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

Daniela Seick Barbarini, Vera Haslinger, Katerina Schmidt, Ann-Marie Patch, Guido Müller, Burkhard Simma

Index: Pediatr. Diabetes 10(7) , 487-91, (2009)

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Abstract

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.