Portal diversion. Treatment for glycogen storage disease and hyperlipemia

Abstract

A high degree of palliation was obtained in the cases of hepatic glycogen storage disease relieved by portal diversion. The liver in this disease is patently abnormal; the liver in homozygous type II hyperlipoproteinemia is morphologically normal. The cause for the elevated serum cholesterol and low density lipoprotein levels is not well understood and it has a poor prognosis if there is no good response to medical therapy. The end-tp-side portaceval shunt was performed in a child to reduce the serum cholesterol and lipoprotein levels. The human is apparently resistant to the toxic effects of portal diversion, though the most serious complication is that of hepatic encephalopathy. In dogs, seizures, coma and death occur regularly within a few weeks after the portacaval shunt. Pathological changes in the hepatocytes are also seen.