LONG-TERM COURSE OF NEONATAL DIABETES

Background. Neonatal diabetes mellitus defined here as hyperglycemia o ccurring within the first month of life that lasts for at least two we eks and requires insulin therapy - is a very rare form of the disease. Little is known about it, particularly with respect to its long-term course. Methods. We studied two brothers who had neonatal diabetes and obtained follow-up information on 34 patients described in the litera ture as well as information on 21 additional patients. Forty-seven of the patients had neonatal diabetes, as defined above, and in 10 others the onset was between the first and third month of life. Results. Twe nty-six of the 57 infants had permanent diabetes, 18 had transient dia betes, and 13 had transient diabetes that recurred when they were 7 to 20 years old. Neonatal diabetes was associated with the Wolcott-Ralli son syndrome in six infants, hyperuricemia due to phosphoribosyl-ATP p yrophosphatase hyperactivity in two, and celiac disease in two. Forty- one of 45 neonates in whom the duration of gestation and birth weight were known were small for their gestational ages. There were two pairs of affected twins and four other families with two or more infants wi th neonatal diabetes, but only three parents had diabetes. The inciden ce of neonatal diabetes mellitus in Germany has been estimated to be 1 in 500,000 neonates. Conclusions. Neonatal diabetes differs from insu lin-dependent diabetes in that its course is highly variable. Some pat ients have permanent diabetes, but others have transient or lasting re missions.