Portal vein thrombosis in a child with homozygous sickle-cell disease

A 13-year old boy with homozygous sickle-cell (SS) disease died suddenly at home folllowing a short history of abdominal pain. Autopsy revealed venous thrombosis of the hepatic, portal, superior mesenteric and splenic veins. Venous thrombosis is rare in SS disease and thrombosis of mesenteric vessels is most frequently seen in chronic myeloproliferative disorders. Its occurrence in SS disease raises the possibility of a common pathogenesis and adds another pathology to the causes of abdominal painful crisis. (AU)

The clinical picture of veno-occlusive disease of the liver in Jamaican children

A study was made of 11 children suffering from "veno-occlusive disease of the liver" (V.O.D.). Clinically the disease was characterized by three overlapping phases, from the acute stage, with the sudden onset of hepatomegaly, with or without ascites, usually in infants from 18 months to three years of age, to the chronic stage, with the development of frank cirrhosis in later childhood. An attempt is made to outline the clinical natural history of V.O.D., as suggested by present information. The histology of V.O.D. is considered in outline. The primary lesion is a wide-spread occlusion of the smaller branches of the hepatic vein, the result of a primary endo-phlebitis; this is followed by the development of a non-portal type of cirrhosis radiating out from the central veins. The aetiology of V.O.D. is unknown, but it does not appear to be primarily or entirely nutritional; affected children are usually apparently adequately nourished. The possibility that V.O.D. is produced by the action of toxins, either bacterial or chemical, is considered, with special reference to "bush teas," which are widely used in Jamaica. Points of similarity are discussed between V.O.D. and serous hepatosis, Chiari's syndrome, senecio poisoning and Indian infantile cirrhosis (Summary)