Background: Neonatal diabetes is a rare cause of hyperglycemia, affecting 1: 500,000 births, with persistent hyperglycemia occurring in the first months of life lasting more than 2 weeks and requiring insulin. This condition in infants less than 6 months of age is considered as permanent neonatal diabetes mellitus.
Case Report: A rare case of permanent neonatal diabetes mellitus presented with intrauterine growth retardation (IUGR; birth weight: 1460 grams; female), hyperglycemia, glycosuria, and mild dehydration, a normal Apgar score of 8 and 9 at 1 and 5 minutes, respectively. The parents, of consanguineous union, had no prior history of diabetes mellitus. Of their 4 children, the first child had a diagnosis similar to the patient (their last child). The patient was initially started on continuous infusion of insulin, and then switched to regular insulin subcutaneously, but response was sub-optimal. She was started on neutral protamine Hagedorn, following which her condition improved. She was discharged on neutral protamine Hagedorn with regular follow-up.
Conclusions: In view of widespread consanguinity in Saudi Arabia it appears prudent and pertinent to suspect permanent neonatal diabetes mellitus following diagnosis of hyperglycemia in small-for-age infants, especially those with positive family history of diabetes. Close blood glucose monitoring is essential as long as hyperglycemia persists. Prolong follow-up is imperative.

Keywords: neonatal, Insulin, Hyperglycemia, diabetes, Consanguinity, Neutral Protamine Hagedorn