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Indian childhood cirrhosis in an American child

M Adamson1, B Reiner, J L Olson

  • 1Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland.

Gastroenterology
|May 1, 1992
PubMed

Insights

Indian childhood cirrhosis, a fatal pediatric liver disease, involves copper buildup in liver cells. This case highlights the disease

Area of Science:

  • Hepatology
  • Pediatric Gastroenterology
  • Medical Genetics

Background:

  • Indian childhood cirrhosis (ICC) is a severe, often fatal, pediatric liver disease.
  • It is characterized by excessive copper accumulation within hepatocytes.
  • The disease typically affects infants and young children.

Observation:

  • A case study of a two-year-old American boy with clinical, biochemical, and histological features of ICC.
  • Liver biopsies showed rapid progression from fibrosis to micronodular cirrhosis.
  • Hepatocytes exhibited significant copper and copper-binding protein accumulation.

Findings:

  • Electron microscopy revealed characteristic dense granules containing copper and sulfur.
  • Hepatic copper levels were markedly elevated (1500 µg/g dry weight) compared to normal (20-50 µg/g).
  • Urinary copper excretion was increased, and serum ceruloplasmin levels were elevated.

Implications:

  • Suggests a potential interplay between genetic predisposition and environmental factors in ICC.
  • Recommends considering ICC in non-Indian infants presenting with progressive liver disease.
  • Emphasizes the importance of copper analysis in diagnosing pediatric liver conditions.

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