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J Pediatr Endocrinol Metab. 2000 Nov-Dec;13(9):1633-6.

Diabetes mellitus and profound insulin resistance in Johanson-Blizzard syndrome.

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1
Department of Pediatrics, Lucile Packard Children's Hospital at Stanford University, California, USA. md.stewi@lpch.stanford.edu

Abstract

Pancreatic exocrine insufficiency in Johanson-Blizzard syndrome (JBS) is well described but only two previous patient reports document pancreatic endocrine insufficiency manifested as diabetes mellitus, and each patient required only a modest dose of insulin to control hyperglycemia. We report a patient with JBS and new-onset diabetes mellitus with profound insulin resistance, with no clinical or laboratory evidence of pancreatic exocrine insufficiency.

PMID:
11154160
[Indexed for MEDLINE]
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