[Neonatal diabetes, heterogeneous syndrome]

Abstract

Neonatal diabetes (ND) is a rare condition of hyperglycaemia which occurs, generally, within the first month of life, requires insulin therapy and lasts more than two weeks. The pathogenesis is uncertain and the different clinical variants are heterogeneous; both a "permanent" and a "transient" form of ND exist. We report the clinical history of three neonates (A.L., A.B. and V.I.) in which a tout-court diagnosis of ND was done; the follow-up allowed us to reevaluate the initial diagnoses and suppose different ethiopathogenic mechanisms. Clinical observations, insulin requirement and biochemical data obtained during the years of follow-up allowed to distinguish three different situations: a "permanent" ND (A.L.), a "transient-recurrent" ND (A.B.), and a Maturity Onset Diabetes of the Young (MODY) (V.I.).