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Pediatr Diabetes. 2009 Nov;10(7):487-91. doi: 10.1111/j.1399-5448.2009.00523.x. Epub 2009 Jun 3.

Neonatal diabetes mellitus due to pancreas agenesis: a new case report and review of the literature.

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Department of Pediatrics, Academic Teaching Hospital, Landeskrankenhaus Feldkirch, A-6800 Feldkirch, Voralberg, Austria.


Neonatal diabetes mellitus (NDM) is a rare condition (1:400,000 neonates) defined as hyperglycemia occurring in the first months of life, lasting more than 2 wk and requiring insulin for management. We here report on a 33-month-old girl with pancreatic agenesis, an extremely rare cause of permanent neonatal diabetes mellitus (PNDM). Timely diagnosis and adequate treatment of both endocrine and exocrine insufficiency may permit survival and normal development.

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