Plasma Insulin Content and Glucose Tolerance in Homocystinuria

Abstract

The plasma insulin level and glucose tolerance were studied In five patients with homocystinuria. Two of them showed a marked hyperinsulinemia and a concomitant pathologic glucose tolerance, while the remaining three showed a normal glucose tolerance, two of them, however, had plasma insulin values in the upper level of the normal range. These preliminary results support the earlier hypothesis that the pancreatic islets β-cells are particularly sensitive to an imbalance in the sulfhydryl levels of the organism since homocystinurics have an increased plasma methionine level due to an inborn error of the methionine metabolism.