Familial and recurrent veno-occlusive disease of liver in Jamaican children (abstract only)

ABSTRACT

The case histories of four children in a family of seven are presented. The eldest of the four, a girl, presented in 1961 at age 4 1/2 years with evidence of liver disease and portal hypertension. A liver biopsy was not performed. This child died in 1964 of hepatorenal failure and unfortunately permission for autopsy was refused. The second affected child, a boy, presented at age 2 years with hepatosplenomegaly and jaundice and a needle biopsy of his liver revealed a histological picture consistent with post V.O.D. cirrhosis. He has remained fairly well since, although signs of cirrhosis persist. In June 1966 two female siblings age 1 year and 2 years respectively, presented at the same time with acute abdominal swelling. Liver biopsies confirmed the clinical diagnosis of acute V.O.D. and their subsequent progress is briefly reviewed. The case history of a boy who has had two proven episodes of acute V.O.D. is presented. This child was first admitted in January 1958 at age 1 year and five months, with acute abdominal swelling. A liver biopsy revealed histological evidence of acute V.O.D. His clinical condition settled and repeat biopsy in March 1958 showed no residual liver disease. He remained well after this until he had an episode of obstructive jaundice in 1960 which subsided spontaneously. In 1965 he was readmitted with a history of acute abdominal swelling and after two months during which time his ascites gradually subsided, a liver biopsy was performed and this showed subacute V.O.D. A final biopsy was done in September 1966 and this showed only minimal increase of fibrous tissue. A brief note on the aetiology of V.O.D. is submitted(AU)