Title: Wilson’s Disease in Children –Experience from a Tertiary Care Centre
Author: Sujay Chaudhuri
DOI: https://dx.doi.org/10.18535/jmscr/v6i6.29
Abstract
Objectives: To assess incidents clinical and investigational profile of Wilson’s disease in children in a northern city of India, this study was done.
Methods: The children (age<10 years) with wilson’s disease who were admitted in pediatric Gastroenterology ward of PGIMER – Chandigarh from July 1993 to June 2003 were enrolled for the study. Clinical examination, investigation (Ultrasonography, serum cerulospasmin, 24 hour urinary copper, KF ring detection, LFT, liver biopsy, liver copper estimation etc.) were done.
Result: 50 cases of Wilson’s disease were admitted in 10 years. Mean age was 7.5 years (range 5 – 10 years) Male was 30 out of 50 cases (70%) hepatosplenomegaly with transaminitis was presenting symptoms. cirrhosis was present in 15 cases (30%), fulminant Wilison’s disease in 5 cases (10%), KF ring in 35 cases (70%), neuropsychiatric symptom in 2 cases (4%) renal tubular acidosis in 5 cases (10%). liver copper was increased in all cases.
Conclusion: Wilsons’s disease is an important cause of chronic liver disease in Northern India. Early detection is mandatory for better management of the cases.
Keywords: Wilson’s disease in children, chronic liver disease.
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