Spectrum of Hepatic Presentation of Wilson’s Disease in Children Attending A Tertiary Care Centre of Dhaka City

Abstract

Background: The incidence of Wilsons disease (WD) is increasing day by day in every ethnic group worldwide. WD has been found as a common cause of chronic liver disease in children. This study was undertaken to find out the occurrence and different types of hepatic presentation of Wilsons disease in children admitted with liver diseases at a tertiary care centre of Bangladesh.

Methodology: This cross sectional descriptive study was carried out at the department of Paediatric Gastroenterology and Nutrition, BSMMU during the period from March 2008 through April 2010. A total number of 71 children of both sexes aged 3-15 years, who had the features of liver disease (jaundice with or without hepatomegaly / splenomegaly and / or raised serum ALT), were enrolled in this study. For the purpose of the study, the diagnosis of WD was made by the presence of any 2 of the 3 features: presence of K-F ring by slit lamp examination, low serum ceruloplasmin level (<20 mg/dL) and urinary copper excretion of >1600 ?gm /24 hours after penicillamine challenge.

Results: Wilsons disease was found in 31 (43.7%) of 71 children. Among them chronic liver diseases were 18 (58%), acute hepatitis 6 (19.4%), acute liver failure 6 (19.4%) and asymptomatic WD case was 1 (3.2%). The mean age ±SD of WD cases at presentation was 9.87±2.6 years and 22 (70%) cases were male. Maximum numbers of WD cases were found in children below 10 year of age. The two common presenting features of WD cases were jaundice 28 (90.3%) and ascitis (58.1%). Other features were K-F ring, 25 (80.6%) and hepatomegaly, 24 (77.4%). Biochemical findings showed low serum ceruloplasmin level (done in 20 patients) in 20 cases and 24 hours urinary copper excretion of >1600 mg/day in 23 cases. About one third of children presented with liver diseases were diagnosed as Wilsons disease and about 50% of WD cases presented with chronic liver diseases.

Conclusion: Wilsons disease is a common cause of chronic liver disease. Penicillamine challenge is a reliable diagnostic test for Wilsons disease.

DOI: http://dx.doi.org/10.3329/bjch.v38i2.21142

Bangladesh J Child Health 2014; VOL 38 (2) : 79-85