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KMID : 1038820150180020121
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Pediatric Gastroenterology, Hepatology & Nutrition
2015 Volume.18 No. 2 p.121 ~ p.127
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Wilson’s Disease in Bangladeshi Children: Analysis of 100 Cases
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Md. Rukunuzzaman
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Abstract
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Purpose: To evaluate clinical and laboratory profile of Wilson’s disease (WD) in children.
Methods: This cross sectional study was conducted at Bangabandhu Sheikh Mujib Medical University Hospital. Bangladesh, over a period of 3 years. One hundred consecutive children of WD between 3 to 18 years of age were evaluated.
Results: Mean age was 8.5±1.5 years. Male female ratio was 2:1. Ninety-one percent of patients were Muslim and 9% Hindu. A total of 53% cases of hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Sixty-nine children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic and neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of CLD were found in 18% patients. Keiser-Fleischser ring was found in 76% total patients. Elevated serum transaminase was found in 85% cases, prolonged prothrombin time in 59% cases and hypoalbuminaemia in 53% cases. A total of 73% patients had low serum ceruloplasmin, basal urinary copper of ＞100 μg/day was found in 81% cases and urinary copper following penicillamine challenge of ＞1,200 μg/day was found in 92% cases.
Conclusion: Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.
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KEYWORD
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Bangladesh, Child, Wilson’s disease
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