CLINICAL PROFILE OF WILSON'S DISEASE IN CHILDREN IN A TERTIARY CARE CENTRE
V. Radhika*, M. Anupama**, Hareesh***, S Geetha****, D. Nirmala*****, B. Bhaskar Raju******
Dept of Pediatric Gastroenterology, Institute of Child Health & Hospital for Children, Egmore, Chennai. *, Dept of Pediatric Gastroenterology, Institute of Child Health & Hospital for Children, Egmore, Chennai. **, Dept of Pediatric Gastroenterology, Institute of Child Health & Hospital for Children, Egmore, Chennai. ***, Dept of Pediatric Gastroenterology, Institute of Child Health & Hospital for Children, Egmore, Chennai. ****, Dept of Pediatric Gastroenterology, Institute of Child Health & Hospital for Children, Egmore, Chennai. *****, Dept of Pediatric Gastroenterology, Institute of Child Health & Hospital for Children, Egmore, Chennai. ******
Aim: To study the

clinical profile of Wilson's disease

in children less than 12 years at presentation.

Materials and Methods:

It is a hospital based descriptive study done at Institute of Child Health & Hospital for children between June 2003 to November 2004. Children with acute and chronic liver disease, speech, gait disturbances, recent deterioration of school performance and siblings of proven cases of Wilson's disease were included. Serum ceruloplasmin <20 mg%, positive KF ring, liver biopsy showing evidence of liver disease were the criteria used in diagnosing Wilson's disease.

Results:

32 cases of Wilson's disease were diagnosed in the study period between the age group of 3-12 years. 56.2% were between 10-12 years. Male to Female ratio was 1.9:1 Hepatic manifestation alone was seen in 53.12% of the cases [Commonest presentation being chronic liver disease (64.7%), Acute hepatitis was seen in 23.52%]. Neurological manifestation alone was seen in 24%. Commonest presentation among them being speech disturbance (75%). Hepato-cerebral manifestations were seen in 18.75%. Renal manifestations were observed in 3.12%. Liver biopsy showed cirrhosis in 56.25% and chronic hepatitis in 43.75%.

Conclusion:

Wilson's disease most commonly presented between 10-12 years with male preponderance and predominantly hepatic manifestations in our study.
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Radhika V, Anupama M, Hareesh, Geetha S, Nirmala D, Raju B B.. Available From : http://www.pediatriconcall.com/fordoctor/ Conference_abstracts/report.aspx?reportid=219
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