstr Surg. Mar 2008; 121(3):959-70. [Medline] 2.Auricular cartilage grafts .Operative Techniques in Otolaryngology, Vol 19, No 4, December 2008 3.Byrd HS, El-Musa KA, Yazdani A. Definitive repair of the unilateral cleft lip nasal deformity. Plast Reconstr Surg. Oct 2007; 120(5):1348-56. [Medline]. 4.D.V. 454 Bendre, F.A. Ofodile Rhinoplasty in adolescent cleft patients / Oral Maxillofacial Surg Clin N Am 14 (2002) 453–461 5.Gorney M. Rehabilitation for the post-cleft nasolabial stigma. Clin Plast Surg. Jan 1988; 15(1):73-82.[Medline]. 6.Gunter JP, Rohrich RJ. External approach for secondary rhinoplasty.Plast Reconstr Surg 1987; 80:161e74.
14.Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair: a 33-year experience. J Craniofac Surg. Jul 2003;14(4):549-58 15.Salyer KE, Genecov ER, Genecov DG. Unilateral cleft lip-nose repair: a 33-year experience. J Craniofac Surg. Jul 2003;14(4):549-58 16.Sykes JM.The importance of primary rhinoplasty at the time of initial unilateral cleft lip repair.Arch Facial Plast Surg 2010;12(1) 53-55:
Steatosis NAFLD FATTY LIVER
GI bleed NASH Cirrhosis
Liver failure
DISEASE ASH AFLD
Liver cancer Steatosis
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Kurunegala Medical Journal, 2013 Review Article
Fatty Liver Dr. Dileep Karunarathna Consultant Radiologist, TH Kurunegala
IntroductionFatty liver, also known as fatty liver disease (FLD), is a reversible condition where large vacuoles of triglyceride fat accumulate in liver cells via the process of steatosis (i.e. abnormal retention of lipids within a cell). Despite having multiple causes, fatty liver can be considered a single disease that occurs worldwide in those with excessive alcohol intake and those who are obese (with or without effects of insulin resistance). The condition is also associated with other diseases that influence fat metabolism.[1] Morphologically, it is difficult to distinguish alcoholic FLD from nonalcoholic FLD, and both show micro vesicular and macro vesicular fatty changes at different stages. Accumulation of fat may also be accompanied by a progressive inflammation of the liver (hepatitis), called steatohepatitis. By considering the contribution by alcohol, fatty liver may be termed alcoholic steatosis or nonalcoholic fatty liver disease (NAFLD), and the more severe forms as alcoholic steatohepatitis (part of alcoholic liver disease) and Non-alcoholic steatohepatitis (NASH). NASH progresses to scarring and ultimately to cirrhosis, with all the complications of cirrhosis, for example, gastrointestinal bleeding, liver failure, and liver cancer. EPIDEMIOLOGY.30% of the general population has fatty liver, approximately 10% have NASH. Approximately 30% of patients with NAFLD have NASH. Although fatty liver and NASH appear to arise under the same conditions, it does not appear that fatty liver progresses to NASH. Approximately 5 percent of children are affected by fatty liver disease. One of the common risk factors for this condition is obesity. Nearly two thirds of obese adults and half of obese children are found to have fatty liver.
Nonalcoholic Steatohepatitis(NASH) â&#x20AC;&#x201C; the more severe form of fatty liver â&#x20AC;&#x201C; is seen around 20% of the obese individuals. With the rise of obesity over the last couple of decades, there has been a rise in number of individuals with fatty liver. The prevalence of FLD in the general population ranges from 10% to 30% in various countries.[2] However, the condition is observed in up to 75% of obese people, 35% of whom will progress to NAFLD,[15] despite no evidence of excessive alcohol consumption. FLD is the most common cause of abnormal liver function tests in the United States.[2] "Fatty livers occur in 33% of European-Americans, 45% of Hispanic-Americans, and 24% of AfricanAmericans."[16] Causes and risk factors Fatty liver (FL) is commonly associated with alcohol or metabolic syndrome (diabetes, hypertension, obesity and dyslipidemia), but can also be due to any one of many causes[2][3]: Metabolic Abetalipoproteinemia, glycogen storage diseases, Weber-Christian disease, acute fatty liver of pregnancy, lipodystrophy Nutritional Malnutrition, total parenteral nutrition, severe weight loss, refeeding syndrome, jejunoileal bypass, gastric bypass, jejunal diverticulosis with bacterial overgrowth Drugs and toxins Amiodarone, methotrexate, diltiazem, expired tetracycline, highly active antiretroviral therapy, glucocorticoids, tamoxifen, environmental hepatotoxins (e.g., phosphorus, mushroom poisoning)
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Other
PATHOGENISIS
Inflammatory bowel disease, HIV, hepatitis C (especially genotype 3)
Defects in fatty acid metabolism are responsible for pathogenesis of FLD, which may be due to imbalance in energy consumption and its combustion, resulting in lipid storage, or can be a consequence of peripheral resistance to insulin, whereby the transport of fatty acids from adipose tissue to the liver is increased. Severe fatty liver is sometimes accompanied by inflammation, a situation referred to as steatohepatitis. Progression to alcoholic steatohepatitis (ASH) or Non-alcoholic steatohepatitis (NASH) depends on the persistence or severity of the inciting cause.
Some of the risk factors for fatty liver disease include1.Obesity 2.Diabetes 3.Metabolic syndromes that include diabetes, prediabetes or insulin resistance, being overweight or obese, having raised blood cholesterol and triglycerides and high blood pressure Symptoms of fatty liver Fatty liver disease rarely causes symptoms until the liver disease is far advanced. At most, there is enlargement of the liver which may give rise to mild right, upper abdominal discomfort. Commonly there are no symptoms. There may, however, be some amount of abdominal pain that may be concentrated in the central or right upper part of the abdomen. There may be fatigue and tiredness in addition. The liver may be enlarged in some children.
Micrograph of inflamed fatty liver (steatohepatitis)
Some patients get acanthosis nigricans that occurs in the neck or in the under arm region.
Diagnosis of Fatty Liver Disease Often a fatty liver shows up during a routine checkup.
Pathology Blood tests. During routine blood tests, elevations in certain liver enzymes may show up. These might include alanine aminotransferase (ALT) or aspartate aminotransferase (AST). However, blood tests may also be normal in many cases. Imaging studies. A fatty liver may also show up on imaging tests, such as an abdominal ultrasound CT and MRI.
Fatty change represents the intracytoplasmatic accumulation of triglycerides (neutral fats). At the beginning, the hepatocytes present small fat vacuoles (liposomes) around the nucleus (microvesicular fatty change). In this stage, liver cells are filled with multiple fat droplets that do not displace the centrally located nucleus. In the late stages, the size of the vacuoles increases, pushing the nucleus to the periphery of the cell, giving characteristic signet ring appearance (macrovesicular fatty change).
ULTRASOUND GRADING OF FATTY LIVER Right kidney echogenicity was used for determination of liver parenchymal echogenity. With the same kidney cortex and liver parenchymal echogenity it is evaluated as normal, Fat infiltration in liver is described in 3 ultrasonographic stages
Macrovesicular steatosis is the most common form and is typically associated with alcohol, diabetes, obesity and corticosteroids.
Grade 0 : No fatty liver
Acute fatty liver of pregnancy and Reye's syndrome are examples of severe liver disease caused by microvesicular fatty change.[5] The diagnosis of steatosis is made when fat in the liver exceeds 5â&#x20AC;&#x201C;10% by weight.[1][6][7]
Grade1 : Mild fatty liver (Fig. 1) Minimal diffuse increase inhepatic echogenicity where diaphragm, and intrahepatic vessel contours seem normal.
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Grade 2: Medium grade diffuse increase in hepatic echogenicty, mild deterioration in the image of diaphragm and intrahepatic vessels. Grade 3: Severe apparent increase in echogenicity. Posterior segment of the right hepatic lobe is difficult to display. Intrahepatic vessels structure and diaphragm contours are vague or not seen.
Treatment of Fatty Liver Disease There is no specific treatment at this time for fatty liver disease. However, getting treatment for any underlying diseases is essential. Diabetics and those with other associated conditions like high blood cholesterol, high blood pressure etc. need to be treated individually for the same. Treating the underlying cause will reverse the process of steatosis if implemented at an early stage. Patients are advised to take a healthy balanced diet and regular exercise in order to lose weight and maintain a normal body weight. They are advised to avoid alcohol and unnecessary medications. And you can take other steps to improve your condition. If you have alcoholic liver disease and you are a heavy drinker, quitting drinking is the most important thing you can do. Find the support you need to be successful. With ALD, continued use of alcohol can lead to advanced disease, including alcoholic hepatitis or cirrhosis. Even for those with NAFLD, however, avoiding alcohol may help. Prognosis /Complication Patients diagnosed with alcohol induced fatty liver disease had a high risk of developing cirrhosis and premature death, for both men and women. In contrast, patients with grade 1 NAFLD9 seemed to have the same life expectancy as the average normal population and the risk of progressing to end stage liver disease was small.
Figure 1. Ultrasonographic fatty liver stages (in 4 different patients).
Grade 0- no fatty liver, Grade 1- mild fatty liver, Grade 2- moderate fatty liver, Grade 3- severe fatty liver Liver biopsy. The only way to confirm a diagnosis of fatty liver disease is with a liver biopsy. This is usually done once other causes have been ruled out. This is to look for signs of fat, inflammation, and damaged liver cells. If inflammation or damage is not present, the diagnosis is simply a fatty liver.
NASH progresses to scarring and ultimately to cirrhosis, with all the complications of cirrhosis, for example, gastrointestinal bleeding, liver failure, and liver cancer.
Flow chart for diagnosis, modified from[3]
Isolated fatty liver does not progress to NASH or cirrhosis. NASH will become the number one reason for liver transplantation unless effective and safe treatments are found. Up to 10% of cirrhotic alcoholic FLD patients will develop hepatocellular carcinoma. The overall incidence of liver cancer in nonalcoholic FLD has not yet been quantified, but the association is well established.
Criteria for nonalcoholic fatty liver disease: consumption of ethanol less than 20 g/day for women and 30 g/day for men
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Possible outcome of fatty liver disease NAFLD-Non Alcoholic Fatty Liver Disease. AFLD- Alcoholic Fatty Liver Disease NASH- Non Alcoholic SteatoHepatitis. ASH- Alcoholic SteatoHepatitis
Stages of liver damage References 1.http://www.wikipedia.org/wiki/Fatty_liver 2.http://www.webmd.com/hepatitis/fatty-liver-disease 3.http://www.medicinenet.com/fatty_liver/article.htm#nonalcoholic_fatty_liver_facts 4.http://www.news-medical.net/health/Fatty-Liver-What-is-Fatty-Liver.aspx
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Kurunegala Medical Journal, 2013 Case History and Review Article
Undiagnosed Bilateral C3-C4 Dislocation Causing Cervical Spine Instability H.M.S. Herath Medical Officer Anaesthesia and ICU Teaching Hospital, Kurunegala
H.M.N.D.H. Menike Medical Officer ICU, Teaching Hospital, Kurunegala.
IntroductionThe diagnosis of cervical spine injuries remains a significant problem in many blunt trauma victims. These injuries commonly occur in Sri Lanka due to road traffic accidents, falls from heights, sports related and gunshot injuries. Correct and early diagnosis of these injuries is imperative as delay or misdiagnosis results in increased mortality and morbidity which may lead to permanent disability. Case Presentation. A 55 year old male presented to the Intensive Care Unit with hypotension and bradycardia, after falling from a bridge while cycling. He fell into a shallow stream (water level was only about 2 feet). Immediately after the fall the patient was taken to a local hospital without any protective measures to stabilize the cervical spine .At the local hospital he was intubated as there was respiratory inadequacy. The first clinical examination revealed no neurological deficit, so no measures were taken to immobilize the cervical spine. The victim was then transferred to a tertiary care centre where ventilation facilities were available. On examination at our unit the patient was conscious (GCS-6 V-1, E-4, M-1), intubated, quadriplegic with sensory impairment. He was also haemodynamically unstable (80/60) and bradycardic (pulse rate 41 bpm). These clinical features led to the suspicion of cervical spine injury and cervical spine was immobilized with a Philadelphia collar. Two view radiographs (lateral / antero -posterior) and CT scan of the cervical spine were ordered. CT scan showed facet dislocation of C3 â&#x20AC;&#x201C; C4 with right sided interarticular fracture and narrowing of the spinal canal with spinal cord compression.
Skull caliper and traction was applied by the Orthopedic Surgeon immediately after the diagnosis was made. However the patient's neurological deficit did not improve and he developed ventilator associated pneumonia and succumbed to death 6 days following admission.
Dislocation ofC3-C4 with interarticular fracture.
After correction with traction.
Discussion
Introduction Cervical spine injury (CSI) accounts for approximately 65% of all spinal cord injuries. It is equal to 2-3% of all trauma patients. Early recognition of these injuries and stabilization might prevent further neurological deterioration and reduce mortality. Pathophysiological changes occurring in cervical spine injury Respiratory System: After SCI, intercostal muscle function is lost and as a result there is a failure of anterior- posterior expansion of the rib cage. As the diaphragm contracts without intercostals' contraction the chest wall is sucked in causing paradoxical chest wall movement. Although innervation to the diaphragm is usually intact (lesions below C3), its function is also greatly affected post SCI. Loss of innervation to the lower thoracic segment causes the diaphragm to start at a more caudal position. 26 24
In addition, as the diaphragm descend, abdominal contents are pushed out due to lost abdominal muscle tone and fail to provide the fulcrum needed to expand the lower chest. The lower rib cage is pulled in while the abdomen is pushed out resulting in the â&#x20AC;&#x153;see â&#x20AC;&#x201C; sawâ&#x20AC;? pattern of respiration. The rapid shallow breathing is inefficient and a greater percentage of each breath ventilates dead space. As a result inspiration becomes prolonged. Lost abdominal muscle activity results in decreased maximal expiratory force (19) and a reduced ability to cough, clear secretions and protect the airway. Atelectasis increases the load placed on already compromised inspiratory muscles and V/Q mismatching occurs. Alveolar hypoventilation and respiratory failure are very common. Cardio Vascular System: Neurogenic Shock: In Lesions above T6, there is loss of cardiac accelerator fibers resulting in bradycardia and patients are unable to increase cardiac output by changes in heart rate. Hypotension occurs due to loss of sympathetic autonomic function and unopposed parasympathetic function leads to loss of vasoconstrictor tone and venous pooling. Although the duration of neurogenic shock is variable, recovery tends to be incomplete and postural hypotension can be a persistent problem.
The vasomotor centre triggers parasympathetic mediated bradycardia .The clinical picture is one of malignant hypertension with reflex bradycardia. Above the level of the injury, the parasympathetic system causes headache, sweating and nasal congestion, while the sympathetic system predominates below the lesion with resultant skin blotching, goose pimples and cool peripheries. Patient becomes restless, agitated and may become unconscious, develop seizures, stroke and potentially die. Other Systems: Delayed gastric emptying and paralytic ileus are common after SCI and may last for 2-3 weeks. Vomiting may occur and there is a high risk of pulmonary aspiration. Secondary to unopposed vagal activity below the level of the injury, gastric stress ulceration is often seen. In SCI patients, constipation is often problematic as sensation of defecation is lost. Vasodilatation causes aberrant temperature regulation and hypothermia with the patient's temperature equilibrating with environmental temperature. Pre Hospital Management The knowledge of pre hospital trauma and emergency care is scanty among Sri Lankan general population. Emergency Medical Services ( EMS ) are still not available in our country. Careful and fast transportation of trauma victims from the site of injury the nearest medical facility is recommended. The standard pre hospital practice for any patient who has suffered injury or trauma (often it may only be a relatively minor injury) is to have full spinal cord immobilization at the scene .This can be achieved by both rigid cervical collar, a spinal board with head blocks or sand bags and tapes to keep the head completely still with spinal column 'in line'. All trauma patients should be treated as with cervical spine injuries until proven otherwise. Hospital Management Every doctor involved in trauma and emergency management should have a thorough knowledge of identifying and managing C spine fractures. In acute severe trauma, resuscitation should be according to the ATLS guidelines. (1, 2)
DVT: The principal cause for deep vein thrombosis in SCI is loss of mobility and vasodilatation resulting in venous pooling and stagnant blood flow. The risk of DVT is increased three fold in SCI; lower in the first 72 hours and increasing thereafter. (19) Sympathetic Hyperreflexia; This manifestation occurs after 4-6 weeks, once the neurogenic shock has resolved and reflexes have returned. Hyperreflexia is common in lesions above T6 (above the splanchnic sympathetic out flow) and rare with lesions below T10. This is a life threatening condition triggered by somatic or visceral stimuli below the level of the injury, classically with bladder or rectal distention. Individual patients will be unaware of this stimulus because of sensory loss. Triggering stimuli generate an ascending sensory nerve impulse (spinothalmic and posterior columns) that stimulates the sympathetic nervous system in the spinal cord (intermediolateral gray matter) below the level of injury and with compensatory baroreceptor mediated vasodilatation above the level of the injury. 27
Injuries below C6 diaphragm and accessory cervical inspiratory muscles are intact with paralyzed intercostals and abdominal muscles. Respiratory failure is rarely seen unless there are co- existing chest or lung injuries. D-Disability-Neurological Examination This includes assessment of GCS, sensory, motor function and reflexes to identify objective signs of focal neurological deficit such as paraesthesia, weakness, or decreased/absent deep tendon reflexes. E-Exposure Log rolling is important. Careful inspection and palpation from the nuchal ridge to at least the first thoracic vertebral prominence should be performed. Look out for any signs of bruising, open wounds, tender points and posterior processes in search for distraction. WHICH PATIENTS TO BE SENT FOR IMAGING? National Emergency X Radiography Utilization Study (NEXUS) in 1997 and Canadian C Spine Rule can be used as a guide to patients who require imaging.
A â&#x20AC;&#x201C;Airway Protection and C Spine Stabilization. If there are indications for Oro- tracheal intubation this should be followed by rapid sequence induction (RSI), manual inline stabilization (MILS) and the use of gum elastic bougie or intubating laryngeal mask airway. Manual in Line Stabilization (MILS) (3) An assistant should kneel at the head end and held the head firmly down on the trolley in the neutral position with the hands on the mastoid processes. He should counteract any movements of the head during laryngoscopes. A second assistant should monitor to ensure that no movement of the head occurs.
NEXUS Low Risk Criteria. (5, 6, 8) 1.Posterior midline cervical tenderness 2.Evidence of intoxication 3.Decreased level of alertness 4.Focal neurological deficit 5. Painful distracting injuries
C Spine Stabilization A combination of a rigid cervical collar and supportive blocks on a back board with straps is very effective in limiting motion of the cervical spine and is recommended. B-Breathing and Ventilation High spinal injuries involving C1-C3 lead to complete paralysis of all respiratory muscles and respiratory arrest which needs immediate intubation and mechanical ventilation. Lesions at C3-C5 causes varied impairment of diaphragmatic contraction leading to respiratory distress. These patients may also need assistance to maintain airway and breathing.
YES
NO
Radiography
No Radiography
The presence of any one of the above features is considered to be clinical evidence that a patient is at increased risk of cervical spine injury and requires radiographic evaluation. Cervical spine injury cannot be excluded if any of the following criterions are present.
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Imaging Plain radiographs, CT scan and MRI are the most common imaging methods in the emergency department. Each has its own advantages and disadvantages. The clinical scenario must be considered when deciding which method or combination of methods to be used. Plain Radiography: This includes 3 views: ・Antero Posterior ・Lateral Odontoid (Open Mouth ) A swimmer's view is recommended if the tip of the T1 is not visualized. Plain radiography has higher false negative results than CT scan. CT scan: Allows easy imaging of the cervical spine when clinically indicated. It is the best for detecting bony abnormalities.
Log Rolling: This requires at least 4 people. The team leader stands at the head of the patient and coordinates the movements as well as maintains the patients head and neck alignment. Two or three other people roll the patient and one person may insert the back board or do the examination or perform patient's nursing care.
Respiratory Management: Respiratory complications remain the most common reason for mortality among spinal cord injured. Paralysis of respiratory muscles leads the patient toward respiratory failure. Respiratory complications include hypoventilation, hypercapnia, reduction in surfactant production, mucus plugging, atelectasis and pneumonia.
MRI: When ligamentous injury or spinal cord injury is suspected without radiographic abnormality. In summary, no single radiographic study can adequately rule out C spine injury in symptomatic patients. Three view plain C spine radiographs combined with CT through areas of difficult visualization and suspicion will detect majority of C spine injuries. FURTHER MANAGEMENT The treatment begins after the initial clinical evaluation and stabilization of life-threatening problems. All patients with radiographic evidence of unstable cervical fracture or dislocation (i.e. atlantooccipital dislocation, bilateral sub axial facet dislocation) and patients with either clinical or radiographic spinal cord injuries must be admitted to ICU. In the ICU, use of cardiac, haemodynamic and respiratory monitoring devices to detect cardiovascular and respiratory insufficiency is recommended. Immobilization: ・ The spine board should be removed as soon as possible, once the patient is on a firm trolley or bed. Prolonged use of back board can lead to pressure injuries. ・ In all these patients maintain spinal cord stabilization with rigid cervical collar and strict log roll precautions should be followed until temporary stabilization using halo traction or halo west is applied.
Early respiratory management includes,
・ Airway: Any intervention before the neck is stable or fixed require cervical in line stabilization and log rolling. ・Chest physiotherapy ・Mucolytics/ Broncoscopy ・Non invasive / Invasive ventilation Cardio Vascular Management:
・ Bradycardia may require atropine or a cardiac pace maker.
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・Blood pressure maintenance: Hypotension should not be attributed to neurogenic shock until blood loss or other trauma related causes have been managed or ruled out. The aim is to maintain mean arterial pressure 85-90 mmHg to minimize secondary injuries due to poor perfusion. These patients may need invasive blood pressure and central venous pressure measurements. Volume correction with crystalloid and blood is done before vasopressor support. Dopamine, nor epinephrine or epinephrine are preferred to provide inotropic and chronotropic support.
Steroid therapy (11,13,14) :
Conclusion:
The usage of high dose Methyl prednisolone is highly debatable. The National Spinal cord Injury Study (NASCIS) has conducted three trials based on these studies; the early use of high dose steroid has practically become the standard care in acute spinal cord injuries. However, the Congress of Neurological Surgeon (CNS) has stated that steroid therapy should only be undertaken with the knowledge that the evidence suggesting harmful side effects are more consistent than clinical benefits. The American College of Surgeons (ACS) has modified their advanced trauma life support (ATLS )to state that methyl prednisolone is “a recommended treatment” rather than “the recommended treatment “.
Spinal cord injuries are common occurrence in many trauma victims. The detection of spinal cord injury can be difficult . A high level of concern and diligence in clearing the spine is needed to avoid missing injuries. References: 1. Schmidt O.I, Gahr R.H., A. and Heyde C.E. (2009). ATLS and Damage Control in Spine Trauma. World Journal of Emergency Surgery, 4:\ 2.Denton M. and Mckinlay J. (2009).Cervical Cord Injury and Critical Care .Continuing Education in Anaesthesia, Critical Care and Pain, 9(3):82-86. 3.Attygalle D., Rodrigo N. and Abayadeera A. chapter 24.Hand Book of Anaesthesia. 4th ed. Collage of anaesthesiologists,Srilanaka.
The Canadian Association of Emergency Physicians (CAEP) is no longer recommending high dose methyl prednisolone as the standard care. Furthermore the updated guidelines issued in 2013 by the CNS and the American Association of Neurologic Surgeons (AANS) recommended against the use of steroid after an acute spinal cord injury. Methyl prednisolone is not recommended because no class1 and 11 evidence support its benefit. Class 1 and 111 evidence indicates a higher incidence of infection, sepsis, increased ICU length of stay and death with steroid use.
4.Tolletson E., Fodenes O., (2012) Respiratory Complications Associated with Spinal Cord Injury. Tidsskr Nor Laegefore., 15,1111-4. 5.Hoffmon J.R., Wolfson A.B., Todd K. and Mower W.R. (1998). Selective cervical spine radiography in blunt trauma: methodology of the National Emergency X-Radiography Utilization Study (NEXUS).Aannals of emergency medicine, 4,461-469 6.Hoffman J.R.,Schriger D.L., Mower w., Luo J.S. and Zucker M., (1992).Low Risk Criteria Cervical Spine Radiography in blunt Trauma: a prospective study. Aannals of emergency medicine, 21,1454-1460. 7.Steill I.G. ,Well G.A., Vandembeen K.L et al. (2001)The Canadian C Spine Rule for radiography in alert and stable trauma patients. JAMA, 286,1841-1848.
DVT Prophylaxis: Thrombo embolic disease is a common occurrence in patients who have sustained a cervical cord injury and is associated with significant morbidity. A combination of mechanical and pharmacological methods is recommended.
8.Stiell I.G., Clement C.M., Mc Knight R.D. et al.(2003).The Canadian C Spine Rule versus NEXUS low risk criteria in patients with trauma. N Eng J Med, 349,2508-2510 9.Panacek E.A., Mower W.R., Holmes J.F. and Hoffman J.R. (2001).Test Performance of the individual NEXUS low risk clinical screening criteria for cervical spine injury. Ann Emerg Med ,38;22-25.
Analgesia and Sedation: Surgical Management:
10.Ackland H.and Cameron p.(2012).Cervical spine assessment following trauma. Aust FAM Physician, 41( 4), 196-201
Early consultation with a Orthopedic or neurosurgeon is mandatory for a optimal management of C spine injuries. Early interventions such as close reduction, halo traction, open reduction or decompression of serious injuries with cord compression provide the best patient outcome.
11.HadleyM.N., Walters B.C., Grabb P.A.,(MD0,Oyesiku N.M.,Przybylski ,ResnickD.K.and RykenT.C.(2013).Guidelines for the Management of Acute Cervical Spine and Spinal 1. cord Injuries. American Association of Neurological Surgeons and the Congress of Neurological Surgeons. 12De Vivo M.J., Chen Yuing, Kruase J., Saunders L.L.(2012).Trends in age adjusted cause specific mortality rates after spinal cord injury.Top spinal cord inj Rehabil,18(1)214.
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Kurunegala Medical Journal, 2013 Articel
Common Oral Ulcerations Dr. Manori Jayasinghe Consultant in Restorative Dentistry, General Hospital, Kurunegala
Introduction Oral ulcers are common lesions encountered in dental practice. They can have a localized etiology or be a manifestation of variety of systemic conditions/disorders. Management depends on the correct diagnosis, but it can be difficult due to similar clinical features. Some of the common oral ulcers are discussed in this paper.
Diagnosis of traumatic ulcerations is usually with clinical features. Traumatic ulcers, especially acute ulcers, present with various degrees of pain and other signs of acute inflammation but chronic ulcers are usually painless. Patients are often aware of traumatic ulcers and they give a history of trauma or can reflect the episode of trauma when questioned. Clinical examination may indicate the cause for the ulceration, especially with chronic cases. Traumatic ulcers are usually single. Child abuse should also be remembered.
Traumatic ulceration Acute and chronic traumatic injury to oral mucosa is a common occurrence and this frequently results in ulceration. Mechanical trauma is the most common cause and it may be caused by dentures, accidents, when soft tissue gets trapped between teeth, or physiological trauma, induced iatrogenically by rotary instruments during dental treatment, removal of dried cotton rolls from mucosa or negative pressure from saliva ejector. Most common cause of ulcers due to chemical trauma is aspirin burns followed by other topical applications and some restorative materials. Thermal burns, as seen with hot food and beverages are often seen on the palate and tongue. Radiotherapy to head and neck and certain chemotherapy regimens can cause oral mucositis which can presents as multiple areas of painful erythema, ulceration and even epithelial sloughing.
Cause for the ulceration should be determined and, if applicable, is removed. Patient should be followed up for maximum of two weeks and if no clinical sign of improvement is present, an incisional biopsy must be performed to exclude more serious aetiology. This is very important as malignant ulcers can mimic a chronic traumatic ulceration or chronic trauma may be present on a malignant ulcer. Antiseptic mouth washes or anti-inflammatory washes are used as a treatment for oral mucositis in cancer patients. Recurrent aphthous stomatitis (RAS) RAS is one of most common conditions leading to recurrent oral ulceration. Exact etiology for the disease is unknown1 but several aetiological factors, including genetic predisposition, local trauma, hormonal changes, infective agents, gastrointestinal disorders, stress, immunological abnormalities and haematological deficiencies (vitamin B12, red cell folate, iron), have been proposed2,3. Majority of patients with RAS are healthy. Systemic conditions, including Behcet's syndrome, and chronic malabsorption conditions, such as Crohn's disease, ulcerative colitis and celiac disease, may be associated with RAS.
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RAS affects 15- 25% of healthy population. Males and females are equally affected with a peak in early adult life. Frequency of ulceration can vary and ulcers can arise every 4 -12 weeks. In some individuals it appears to be a continuous process as the interval between healing ulcers and new ulcers is minimal. Ulcers heal within 7-10 days in most cases without any complication. RAS may be classified as minor, major and herpetiform, affecting approximately 80%, 10% and 5 to 10% of the patients respectively.
Usually symptoms of oral carcinoma are disproportionate to the size of the lesion. Early lesions are usually painless and may appear as small, apparently harmless areas of induration, erosion or keratosis. Advanced oral cancer can present as a persistent painless ulcer with indurated, rolled margins. It may be fixed to underlying soft tissues and bone, and regional lymphadenopathy may be present. These are usually painless unless secondarily infected. In advanced cases, trismus or impaired tongue movement and persistent pain may be present. The buccal mucosa that includes all oral lining mucosa is the commonest area predisposed to squamous cell carcinoma (SCC) in patients in the Indian subcontinent region, Taiwan and East Asia but is very rare in developed countries. This pattern is due to the habits associated with different cultures for example, betel chewing in the Indian subcontinent whereas alcohol and smoking in developed countries. Squamous cell carcinoma of the buccal mucosa (SCC- BM) is usually asymptomatic but is often traumatized by daily functions leading to ulceration. Any suspiciouslooking oral mucosal lesion, including any ulcer not healing within 2 weeks after conservative treatment, must be biopsied to establish diagnosis. Treatment for oral cancer may vary from localized excision to extensive surgery with adjuvant chemo- and radiotherapy.
Diagnosis is based on history of recurrences of painful, self-healing ulcers at regular intervals on non-keratinized mucosa and biopsy is rarely indicated. Most important differential diagnosis is the ulceration associated with herpes simplex viral infection in immunocompromised individuals. Twenty percent (20%) of patients with RAS may have vitamin B12, folate or iron deficiency and more likely to be present in older patients whose ulceration starts or worsens in middle age. Correcting deficiency in these patients has been shown to bring rapid relief. Treatment is mostly symptomatic, aimed mainly at pain relief, reducing the frequency of ulceration and preventing secondary infection4. Choice of therapy depends on severity and frequency of ulceration. Topical and systemic ste ro i d s , a nt i s e p t i c m o u t hwa s h e s , a nt i inflammatory agents, analgesics, anesthetic agents and antibiotics have been used but success is limited in most.
Cutaneous disease Ulcers on the oral mucosa may be a manifestation of several skin diseases, most common being lichen planus, pemphigus vulgaris, erythema multiforme and mucous membrane pemphigoid. Oral Lichen Planus O (LP)
Oral Malignant ulcers Squamous cell carcinoma (SCC) of the oral cavity (Oral carcinoma) is the sixth most frequent cancer in the world, the first in the Indian subcontinent including Sri Lanka5 and common in elderly with the highest incidence in the fifth and sixth decades of life. Oral cancer is largely a preventable disease, as the primary aetiological agents are tobacco products, heavy use of alcohol, and combination of both. More than 90% of oral cancer patients give a history of tobacco use. Males are more affected. Most oral carcinoma occurs in one of four ways including (1) red patch (2) white patch (3) endophytic ulcerative lesion and (4) exophytic mass with rolled margins and central ulceration.
OLP is a chronic mucocutaneous inflammatory condition affecting stratified squamous epithelium. It is relatively common affecting about 1- 2% of the adult population6. Around 40% of the patients may present with OLP without skin involvement and some of them may proceed to skin manifestations. Although cutaneous lichen planus (LP) causes itching and is self-limiting, OLP is chronic and rarely undergoes spontaneous remissions and difficult to eliminate. It is a potentially malignant condition and causes considerable morbidity. Malignant transformation rate is less than 1%.
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OLP develops commonly in middle aged patients. OLP in children is well documented and when present it is not different from that in adults. Females are affected twice as much as males. Clinically OLP can present as a symptomatic disease or may present without any symptom. The most common clinical form is the reticular type giving its name lichen because of appearance of the lichen. Most of these reticular types are asymptomatic. Patient may present with symptomatic erosive, ulcerative or bulous types. It is not rare to see more than one clinical type in one patient. Commonest site to be involved is the buccal mucosa followed by tongue and gingiva. The characteristic clinical manifestations may be enough to make a diagnosis, if the classical features are present especially the skin lesions. An incisional biopsy is recommended to confirm the clinical diagnosis by histopathological means and to exclude the possibility of malignant transformation and dysplasia. Management protocol for OLP varies and the main aims must be to eliminate ulcerative and erosive lesions and by doing so, to reduce the symptoms and the malignant transformation potential. Asymptomatic reticular lesions do not need any treatment. All cases have to be followed up for a sufficient period of time to detect any malignant changes. The most commonly used agent for treatment of OLP is corticosteroids either local or systemic. Pemphigus vulgaris (PV)
Erythema multiforme (EM) EM is an inflammatory disease of immune origin affecting skin and mucosa. It arises due to immune complexes binding to vessel walls, with subsequent inflammatory process that leads to tissue destruction. Specific etiology in causing EM is unknown, although herpes simplex virus and certain drugs including NSAIDs, antiepileptic drugs may be involved. EM is characterized by the rapid onset of oral, cutaneous and ocular lesions. Oral lesions are seen in 30 to 60% of cases, and may be confined to the oral cavity. Oral lesions in EM are areas of painful erythema, bullae and ulcers, as well as areas of bleeding and crusting. Buccal mucosa, lips, soft palate and tongue are the most commonly involved sites. Clinical course of the disease is usually over 3-4 weeks but recurrences are common with usually at intervals of several months over a period of years. Progression of the disease can occur resulting more severe forms of EM (Steven Johnson Syndrome and toxic epidermal necrolysis), with extensive systemic involvement.
PV is an autoimmune, mucocutaneous, life threatening disease characterized by antigenic attack and destruction of desmosomal attachment of epithelial cells. This results in epithelial separation above basal cell layer, causing intra epithelial bullae which rupture soon with minor trauma leaving erosions and ulcerations. Failure to identify early oral lesion can lead to a delay in diagnosis of PV. All mucosal surfaces may be affected, but soft palate is the most commonly involved site, followed by buccal mucosa, tongue and gingiva causing desquamative gingivitis. Diagnosis should be confirmed with an incisional biopsy. Immunofluroscence studies are helpful. Before the introduction of steroids, most patients with PV died due to the disease but now, most of the deaths are due to the complication of steroids.
Mild cases of EM require only symptomatic treatment, which may include analgesics and antiinflammatory such as aspirin or non-steroidal antiinflammatory drugs. But care should be taken to check the history to ensure the EM was not precipitated by NSAIDs.
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Topical steroids are helpful in most cases7 but systemic steroids may be indicated in widespread involvement. Mucosal damage can be minimized by a bland liquid diet. Proper hydration and maintainance of oral hygiene is essential. Mucous membrane pemphigoid (MMP) MMP is an autoimmune disease, most commonly seen in middle-aged to older females. It is characterized by bullous eruptions and ulceration, particularly of oral mucosa and conjunctiva. 80% of affected patients have oral ulcerations. Desquamative gingivitis is also common. Treatment for MMP is similar to that of pemphigus vulgaris. Topical corticosteroids are indicated in most cases, especially for gingival lesions but for the severe cases, systemic corticosteroids have to be used. Maintaining oral hygiene to prevent secondary
References
infections is also important. Oral hygiene may be
1.Ship JA. Recurrent aphthous stomatitis. An update. Oral Surg
difficult for the patient, given the desquamative
Oral Med Oral Pathol Oral Radiol Endod 1996; 81:141–7.
gingivitis and mouth washes containing agents such as chlorhexidine are beneficial in those patients.
2.Scully C, Gorsky M, Lozada-Nur F. The diagnosis and management of recurrent aphthous stomatitis: a consensus approach. J Am Dent Assoc 2003; 134: 200–7.
3.Porter S, Scully C. Aphthous ulcers: recurrent. Clin Evid 2002: 1232–8.
4.Scully C & Felix DH. Oral Medicine-an update for the dental practitioner. Aphthous and other common ulcers. British Dental journal 2005;199(5):159-264
5.Guideline for the screening, surveillance, diagnosis and management of oral potentially malignant disorders. National cancer control programme. Ministry of Health Sri Lanka 2013
6.Axell T, Rundqvist L. Oral lichen planus - a demographic study. Community Dent Oral Epidemiol 1987;15:52-56.
7.Sawage NW & McCullough MJ. Topical corticosteroids in dental practice. Australian Dental Journal Medications Supplement 2005; 50:4.
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Kurunegala Medical Journal, 2013 Audit Reports
Effectiveness of minimal access treatment of varicose veins with foam sclerotherapy.An audit in a peripheral surgical centre Sirisean RCK, Manoagaran S, Abeyrathna PGPL,Tirichelvan B, General Hospital, Trincomalee, Sri Lanka
18 patient with SFI and SPI were admitted to surgical ward in GH-Trincomalee.12 patients had SFI.6 patients had SPI.Two patients had SFI withchronic venous ulcers more than 5 years.
Conclusion Success rate in treatment of varicose veins with foam sclerotherapyis 100% within first 3 months. This procedure is a good alternative for open SFL or SPL because of early discharge, less cost and early return to work.
All patients had USS guided Foam Sclerotherapy. All of them werereviewed after first and second weeks and planned to be reviewed after three months. All patients were discharged within one hour. Not a single patient developed anaphylactic reactions.2 patients complained pain during injection.1 patient developed thrombophlebitis one month after injection. Blood flow stopped immediately after foam injection (12 sec) confirmed by USS. All patients had sclerosed varicose veins during first visit. No DVT was found in either patient. Every patient was advised to walk 30 minutes after procedure and daily for about a week. All patients were advised to use cotton and crape bandage for two weeks. All patients were managed with Paracetamol and NSAIDS. All returned to work on the following day. Total cost is approximately. 1500 per session.
USS guided foam sclerotheraphy is a cost effective mode of treatment of varicose veins and venous ulcers. The number of patients in the sample is a limitation and therefore further ongoing study may be needed.
Results Rate of occlusion of veins was 100%. All patients discharged within one hour. One patient developed symptomatic thrombophlebitis which needed hospital admission. She was managed with antibiotics and NSAIDS only. Percentage of DVT was 0%. Rate of ulceration 0% Chronic Venous Ulcers in all patients started healing significantly four weeks after sclerosing the veins.
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Kurunegala Medical Journal, 2013 Case Reports
Effectiveness of minimal access treatment of appendicitis. An audit in a peripheral surgical centre Sirisean RCK, Abeyrathna PGPL, Tirichelvan B, B, Malwatta K, Thangavel N, Goonetilleke CU, Kekulthotuwa KDPD, Weerasena R. General Hospital, Trincomalee,Base Hospital Kuliyapitiya,Sri Lanka.
20 patients underwent laparoscopic appendicectomy within last 18 months in GH Trincomalee and Base Hospital Kuliyapitiya. 15 out of 20 were male patients. Every patient had 3 port laparoscopic appendicectomy. Open technique was used to enter the abdomen. Observations were made with regard to operation time, pain score, analgesic requirement and size of the scar, time duration for mobilization, date of discharge and return to work. Results All underwent laparoscopic procedure, conversion rate was zero. Average pain score was 4. Time taken for full mobilization was 6 hours after being sent to the ward. On the same day every patient was fed and no post op vomiting was observed. One patient had an early mass formation. One patient had retrocaecal appendix which needed mobilization of caecum. 19 out of 20 patients were discharged on the next day. One patient was kept for 3 days for antibiotics as he had pus in the pelvis and early mass formation. Conclusion Laparoscopic appendicectomy is an effective procedure for treatment of appendicitis even with minimum facilities. Zero conversion rate can be achieved in laparoscopic appendicectomy even with early mass formation.
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Kurunegala Medical Journal, 2013 Case Report
Scleredema but not Sceroderma D.M.Munasinghe Consultants Dermatologist – T.H. Kurunagala
Introduction Scleredema is an uncommon condition of unknown e t i o l o g y . S c l e re d e m a i s c h a ra c te r i ze d b y a nonpittng induration of the skin. It may be associated with a history of an antecedent febrile illness , diabetes mellitus or blood dyscrasia . Although regarded as a benign , self -limited skin disease scleredema may be persistent and involve the viscera . The term s c l e re d e m a i s a m i s n o m e r b e ca u s e n e i t h e r sclerosis nor oedema is found on microscopic examination. The histologic findings of sleredema include deposition of mucin between swollen dermal collagen bundles . The deposition is greatest in the deep dermis. The elastic fibers are normal.
figure 4( H & E shows separated collagen bundles in the dermis and subcuitis ) figure 3. A.S.O.T. - negative, E.C.G.- normal , C.X.R. – normal, E.S.R. – 21 , S.Creatinin , Calcium – normal , S. Protein Electrophorosis normal
Case report Grand mother has noticed tightness of skin of her nine year old school girl for last two weeks duration. She later on complained of pain while opening her mouth. No difficulty in breathing or swallowing. Bowel habits and micturation are normal. No loss of appetite or weight. Developmental history too was normal. She had upper respiratory tract infection two weeks ago. Other siblings were normal. She lives with her grandmother as her father and mother were employed abroad. She is not pale, afebrile and her skin is woody tight in face , abdomen , chest and upper limbs sparing the extremities. Nails and fingertips are normal. No depigmented macules on face or neck.No Raynaud's phenomena. Eye lids are difficult to retract )figure 1(. The skin is difficult to pickup or pinch together ) figure 2(.Skin histology reviled deposition of mucin between the collagen bundles in Alcian blue stain )
figure1
figure 2
figure 3
figure 4
Discution The exact etiology of scleredema is unknown. It is characterized by woody nonpitting induration of the skin. The initial skin changes of scleredema occurs on the face, neck and the upper part of the trunk. Patient may report difficulty in smiling, opening mouth and wrinkling their forehead. Hands and feet are typically spared. Sclereema patients with tongue involvement may report difficulty in tongue protrusion and dysarthria. Internal organ involvement is very rare. 38
Scleredema can be categorized in to three sub groups. G r o u p 1- I n c l u d e s s c l e r e d e m a a f t e r a c u t e respiratory tract infection )scleredema adultorum (. The onset of the skin lesion is rapid and the condition usually clears spontaneously in 6 months to 2 years . Most pediatric patients fall in to this group Group 2- Includes scleredema patients whose disease tends to occur insidiously, with no preceding illness . This group is associated with a monoclonal gammopathy. Group 3 â&#x20AC;&#x201C;scleredema associate with diabetes mellitus )scleredema diabetecorum (. Which is typically of adult onset and often type 1. The upper b a c k t y p i c a l l y d e m o n st ra te s e r y t h e m a a n d induration. There is no definitive treatment for scleredema . Systemic penicillin , systemic corticosteroids and PUVA therapy have been tried with variable success. Our child belongs to group 1 scleredema . Woody non pitting sclerosis occurred suddenly sparing the extremities following sore throat . No features to suggest scleroderma. Other possible associations were excluded and histology confirmed the clinical diagnosis. Short course of steroids and oral penicillin was given. Her skin tightness reduced remarkably by 2 months . Acknowledgements Dr. Jayantha Edirisooriya, Con. Pathologist , Dr. Nilanthi Senevirathne â&#x20AC;&#x201C;M.O.Dermatology Dr. H.M.P.S. Herath M.O. Dermatology , Dr. N. Athapattu M.O. Dermatology , Dr. H.P.C. Thilakasiri M.O.Dermatology , Dr. Indika Rathnayake M.O.Dermatology References 1.emedicine.medscape.com/article/1066175-overview 2. dermnetnz.org/immune/scleredema.html 3. dermis.net/dermisroot/en/39355/diagnose.htm 4. O.Braun -Falco , G.Plewig , H.N.Wolff , W.H.C.Burgdorf Dermatology 2nd completely revised edition
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Kurunegala Medical Journal, 2013 Case report
“A herbal plant in near fatal uremia” K.L.R. Kalupahana , W.M.P.G.R Dissanayake,H.M.A.N.Herath Medical unit B- Teaching Hospital Kurunegala
Abstract There are number of case reports of patients who developed complications after ingestion of Acalyphaindica(Sinhala: Kuppameniya) in the form of a food item or as an herbal medicine. We report a case of Acalyphaindica induced intravascular haemolysis and acute uremia who required blood transfusions and haemodialysis.
Laboratory investigations on admission Hb 6.9g/dl, WBC21x109/l (Neutrophils:70%,lympho: 27%, mono:3%) Platelet count 245x109/l Bilirubin,total: 143.9µmol/l Bilirubin, indirect 75.6µmol/l ALT 75 U/L, AST 175 U/L, ALP 103 U/L, Albumin 39.3g/ During the hospital stay further tests were done: Blood urea 234.6 mg/dl Creatinine 402µmol/l Viral markers for Hepatitis A,B and C were negative. Reticulocyte count: 49% Lactate dehydrogenase: 5988 u/l (NR 50-380), Direct and indirect Coomb's tests: negative Blood picture: Bacterial infection and Haemolysis
Introduction Glucose-6-phosphate dehydrogenase is an enzyme in the hexose monophosphate shunt which generates reductase potential that protect red cells from oxidative stresses. This enzyme deficiency is inherited as an X linked recessive manner. It is common in males but females develop the disease as a consequence of lionization, While G6PD deficiency is found throughout the world, it is most common in those of African or Mediterranean descent and is also found in those from Southeast Asia. Its geographical distribution corresponds with that of malaria.
The patient was managed in the ICU with intravenous fluids, antibiotics, several blood transfusions and other supportive care. His urine output declined and renal functions deteriorated requiring haemodialysis. Subsequently he made a good recovery. Discussion G6PD deficiency is an X-linked recessive disease and is the most common human innate error of metabolism, affecting more than 400 million people worldwide . At the Teaching Hospital Kurunegala , we have observed a high prevalence of G6PD deficiency in our patients; Because of wide availability of kuppamania ,more and our people are seek native treatment., however, there are no reports concerning the prevalence of this enzymatic deficiency in this country.
These individuals are at an increased risk of oxidative destruction of erythrocytes within the circulation, upon exposure to certain drugs and toxins as well as following acute infections and critical illness. The commonest toxin worldwide is fava beans but in Sri Lanka, kuppamania is implicated as the commonest agent. Case report A 24 years old male patient presented to the medical casualty unit with a two day history of passing cola coloured urine, yellowish discoloration of eyes and headache. He has a history of ingestion of leaves of kuppamaniya three days back, as a self-remedy for a chronic respiratory illness.On examination. He was febrile, confused, pale and jaundiced. He had right upper quadrant abdominal tenderness and mild splenomegaly. The patient deteriorated rapidly. 40
Here we present the first documented case of kuppamania induced haemolysis and renal failure in kurunegala in a G6PD deficient male. The patient presented with severe anemia (6.5 mg/dl)and haemoglobinuria. He was treated supportively with iv fluids, blood transfusion and haemodialysis. And he had a prolonged hospital stay but made a complete recovery. G6PD deficiency testing was positive confirming the diagnosis. Further we required to demonstrate the G6PD Mediterranean polymorphic variant genotype by Mutational analysis.
7. Mohanty D, Mukerjee MB, Colah RB. G6PD deficiency in India. Indian J Pediatrics 2004; 525-529. 8. Nakasuj T, Minsa S. Incidence and characteristics of G6PD variants in Japan. Hum Genet 1979; 51: 297. 9. Kirman HN, Hendrickson EM. Sex-linked electrophoretics difference in G6PD deficiency. Am J. Hum Genet 1963; 5: 241. 10. Martini G, Toniolo D, Vulliamy T, et al. Structural analysis of the x-linked gene encoding human G6PD.EMBOJ 1986; 5: 1849. 11. Beutler E. The molecular biology of enzymes of erythrocyte metabolism in the Molecular basis of blood disease, Stamatyannopoulos G, Nienhus AW, Majerus PW, et al, WB Saunders, Philadelphia 2003.
Conclusion G6PD deficiency is common in North western province at least partly reflecting malaria endemicity as well as close marriages. Kuppamania is either prescribed by native physicians or ingested by people in belief of its medicinal properties. As kuppamania induced haemolysis and renal failure is being reported increasingly, it is high time for the physicians to be aware of similar presentations as well as to educate the populations about the G6PD deficiency and the toxicity of kuppamania in such people.
12. Choudhary VP, Madan N, Sood K. Intravascular hemolysis and renal insufficiency in children with G6PD deficiency following antimalarial therapy.Ind J Med Res 1980; 71: 561-563. 13. Beutler E. G6PD deficiency. Blood 1994; 84: 3613.
1. Rose BD. Pathophysiology of Renal Diseases, 2nd ed, McGraw-Hill, New York, 1987, pp: 10-1. 2. Barn RB, Rowles E. Factors affecting coloration of urine and feces. J Am Pharm Assoc 1973;13: 139. 3. Watson WC, Luke RG, Inall JA. Beeturia: Its incidence and clue to its mechanism. Br Med J 1963; 2:971. 4. GladerBE. Glucose 6 phosphate dehydrogenase deficiency and related disorders of hexose monophosphate shunt and glutathione metabolism. In Wintrobe's Clinical Hematology, 10th edn. Lee GR, Forester J, Lukens J, et al. William and Wilkinis, Baltimore p: 1176-90. 5. Beutle E. Glucose 6 phosphate dehydrogenase deficiency. N Engl J Med 1991; 324: 169. 6. Chan TK, Todd D. Characteristics and distribution of G6PD variants in South China.Am J Hun Genet 1972; 24: 457. Gomal Journal of Medical Sciences Janâ&#x20AC;&#x201C;June, 2005, Vol. 3, No. 1 29
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Kurunegala Medical Journal, 2013 Case report
Mycetoma (Madura foot) Munasinghe CS, Perera MDS Senior Registrar in Radiology and Consultant Radiologist , Teaching hospital, Kurunegala
Abstract Mycetoma is an uncommon chronic infection of skin and subcutaneous tissues in tropical countries is caused by true fungi or by filamentous bacteria. We present a case of Madura foot with multiple discharging sinuses in the left foot and inguinal lymph nodes. Plain X- ray features of the left foot were suggestive of chronic osteomyelitis of fungal origin. CT of the foot showed multiple cystic spaces with cortical disruption and CECT of the left inguinal region revealed lymphadenopathy with dermal sinuses. Histopathologic diagnosis of fungal infection was achieved by biopsy of the left foot.
Fig.1 : Photograph of the left foot showing multiple cutaneous sinuses.
Fig 2: Photograph of the left inguinal region showing lymphadenopathy with sinus openings.
On examination, the left foot was swollen up to the ankle and skin was discolored with multiple cutaneuos sinus openings on the dorsal and medial side of ventral aspect of the left foot. Also soft tissue nodules in the left inguinal region with sinus openings. Laboratory examination revealed anaemia( Hb - 9g/dl) and blood picture suggested anaemia of chronic disease.
Introduction Madura foot or mycetoma is a chronic granulomatous disease characterized by localized infection of subcutaneous tissues by true fungi (eumycetoma) or by filamentous bacteria (actinomycetoma). The recurrence rate for the disease if treated inadequately is very high.
X- ray of the left foot showed soft tissue swelling, multiple lytic (cystic) lesions with peripheral sclerosis involving tarsal and proximal metatarsal bones and distal tibia and fibula.(Stage V). There was no true sequestra. The shaggy periostitis, reactive sclerosis and resorption of bone gave a melting snow appearance.
Case report A 22 year old girl presented with painful swelling and draining sinuses with periodic purulent discharge of the left foot since 15 years of age. 7 years back she had an accidental puncture to the sole of the left foot by a metal rod. At the time she had taken western and indigenous treatment and had been exposed to mud in bare foot. After a few weeks she developed left inguinal lymphadenopathy with discharging sinuses similar to that on the foot. Thereafter she had defaulted treatment.
Fig 3: X-ray oblique view of the left foot revealing melting snow appearance.
Fig 4 : X-ray left ankle revealing the involvement of the distal tibia and fibula.
Ultrasound scan of the foot revealed sinus tracts, heterogeneously echogenic soft tissues including tendons. The characteristic â&#x20AC;&#x153;dot-in-circleâ&#x20AC;? sign was not demonstrated. 42
Fig. 5: USS revealed the sinus tract and irregular bone cortex
Fig.7 : Saggital CT of the left foot
Contrast enhanced CT of the foot and left inguinal region were done. There was sclerosis with small hypodense lytic lesions (intra osseous cavities) involving the left tarsal bones, left proximal metatarsals, left distal tibia and fibula. Configuration of bones were disrupted. Cortical margins of the bones were irregular and ill defined. There was reduction of joint spaces involving ankle, intertarsal and tarsometatarsal joints. No sequestra. Adjacent soft tissues were heterogenous in density.
Fig 8 : CECT of the left inguinal region revealed enhancing enlarged lymph nodes.
No intra osseous or soft tissue focal collections to suggest abscess formation.
CECT of left inguinal region revealed inguinal lymphadenopathy with homogenous contrast enhancement, dermal sinuses and surrounding inflammatory changes. No bony involvement. The largest node measured 2 cm x 2.8 cm in size. Biopsy of the left foot was done at the initial presentation which showed fungal hyphae surrounded by suppuration and granulation tissue consistent with a fungal infection. Discussion Mycetoma is endemic in the tropics and subtropics, namely Africa, Mexico and India, and is named after the region of India where it was first described in 1842. However, it can also be found in natives of areas of Central and South America and the Middle or Far East between latitudes 15째S and 30째N. The incidence of mycetoma is likely to rise in temperate regions as well, due to increases in worldwide travel. It commonly presents between 20 to 50 years of age, with a male to female ratio of 2.2:1.
Fig 6 : Axial CT of the left foot revealed lytic lesions with cortical disruption.
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Mycetoma is an uncommon chronic infective disease of the skin and subcutaneous tissues characterized by the triad of tumefaction, draining sinuses and presence of colonial grains in the exudates. The infection in Madura foot results in a granulomatous inflammatory response in the deep dermis and subcutaneous tissue, which can extend to the underlying bone. More than 20 species of fungi and bacteria can cause mycetoma. The ratio of mycetoma cases caused by bacteria (actinomycetoma) to those caused by true fungi (eumycetoma) is 197:67.
Radiographic classification of bone involvement (stages 0â&#x20AC;&#x201C;6) has been suggested. Stage 0 - A granulomatous soft tissue reaction surrounds the entry site, causing a localized increase in the soft tissue volume that may progress to a soft tissue mass Stage 1 - These soft tissue swellings become nodular, hard and expansive. Initially, the bone is displaced, bowed or compressed from one or both sides. No bone invasion occur at this stage.
The most common site of occurrence is foot (approximately 70% cases) with infection of the dorsal aspect of the foot being typical. Hand is the next most common site.
Stage II - The next stage occurs when the organism irritates the bone surface before reaching the intraosseous compartment. This irritation causes one of two osteoblastic responses: a periosteal reaction or a diffuse reactive sclerosis.
Plain X-rays are used to assess for evidence of bone involvement. Czechowski et al. [10] found that radiography had a sensitivity of only 50 % in a series of 20 patients. Early or minimal soft-tissue reaction and minute cortical erosion can be missed totally by radiography because of their small size. [4] Radiographs may demonstrate soft tissue enlargement, bone sclerosis, bone cavities, periosteal reaction, bone expansion, extrinsic cortical scalloping, fanning of the rays or osteoporosis. The bones are almost always attacked from the outside, in contrast to bacterial osteomyelitis.
Stage IIl - Penetration of the periosteum and cortex leads to formation of bone cavities. Cavitation can be limited to a solitary bone. Stage IV - The infection may spread longitudinally along only one adjacent ray of metatarsal bone and phalanx. Stage V - Horizontal spread will involve more than one ray but is limited to one or two contiguous rows of small bones. Stage VI - When the infection is neglected or uncontrolled, it spreads in more than one direction and destroys most of the bones in the foot, leading to total disorganization and mutilation of the foot structure. A few radiographic bone changes have been described that help distinguish between actinomycetoma and eumycetoma. Eumycotic lesions tend to form a few cavities in bone that are â&#x2030;Ľ 1 cm in diameter, while actinomycetes often form smaller but more numerous cavities, leading to a moth-eaten appearance.
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CT scan provides better delineation of the bone changes than radiographs and is the most sensitive diagnostic informative tool with conventional radiography, which is the method of choice for the pretherapy assessment of pedal mycetoma. MRI has a limited role in finding bony (cortical) changes, because of the absence of a signal caused by lack of movable protons. Coarse trabecular pattern, frank bone destruction, marrow infiltration and sequestra that are readily detected by MRI are late manifestations of the disease. Conclusion If it is not diagnosed early on, mycetoma can cause functional and esthetical impairments. Therefore, early detection of this is disease is necessary if major surgery is to be avoided. Recent literature suggest that all mycetomas may be amenable to medical treatment , particularly since the introduction of new azole-derivatives. A prolonged follow-up period is necessary to monitor for disease recurrence.
References 1.Lichon V, Khachemoune A; Mycetoma : a review. Am J Clin Dermatol. 2006;7(5):315-21. 2.Ania BJ et al; Mycetoma, eMedicine, Aug 2008 3.Fahal AH; Mycetoma: a thorn in the flesh. Trans R Soc Trop Med Hyg. 2004 Jan;98(1):3-11. 4.Abd El Bagi ME, Sammak B,Al Shahed M, et al.Rare bone infections “excluding the spine”. Eur Radiol 1999:9: 1078-1087 [CrossRef] [Medline] 5.Mahgoub ES, Medical management of mycetoma. WHO Bulletin 1976; 54:303-329 [Medline] 6.Yera H, Bougnoux ME, Jeanrot C, et al; Mycetoma of the foot caused by Fusarium solani: identification of the etiologic agent by DNA sequencing. J Clin Microbiol. 2003 Apr;41(4):1805-8. 7.Corr P; Clinics in diagnostic imaging (26). Madura foot (or mycetoma). Singapore Med J. 1997 Jun;38(6):268-9. 8.Ahmed AA, van de Sande WW, Fahal A, et al; Management of mycetoma: major challenge in tropical mycoses with limited international recognition. Curr Opin Infect Dis. 2007 Apr;20(2):146-51. 9.Barnetson R, Milne LT. Mycetoma (review) Br J Dermatol. 1978;99:227–30.[PubMed] 10.Czechowski J, Nork M, Haas D, Lestrinhant G, Ekelund L. MR and other imaging methods in the investigation of mycetoma. Acta Radiol 2001 ; 42:24-26 [CrossRef] [Medline]
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Kurunegala Medical Journal, 2013 Case report
Paget's disease involving the entire breast with underlying ductal malignancy- A rare presentation of breast cancer Bandara I.M.H.W1, Somathilaka S.P.D.U1, Gajanthan K1 Udayakumara E.A.D1 1.Surgical Unit-A, Teaching Hospital Kurunegala Abstract Paget's disease of the breast is an uncommon form of breast cancer. It comprises 1-4% of all breast malignancies. It is characterize by infiltration of the nipple epidermis by malignant cells leading to chronic eczematous eruptions on the nipple areola complex. The diagnosis remains elusive with varied presentations, mimicking many benign skin diseases, the awareness of which is necessary for the diagnosis and minimizing morbidity. We report a case of Paget's disease of the nipple, with underlying ductal carcinoma insitu.
On examination, she had an erythematous plaque covering the nipple-areola complex of the left breast, (figure 01). There were areas of ulcerations on the surface of the plaque covered with purulent to hemorrhagic crusts. The nipple-areola complex was completely destroyed. A single mobile lymph node measuring 1 cm Ă&#x2014; 2 cm was palpable in the left axillary region and there was no palpable breast mass, nor any nipple discharge on expression. The right breast was normal both on inspection as well as on palpation. A fine needle aspiration of left breast showed largely ulcerated epidermis. Preserved areas showed large atypical round to oval cells infiltrating the lower part of epidermis (figure 2). The patient underwent total mastectomy with level IIaxillary node clearance. The surgical specimen confirmed Paget's disease limited to the epidermis with ductal carcinoma insitu involvement of underlying breast tissue. Patient referred to oncologist for further management.She was asymptomatic during 6 months of follow up Discussion Management of Paget's disease of breast is depend on the whether it is confined to the skin or associated underline breast tissue involvement. As regards to the therapy, simple mastectomy with or without axillary lymph node dissection has been the historic standard treatment for Paget's disease of the breast if it is confine to the skin.[1],[6] In the recent times, breast conserving surgery followed by whole breast radiotherapy has been increasingly recommended in patients where the disease is limited and there is no ev i d e n c e o f m a l i g n a n c y o n ra d i o l o g i ca l examination.[7]The standard treatment in the presence of underline breast tissue involvement is mastectomy with axillary clearance. In our case, we performed a total mastectomy with level 11 axillary clearance as suggest by consultant oncologist. In conclusion any chronic eczematous lesion over the breast should be evaluated thoroughly both clinically as well as histologically to rule out malignancy.
Introduction Paget's disease is a type of breast cancer that occurs in the ducts adjacent to the nipple-areola complex and spreads to the skin. It accounts for less than 2 percent of all breast cancer cases, predominantly in women but less commonly in men. Symptoms can be similar to eczema. Paget's disease is usually associated with ductal carcinoma in situ (DCIS), limited to the nipple- areola complex of the breast. There were very few reports which described the disease has occurred independent of any underlying malignancy.[1]In this condition, infiltration of the nipple epidermis by malignant cells causes chronic eczematous eruptions on the nipple-areola complex.[2],[4],[5]We report a case of Paget's disease of the nipple, with underlying ductal carcinoma insitu. Case presentation A 52-year-old female presented to the surgical casualty with a complaint of reddish itchy, oozylesion on her left breast for 3 months duration. The lesion started from the nipple and then gradually progressed. There was no history of nipple discharge, any palpable mass or any swelling in the axillary region. Her family history was unremarkable.
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Reference 1.Meibodi NT, Ghoyunlu VM, Javidi Z, Nahidi Y. Clinicopathologic evaluation of mammary Paget's disease. Indian J Dermatol 2008;53:21-3. 2.Lloyd J, Flanagan AM. Mammary and extramammary Paget's disease. J ClinPathol 2000;53:742-9. 3.Morrogh M, Morris EA, Liberman L, Van Zee K, Cody HS 3 rd , King TA. MRI identifies otherwise occult disease in select patients with Paget disease of the nipple. J Am CollSurg 2008;206:316-21.
Figure 01- An ulcerated erythematous plaque covering whole of the left breast, the sub mammary covered with purulent to hemorrhagic crusts
4.Lim HS, Jeong SJ, Lee JS, Park MH, Kim JW, Shin SS, et al. Paget disease of the breast: Mammographic, US, and MR imaging findings with pathologic correlation. Radiographics 2011;31:1973-87. 5.Jones RE Jr. Mammary Paget's disease without underlying carcinoma. Am J Dermatopathol1985;7:361-5. 6.Mori O, Hachisuka H, Nakano S, Maeyama Y, Sasai Y. A case of mammary Paget's disease without an underlying carcinoma: Microscopic analysis of the DNA content in Paget cells. J Dermatol1994;21:160
Figure 02-Histopathological section from the plaque showing large atypical round to oval cells (arrow) infiltrating the lower part of epidermis having a pale cytoplasm with prominent hyper chromatic nuclei (H and E, Ă&#x2014;400)
7.Campana F, Vielh P, Fourquet A, Schlienger P, Jullien D, Durand JC, et al. Paget's disease of the nipple without any associated mammary tumor clinically or radiologically detectable. Apropos of 51 c a s e s t re a t e d a t t h e C u r i e I n s t i t u t e . J GynecolObstetBiolReprod (Paris) 1987;16:1069-73.
The patient underwent total mastectomy with level IIaxillary node clearance. The surgical specimen confirmed Paget's disease limited to the epidermis with ductal carcinoma insitu involvement of underlying breast tissue. Patient referred to oncologist for further management.She was asymptomatic during 6 months of follow up
8.Sakorafas GH, Blanchard K, Sarr MG, Farley DR. P a g e t ' s d i s e a s e o f t h e b re a s t . C a n c e r TreatRev2001;27:9-18. 9.Singla V, Virmani V, Nahar U, Singh G, Khandelwal NK. Paget's disease of breast masquerading as chronic benign eczema. Indian J Cancer 2009;46:344-7. 10.Nicoletti G, Scevola S, Ruggiero R, Coghi AM, Toussoun GS. Gigantic Paget disease of the breast.Breast2004;13:425-7.
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Kurunegala Medical Journal, 2013 Case report
Congenital pseudarthrosis of radius D. Tennakoon1 , D. Dissanayaka2 1.Consultant Orthopaedic Surgeon 2.Senior House OfficersOrthopaedic Unit A Teaching Hospital Kurunegala
Abstract 3 years old boy with congenital pseudarthrosis of the radius was referred to us from DGH Kegalle for further managment. He had a history of fractures of both bones of the left forearm after trivial trauma one and half years ago in 2011. He was treated conservatively by POP and was reported to DGH Kegalle in September 2013 complaining of a gradually increasing deformity of the left forearm.
Introduction Congenital pseudoarthrosis of the radius is an extremely rare condition. It is usually associated with an autosomal dominant disorder, neurofibromatosis type 1 (von Recklin- ghausen's disease). Mutation of the NF1 gene on chromosome 17 causes von Recklinghausen's disease. The NF1 gene is a tumour s u p p res s o r gen e; it en co d es a p ro tein , neurofibromin, which modulates signal transduction through the ras GTPase pathway.
Radiologically, it was an apex anteromedial deformity of the distal aspect of the left radius. Clinically he had multiple cafĂŠ au lait spots over back of his body. Neurological and ophthalmological examinations were normal. The fibrous tissue and the fracture ends were excised with a cuff of diseased periosteum. The fracture was stabilized with a 6-hole Plate with bone graft. Congenital pseudarthrosis of the radius is an extremely rare condition.There are several treatment options including Dual onlay bone graft and vascularised fibular graft. Currently vascularised fibular graft is being the preferred one. But the disease being extremely rare not much has been documented about the treatment options. We treated this case by excision of the sclerotic bone ends along with a cuff of periosteum and internal fixation with DCP to restore the length and alignment.
In all cases reported, pseudarthrosis of the radius occurred in the distal third. Numerous treatment options have been explored with varying degrees of success and the reports have demonstrated successful healing. Case report A 3-years-old boy was brought to our centre by his parents with a complaint of gradually progressive apex anteromedial deformity of the left forearm for the last one and half years. He was a full term baby born by normal delivery with normal developmental milestones. He had sustained a trivial trauma at the age of 1 1/2 years following which he developed pain and swelling in his left forearm.(Fig 1)
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He was treated conservatively in a POP cast at a Base Hospital. (Fig 2)
Fig 6
Under general anaesthesia surgery was performed using the anterior approach. Fibrous tissue between the fracture ends was seen. The tips of the fracture ends were sclerosed and tapered with thickened periosteum. The fracture ends were excised till bleeding normal bone was seen. Hamatomatous thickened periosteal sleeve was completely excised proximal and distal to the fracture site.(Fig 7 )
After being treated in a POP cast for 4 weeks he developed a deformity which had progressively ultimately culminated in an apex anterioromedial deformity of the left radius over one and half years when the patient reported to us on September 2013. On detailed clinical examination we found he had multiple cafĂŠ au lait spots over his back. There was tenderness and mobility at the fracture site with shortening, apex ulnar deformity of the left forearm and radially deviated hand with restriction of pronation. (Fig 4 & 5) There was no family history of neurofibromatosis but all siblings are having cafĂŠ au lait spots over their back. X-rays of the lower limbs are normal. We referred the patient to an ophthalmologist for further investigation, and the ophthalmologic examination was normal.
Fig 7
Radius was realigned after a second osteotomy made proximal to the fracture and stabilised using a six-hole DCP and screws.(Fig.8 & 9)
Fig 8 Fig 4
Fig 9
Fig 5
Post-operatively fixation was protected with POP cast.(Fig 10)
On radiographs a cystic lesion was seen at the distal end of left radius with apex anteromedial deformity. At this stage Ulna fracture was completely healed indicating only the radius involvement.( Fig 6)
Fig 10
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Discussion Congenital pseudarthrosis of the forearm is a rare condition. About 60 cases have been reported in the literature [1]. The ulna is more commonly involved than the radius. Only 10 cases of congenital pseudarthrosis of the radius seem to have been documented [2]. Boyd and Sage [3] suggested dual onlay bone grafting as the treatment. Ka- meyama and Ogawa [4] reported good results after com- plete resection of the involved radius with the surrounding periosteum and free vascularised fibular graft.
References 1.
2.
More recently vascularised fibular grafting has become the preferred treatment [1, 5–7]. Other procedures like external electrical stimulation of the forearm and reversal of the bone segment to place the bone adjacent to the pseudarthrosis in contact with the normal bone have failed to produce union [5]. Cleveland et al [8] treated 4 cases of congenital pseudarthrosis of radius with corticocancellous bone graft- ing and achieved union in 3 cases. In conclusion, we report a case of congenital pseudarthrosis of the radius treated by excision of the sclerotic bone ends with a cuff of diseased periosteum and internal fixation with DCP.
3.
4.
5.
6.
7.
8.
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Witoonchart K, Uerpairojkit C, Leechavengvongs S, Thu- vasethakul P (1999) Congenital pseudarthrosis of the forearm treated by free vascularised fibular graft: a report of three cases and a review of the literature. J Hand Surg Am 24:1045–1055 Talab YA (1993) Congenital pseudarthrosis of the radius. A case report and the review of the literature. Clin Orthop Relat Res 291:246–250 Boyd HB, Sage FP (1958) Congenital pseudarthrosis of the tibia. J Bone Joint Surg Am 40:1245 Kameyama O, Ogawa R (1990) Pseudarthrosis of the radius associated with neurofibromatosis: report of a case and review of the literature. J Pediatr Orthop 10:128 Allieu Y, Meyer ZU, Reckendorf G et al (1999) Congenital pseu- darthrosis of both bones: long-term results of two cases managed by free vascularised fibular graft. J Hand Surg Am 24:604–608 Gregg PJ, Price BA, Ellis HA, Stevens J (1982) Pseudarthro- sis of the radius associated with neurofibromatosis. A case report. Clin Orthop Relat Res 171:175–179 Lee KS, Lee SH, Ha KH, Lee SJ (2000) Congenital pseudar- throsis of ulna treated by free vascularised fibular graft – a case report. Hand Surg 5:61–67 Cleveland RH, Gilsanz V, Wilkinson RH (1978) Congenital pseudarthrosis of the radius. Am J Roentgenol 130:955–957
Kurunegala Medical Journal, 2013 Case report
Incarcerated massive parambilical hernia in a morbidly super-super-obese patient
Bandara I.M.H.W1, Subasinghe D1, Gajanthan K1,Udayakumara E.A.D1 Abstract Abdominal wall hernias are more prevalent in the morbidly obese. Incarceration of external hernias is a relatively common process in adults and is associated to a high rate of complications and mortality. We present the case of a morbidly supersuper-obese patient (body mass index, 66.6 kg/m2) who underwent emergency surgery for an incarcerated paraumbilical hernia with segmental intestinal resection and mesh hernia repair. Introduction Incidence of paraumbilical hernias is estimated at approximately 2.3% of all hernias . It is estimated that they account for approximately 5-10% of all hernias in adults. Most of them occur in women aged over 50 years . It is also known as a herniated belly button a protrusion of the abdominal contents through a defect of the muscles surrounding the belly button. In adults generally it occurs more commonly above the belly-button when the abdominal wall is stretched because of obesity, pregnancy and other uncommon conditions. In patients with this condition, the muscles of the abdominal wall are weak or separated, allowing the abdominal lining to push through. In a smaller paraumbilical hernia, the protrusion may include only some fluid and tissue. Larger hernias can include sections of the intestine, raising the risk of strangulation, where the tissue becomes twisted with compromising the blood supply. Here we describe a female with morbid obesity with large paraumbilical hernia, who underwent surgery with herniotomy,intestinal resection and abdominoplasty successfully. Case presentation
Figures.1
Figures.2
Figures.3
A 60-year-old morbidly obese female presented to our surgical unit with a complaint of massive paraumbilical hernia of four years duration. She didn't have feature of intestinal obstruction such as vomiting, abdominal pain. Her bowel habits were normal. She had no past history of bowel obstruction.
Figures.4
Figures 01,02-Showing lax abdomen with huge paraumbilical hernia 03,04-Operative figure showing paraumbilical herniotomy and mesh repair with abdominoplasty
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On past medical history she was a diagnosed patient with diabetes mellitus and hypertension for 6year. Her glycaemic control was satisfactory on admission with mixtard insulin and blood pressure under controlled. Initially she was given dietary advices to reduce the weight but was not satisfactory. On examination she was morbidly obese (weight 101 kg,BMI=66.6kg/m2).On abdominal examination she had lax abdomen with huge paraumbilical hernia.(Figure 01)There was no tenderness on p a l p at i o n . S u bs e q u e nt l y s h e u n d e r we nt preoperative investigation to assess the fitness for surgery. Her 2-DECHO and lung function test were normal. On USS abdomen there were no Intraabdominal lumps. She underwent paraumbilicalherniotomy. Hernial sac contained small intestines and omentum. During surgery part of unhealthy ileum was resected and end to end anastomosis was done. The defect of the rectus sheath was repaired and on mesh repair was done. She also underwent abdominoplasty (Figure 02). During initial post-operative period she was given intensive care and recovery was uneventful . Discussion Paraumbilical hernia is one of the commonest surgical conditions affecting especially the female population. Surgical repair is the mainstay of treatment.As with any other surgical procedure, this is associated with possible complications. These include, superficial wound haematoma, superficial wound infection, serous effusion, post op respiratory distress, abdominal compartment syndrome andrerecurrence of hernia, Most of these complications are of mild to moderate degree and can be usually treated by a conservative approach. Recent advances in different surgical techniques and equipment claimed to have less complications but none are completely devoid of them. The case which was discussed here is a massive, long standing, complicatedparaumbilical hernia in a morbidly super obese patient with added medical co morbidities. In conclusion we achieved a good outcome asthis surgery was performed by an experience hand in a tertiary care hospital.
Reference 1. Russel RCG, Williams NS, Bulstrode CJK, (eds.): Bailey & Love's Short Practise of Surgery. 25th edition.Hodder Arnold; 2008. 2. Sugerman H, Windsor A, Bessos M, Wolfe L: Intraabdominal pressure, saggital abdominal diameter and obesity comorbidity.J Intern Med 1997, 241(1):713. McKay A, Dixon E, Bathe O, Sutherland F: Umbilical hernia repair in the presence of cirrhosis and ascites: results of a survey and review of the literature. Hernia 2009, 13(5):461-468. 4.Belghiti J, Durand F: Abdominal wall hernias in the setting of obesity Semin Liver Dis 1997, 17(3):2195. Chao A, Chao A, Yen YS, Huang CH: Abdominal compartment syndrome secondary to ovarian mucinous cystadenoma. ObstetGynecol 2004, 104(5 Pt 2):1180-
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Kurunegala Medical Journal, 2013 Case report
Is Right Hemicolectomy too much for Caecal Leak After Certain Appendicectomy Rajapaksha D.C1, Wickramarathna S. J.D1 1Teaching Hospital Kurunegala, General Surgical unit
Background Acute appendicitis is the most common reason for intervention in acute abdominal pain .diagnosis is made based on full clinical history and examination as well as supported by routine blood and urine investigations. It is common condition can be difficult in making a diagnosis when the clinical picture Is borderline suggestive of acute appendicitis .specially in children acute meckel's diverticulitis must be kept in mind as a clinical picture is indistinguishable from acute appendicitis. Perforation of large bowel Is associated with severe acute appendicitis but further surgical management of the condition uncommonly described. Case report An eleven year old boy presented with a right lower abdominal pain associated with nausea fever and anorexia .he was diagnosed as appendicitis and Appendicectomy performed at peripheral base hospital. After two weeks child developed features of bowel obstruction localized tenderness over the right lower abdomen without discharge at Appendicectomy site. He was consulted at teaching hospital Kurunegala later. On physical examination his temperature was 39 Celsius, pulse 110 per minute abdomen was distended and right lower abdominal tenderness with some guarding White cell count 18000, Hb-12g/dl, platlet-330000, Na-140, K-3.6, UFR-negative. Plain abdominal X-ray showed distended small bowel and chest X-ray was not remarkable. Post Appendicectomy small bowel obstruction was made clinically and after resuscitation the patient was consented for exploration under general anesthesia.
Operation Intravenous antibiotics were commenced pre operatively. Lower midline incision was made and approximately 100ml of purulent fluid found in the peritoneal cavity around the caecum .terminal ileum was kinked with adhesions and there was a 2 * 2 cm caecal perforation seen at the base of the appendix. Perforation of the caecum was repaired with an absorbel suture and omental patch was used to cover the caecum thorough washout was made with warm saline .A closed passive drain was placed around the caecum and abdominal incision was closed by mass manner by using nylon and skin also with nylon. Postoperatively inflammatory markers were responding with intra venous antibiotics .no further evidence of obstruction or spiking temperature was noted. The drain was removed postoperatively day5 and patient was discharged at 7TH day. Discussion Appendicular perforation commonly occurs at the tip of the appendix is associated with presence of a feacolith .perforation of the caecum is uncommon differential diagnosis for an acute appendicitis. Other possible causes of caecum perforations include perforated right diverticulitis; caecal tumor and rarely foreign bodies. Surgeries for colonic perforations are associated with high morbidity and motility rates. While primary repair with or without omental patch is a common surgical approach to manage stomach and duodenal perforations. There are only few reports in the literature that compare two different surgical approaches primarily repair vs. right hemicolectomy.In the presence of uncomplicated perforations, absences of severe infections and well controlled localized heamostasis ,less invasive surgical approach with post operative intravenous antibiotics would be the management of choice. 53 41
Right hemicolectomy carries higher mortality and morbidity but it is generally recommended only in selected cases as severe inflammation, torsion, hemorrhage, inflammatory mass, caecal neoplasms found intraoperatively.The presence of severe appendicitis with caecum appear necrotic in some cases warrant right hemicolectomy to be performed. The most frequent operation for perforated caecum is right hemicolectomy although some surgeons might advocate over sewn the perforation is equally adequate in repairing the defect. The advantage of the latter is associated with shorter length of hospital stay less blood loss easier heamostasis low risk of anastamosis breakdown. However there is no clinical data yet to support the hypothesis. Conclusion A primary hemicolectomy in perforated lesion of the caecum is recommended but there have been no recent studies comparing the approach with primary caecal repair with or without omental patch. A large prospective study is needed to compare both approach and long term outcome.
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Kurunegala Medical Journal, 2013 Report
Hepatocellular carcinoma: Experience in a general surgical unit at KGTH Rajapaksha D.C 1 Bogamuwa M.M.M.P.2 1 Consultant General Surgeon Kurunegala Teaching hospital 2 Intern Medical officer Kurunegala Teaching hospital
Introduction Hepatocellular carcinoma (HCC) is a primary malignancy of the liver. The pathophysiology of HCC has not been definitively elucidated and is clearly a multifactorial event. However they are typically associated with hepatitis b in the west and hepatitis C in east and also chronic alcohol intake. Hepatitis viruses lead to the development of chronic liver disease and subsequent development of HCC. But in a Sri lnakan context the causes for HCC are not clear. There are two types of HCC's: HCC in a cirrhotic liver and Non Cirrhotic Hepatomas(NCH) later being rare. The incidence of HCC increases with age, peaking at 70-75 years; however, an increasing number of young patients have been affected, as the demographic shifts from primarily alcoholic liver disease to viral hepatitis. Methods The study population included the patients who were referred to the surgical unit B of Kurunegala Teaching hospital from 07/01 to 11/01 .All data were collected prospectively. Results Total of 5 patients were referred over a period of 4 months. Median age at presentation was 52 Yrs (range 48-72Yrs) Among them most were males (80%). The initial presentation of 60% of patients was abdominal pain. Imaging showed that 4/5(80.0%) of patients had HCC's in background cirrhotic livers. 3/5 (60%) patients were heavy alcohol intakes. Median Alpha FetoProtein levels (AFP) was 91.57ng/ml (range 1.8 â&#x20AC;&#x201C; 395.90). Conclusion In our study population there was a male preponderance. Most Hepatocellular carcinomas occurred in a cirrhotic liver. At diagnosis patients were in 4th-7th decades of their lives. Commonest presentation of HCC's was abdominal pain. The AFP levels at presentation were not very high. And there was an association with alcohol intake. 41 55