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June 1993

Permanent Neonatal Diabetes Mellitus and Pancreatic Exocrine Insufficiency Resulting From Congenital Pancreatic Agenesis

Am J Dis Child. 1993;147(6):607-609. doi:10.1001/archpedi.1993.02160300013005

Sir.—Neonatal diabetes mellitus (DM) is uncommon and usually presents with hyperglycemia, glucosuria, significant dehydration, and small size for gestational age.1 The transient form of neonatal DM usually resolves in weeks to months.1 The origin of the much rarer, permanent form of neonatal DM remains unclear. In older children DM usually results from autoimmune destruction of the pancreas as reflected by the presence of islet cell antibodies. However, islet cell antibodies have not been detected in neonates with DM.2,3

Neonates having both pancreatic exocrine and endocrine insufficiency are rarer still. Winter and colleagues4 diagnosed congenital pancreatic hypoplasia in two brothers who presented with neonatal DM and subsequently developed pancreatic exocrine insufficiency at ages 5 and 21 years. Previously pancreatic agenesis was considered fatal.5,6 However, a report by Howard and colleagues7 described the survival of an infant with permanent neonatal DM who was found to have