K. C. CHAUDHURI, m.b. Physician-in-Charge, Children's Department, Chittaranjan Seva Sadan, Calcutta The frequency of cirrhosis of the liver in infancy and childhood in India has been long recognized, but cases of disorder of the liver following a course simulating that of acute necrosis have not been reported very often in this country. Furthermore, the subject of cirrhosis has by no means been...

Two young brothers with cirrhosis are reported. They are the issue of a first-cousin marriage between Bangladeshi parents. The older boy was born in Bangladesh, the younger in England. A diagnosis of Indian childhood cirrhosis (ICC) was made on the basis of the clinical features and the liver histology. The younger brother represents the first example of this disease in a patient born in the Un...

Antibody to hepatitis A virus (anti-HAV) was found in 50% of patients with Indian childhood cirrhosis; this was not significantly different from the prevalence of anti-HAV in age- and sex-matched controls.

Background: Autoimmune hepatitis (AIH) in childhood has variable modes of presentation, and the disease should be suspected and excluded in all children presenting with symptoms and signs of prolonged or severe acute liver disease. In AIH, the liver biopsy histopathology shows inflammation in addition to presence of serum autoimmune antibodies and increased levels of immunoglobulin G (IgG). Ob...

Progressive familial intrahepatic cholestasis (PFIC) is characterized by early onset cholestasis, progressive liver cirrhosis, pruritus, poor growth and inexorable progression to liver cirrhosis in early childhood. The serum level of gamma-glutamyl transferase is low or normal, which is discordant with severe cholestasis. Five Malaysian patients with PFIC, who all had typical features of PFIC w...

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is varies from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. Unlike other autoimmune liver diseases, PBC has rarely been reported in childhood. We report a case of primary biliary cirrhosis in a 12-year-old girl. In addition to characteri...

Recent advances in molecular biology have made possible the identification of genetic defects responsible for Wilson's disease, Indian childhood cirrhosis and copper toxicosis in Long Evans Cinnamon rats, toxic milk mice, and Bedlington terriers. The Wilson's disease gene is localized on human chromosome 13 and codes for ATP7B, a copper transporting P-type ATPase. A genetic defect similar to th...