Up-to-date, most patients with serious chronic hepatic disease are best treated by liver transplantation. It has been confirmed the striking benefit of liver transplantation also for patients with glycogen storage disease or homozygous familial hypercholesterolemia who were refractory to medical treatment. Nevertheless, the advantage of achieving palliation without transplantation, thereby avoiding the need for chronic immunosuppression, is obvious. With reference to the mentioned above diseases, end-to-side portacaval shunt was used. A favourable effect was noted on body growth and a number of metabolic abnormalities. Hepatic failure did not occur, although in a few patients blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. To avoid an incomplete palliation provided by portacaval shunt, appropriate case selection is a problem. The Authors report their personal experience with portacaval shunt for the treatment of glycogenosis and familial hypercholesterolemia.
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