title: Cystic-fibrosis related-diabetes (CFRD) is preceded by and associated with growth failure and deteriorating lung function.
abstract: BACKGROUND
  Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents.
  
  
  METHODS
  A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed. Standard deviation scores (SDS) of height, growth, weight, body mass index (BMI), forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and forced expiratory flow at 75% of expired FVC (FEF75) were recorded during a mean observation period of 13 years per patient.
  
  
  RESULTS
  SDS of height and weight were reduced in CF patients with diabetes compared to those without, not only at the point of diagnosis (both p<0.05) but years before the evidence of diabetes. Afterwards there was a significant decline in height (p<0.001) and weight (p<0.01) SDS in CFRD patients and an increasing difference between the height and weight of CF patients with or without diabetes. In contrast, no significant reduction of BMI-SDS was observed in CFRD patients. All analysed lung function parameters showed a marked decline in CFRD patients starting 1 year prior to the diagnosis of diabetes.
  
  
  CONCLUSIONS
  Deteriorating growth, reduced weight and impaired lung function are related to the development of CFRD and are obvious several years before the actual diagnosis of diabetes.
authors.affiliation: .
authors.forename: Nicolas
authors.initials: N
authors.lastname: Terliesner
authors.affiliation: .
authors.forename: Mandy
authors.initials: M
authors.lastname: Vogel
authors.affiliation: .
authors.forename: Anna
authors.initials: A
authors.lastname: Steighardt
authors.affiliation: .
authors.forename: Ruth
authors.initials: R
authors.lastname: Gausche
authors.affiliation: .
authors.forename: Constance
authors.initials: C
authors.lastname: Henn
authors.affiliation: .
authors.forename: Julia
authors.initials: J
authors.lastname: Hentschel
authors.affiliation: .
authors.forename: Thomas
authors.initials: T
authors.lastname: Kapellen
authors.affiliation: .
authors.forename: Sabine
authors.initials: S
authors.lastname: Klamt
authors.affiliation: .
authors.forename: Julia
authors.initials: J
authors.lastname: Gebhardt
authors.affiliation: .
authors.forename: Wieland
authors.initials: W
authors.lastname: Kiess
authors.affiliation: .
authors.forename: Freerk
authors.initials: F
authors.lastname: Prenzel
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology; D003920:Diabetes Mellitus / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005951:Glucose Tolerance Test; D006130:Growth Disorders / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D015430:Weight Gain / Q000502:physiology; D055815:Young Adult
keywords: cystic fibrosis; cystic fibrosis-related diabetes; growth failure; lung function