Indian Childhood Cirrhosis: Case Report and Pediatric Diagnostic Challenges

Introduction: Indian childhood cirrhosis is a chronic liver disease usually seen in paediatric age group and is unique to the Indian subcontinent. The definitive causative factor for the disease is not found till now but excess copper ingestion has been associated with it.Case presentation: An Indian origin one and half year old premorbidly normal male child presented with history of gradual distension of abdomen for 6 months and jaundice, generalized body swelling, high colour urine for 20 days. There was no history of any bleeding or feature suggestive of hepatic encephalopathy. On physical examination child was icteric, pale and had anasarca, massive hepatosplenomegaly and ascites. The child was evaluated for various causes of hepatic failure in pediatric age group including infective, metabolic and autoimmune etiologies. Unfortunately the child succumbed to the illness. The post-mortem liver biopsy and copper estimation cleared the air, revealing Indian childhood cirrhosis as the underlying etiology.Conclusion: Though Indian childhood cirrhosis is a rare entity and reported less frequently in literature, the treating pediatrician should keep this as a differential in case of pediatric hepatic failure. The liver biopsy and hepatic copper estimation are the gold standard diagnostic tests for diagnosing Indian Childhood cirrhosis.

Keywords:

Childhood cirrhosis , Copper , Mallory hyaline bodies