Portacaval Shunt for Glycogen Storage Disease and Hyperlipidaemia

Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

“…In 1952, GSD I became the first inborn error of metabolism proven to be caused by an enzyme deficiency when Gerty and Carl Cori demonstrated deficiency of glucose‐6‐phosphatase activity in liver samples obtained in patients who had a clinical diagnosis of von Gierke disease . In the 1960s, portacaval shunts were introduced as a way to maintain normal blood glucose concentrations in the systemic circulation, but the condition was almost universally fatal until 1972 when continuous glucose therapy was introduced . While continuous feeds improved survival for this population, hyperinsulinemia caused by the glucose infusion was associated with life‐threatening hypoglycemia and possible sudden death when any interruption occurred .…”

Abnormal Growth in Autoimmune Chronic Active Hepatitis?

“…They suggest subnormal growth prior to corticosteroid treatment and supranormal growth both during alternate-day therapy of CAH and after discontinuation of steroids. The only previous report of increased growth velocity following treatment of a liver disease has been with portacaval shunts for glycogen storage disease (10). Have we been remiss in not documenting these growth abnormalities previously?…”