Permanent neonatal diabetes mellitus: a case report with plasma insulin studies.

The 11th case of permanent neonatal diabetes mellitus appearing during the first month of life is reported. A critical review of the literature is also presented. The permanence of diabetes is demonstrated by the duration of insulin therapy still necessary after 30 months. Insulin-stimulation tests have been performed some for the first time in such a young diabetic. They have shown a nearly total failure in beta-cell response, only very high doses of glucagon provoking a moderate insulin secretion. The absence of acetonuria is discussed. It can perhaps be explained by the hyperglycemia which, by a mass effect, brings about cellular glucose penetration and this stops liberation of Nefa's from adipose tissue.