[Familial form of hypoglycemia due to leucine hypersensitivity].

The authors report on a boy and his mother showing signs of hypoglycemia due to hypersensitivity to leucine that is related to a that dominant autosomal. The cyclic inheritance glycemia test shows hyperinsulism; oral as well as intravenous loading dose of leucine tests showed that this hyperinsulism is due to hypersensitivity to leucine. The clinical picture, outcome of the disease dietary and medical treatment with diazoxide, and the role of GIP in insulin secretion is discussed.