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

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.

• Authors: W J Steinbach, R L Hintz
• Journal: Journal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) 2000 Nov-Dec Vol. 13 Issue 9 Pg. 1633-6 ISSN: 0334-018X [Print] Germany
• PMID: 11154160 (Publication Type: Case Reports, Journal Article)

Topics

• Abnormalities, Multiple (physiopathology)
• Anus, Imperforate (complications)
• Cardiomegaly (complications, congenital)
• Child
• Deafness (complications)
• Diabetes Mellitus (etiology)
• Dwarfism (complications)
• Female
• Humans
• Insulin Resistance
• Microcephaly (complications)
• Nose (abnormalities)
• Syndrome