Wilson's Disease in Bangladeshi Children: Analysis of 100 Cases.
10.5223/pghn.2015.18.2.121
- Author:
Md RUKUNUZZAMAN
1
Author Information
1. Department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. dr.rukon@gmail.com
- Publication Type:Original Article
- Keywords:
Bangladesh;
Child;
Wilson's disease
- MeSH:
Bangladesh;
Ceruloplasmin;
Child*;
Christianity;
Copper;
Female;
Hepatitis;
Hepatolenticular Degeneration*;
Humans;
Hypertension, Portal;
Islam;
Jaundice;
Liver Diseases;
Liver Failure, Acute;
Male;
Neurologic Manifestations;
Penicillamine;
Prothrombin Time
- From:Pediatric Gastroenterology, Hepatology & Nutrition
2015;18(2):121-127
- CountryRepublic of Korea
- Language:English
-
Abstract:
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 microg/day was found in 81% cases and urinary copper following penicillamine challenge of >1,200 microg/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.
