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Wilson's Disease in Bangladeshi Children: Analysis of 100 Cases

Md Rukunuzzaman1

  • 1Department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.

Pediatric Gastroenterology, Hepatology & Nutrition
|July 10, 2015
PubMed

Insights

Wilson's disease (WD) in children often presents with liver issues, with most cases showing chronic liver disease. Early jaundice in children over three warrants investigation for WD.

Area of Science:

  • Pediatric Hepatology
  • Neurology
  • Clinical Biochemistry

Background:

  • Wilson's disease (WD) is an inherited disorder of copper metabolism.
  • Early diagnosis and treatment are crucial to prevent severe liver and neurological damage.

Purpose of the Study:

  • To characterize the clinical and laboratory features of Wilson's disease in a pediatric cohort.
  • To identify key diagnostic indicators in children with suspected WD.

Main Methods:

  • A cross-sectional study involving 100 children (3-18 years) diagnosed with Wilson's disease.
  • Evaluation of clinical presentation, hepatic and neurological manifestations, and laboratory findings including serum and urinary copper levels.

Main Results:

  • The majority of pediatric WD patients presented with hepatic manifestations (69%), commonly chronic liver disease (42%).
  • Neurological and psychiatric symptoms were observed in some patients, with onset typically in the 10-15 year age group.
  • Key laboratory findings included Kayser-Fleischer rings (76%), elevated transaminases (85%), low serum ceruloplasmin (73%), and abnormal urinary copper excretion (81-92%).

Conclusions:

  • Wilson's disease in children frequently manifests with hepatic involvement, particularly chronic liver disease.
  • Investigating jaundice in children over three years of age for Wilson's disease is recommended.
  • Comprehensive clinical and laboratory assessment is vital for diagnosing pediatric Wilson's disease.
Abstract

Keywords:
BangladeshChildWilson's disease

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