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Pediatr Pathol Lab Med. 1995 May-Jun;15(3):477-83.

Neonatal diabetes mellitus associated with severe diarrhea, hyperimmunoglobulin E syndrome, and absence of islets of Langerhans.

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Department of Pathology, Royal Brisbane Hospital, Herston, Australia.


Neonatal insulin-dependent diabetes mellitus (DM) is very rare and descriptions of the pancreatic pathology in affected infants vary considerably. Death after 10 months of a male child who suffered the onset of insulin-dependent DM as a neonate, together with severe diarrhea and features of the hyperimmunoglobulin E syndrome, was found to be associated with absence of islets of Langerhans. There was no evidence of any pancreatic exocrine abnormality or other endocrinopathy. Two male relatives with insulin-dependent DM have also died as infants, and after review of the literature it is suggested that this disease process may be part of the spectrum of an X-linked syndrome of diarrhea, polyendocrinopathy, and fatal infection in infancy. Evidence is presented to support the suggestion that this syndrome is an autoimmune disorder.

[Indexed for MEDLINE]

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