The effect of portacaval transposition on carbohydrate metabolism: Experimental and clinical observations

An investigation was conducted of the influence of portacaval transposition upon carbohydrate metabolism in 45 dogs. In 17 dogs, hepatic glycogen content was measured before and from 45 to 75 days after transposition. A reduction in glycogen content, principally in the TCA soluble fraction, was noted in 14 animals. The mean loss of total glycogen was 51 percent, and the mean loss of TCA soluble glycogen was 70 percent. In control animals hepatic deglycogenation did not occur. Despite the reduction in hepatic glycogen content, the animals were capable of glucagon-induced glycogenolysis using very small test doses. After transposition, a greater response to intraportal injection was noted as compared to that obtained with systemic venous infusions. Other alterations in carbohydrate metabolism were also measured. These included a reduction in the duration and magnitude of the hyperglycemic response to oral glucose loads. The profile of glycemic response under these conditions was studied, and demonstrated to be greatest in the portal vein, least in the peripheral venous blood, and of intermediate magnitude in the peripheral arteries. Based upon the hepatic deglycogenating effect of portacaval transposition in dogs, this operation was used for the treatment of an 8 1 2-year-old child with glycogen storage disease and concomitant portal cirrhosis. The portacaval transposition was performed in preference to a standard portacaval shunt. The enzyme defect in the patient was extensively studied before and after transposition. Prior to surgery, she was demonstrated to have Type IIIB glycogenosis (amylo-1,6-glucosidase deficiency confined to the liver). Eight and one half months after operation, the quantities of glycogen in liver and muscle and the enzyme activities showed no significant alteration. The clinical response to portacaval transposition was gratifying. There has been a decrease in the hepatosplenomegaly, rapid growth, a diminution in the pre-existing hypersplenism, and a considerable increase in the child's physical activity. Most of these benefits are ascribable to the effective portal decompressive procedure. Whether any metabolic benefit derived from the portacaval transposition is problematical. © 1965