title: Diabetes mellitus associated with cystic fibrosis.
abstract: The prevalence of overt diabetes mellitus and carbohydrate intolerance was studied in 448 patients with cystic fibrosis (CF). Insulin-dependent diabetes (IDDM) developed in 7.6% of patients (13 male and 21 female). Survival was significantly lower (P less than 0.01) in the IDDM-CF group, with fewer than 25% surviving to age 30 years, whereas nearly 60% of the nondiabetic CF population reached this age. A significant deterioration in CF clinical status, based on NIH score, became apparent 2 years before onset of overt IDDM (P less than 0.05 at 2 years prior, P less than 0.01 at IDDM diagnosis). Total glycosylated hemoglobin (HbA1) was significantly (P less than 0.001) higher for the total CF population (7.3% +/- 1.2%) than for the general non-CF population (6.5% +/- 0.7%), and in the IDDM-CF group (P less than 0.05) compared with normoglycemic CF control patients. Female patients had a higher mean HbA1 after 12 years of age than their male counterparts did (P less than 0.02). HBA1 did not predict the development of IDDM, but there was a weak inverse relationship between HbA1 and both NIH clinical score (r = -0.41, P less than 0.02) and standard pulmonary function tests (forced vital capacity, r = -0.25, P less than 0.01) in the general CF population. Therefore, impaired carbohydrate tolerance in CF is associated with progressive clinical deterioration.
authors.affiliation: Department of Laboratory Medicine, University of Minnesota, Minneapolis 55455.
authors.forename: S M
authors.initials: SM
authors.lastname: Finkelstein
authors.forename: C L
authors.initials: CL
authors.lastname: Wielinski
authors.forename: G R
authors.initials: GR
authors.lastname: Elliott
authors.forename: W J
authors.initials: WJ
authors.lastname: Warwick
authors.forename: J
authors.initials: J
authors.lastname: Barbosa
authors.forename: S C
authors.initials: SC
authors.lastname: Wu
authors.forename: D J
authors.initials: DJ
authors.lastname: Klein
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000401:mortality; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000401:mortality; D005260:Female; D006442:Glycated Hemoglobin / Q000032:analysis; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D008910:Minnesota; D012129:Respiratory Function Tests
chemical_list: D006442:Glycated Hemoglobin