title: Hyperphagia among patients with Bardet-Biedl syndrome.
abstract: BACKGROUND
  The importance of hyperphagia as a cause for energy imbalance in humans with Bardet-Biedl syndrome (BBS) has not been established. We therefore compared hyperphagic symptoms in patients with BBS vs. controls.
  
  
  METHODS
  We studied 13 patients with BBS and 23 non-syndromic controls with similar age, sex and body mass index (BMI) z-score. A 13-item hyperphagia questionnaire was completed by patients' parents/guardians.
  
  
  RESULTS
  Total hyperphagia questionnaire score was higher in BBS than controls (27.6 ± 9.0 vs. 19.1 ± 7.9, P = 0.005). Behaviour and drive subscales were higher for BBS than controls (12.5 ± 4.1 vs. 7.8 ± 3.2, P = 0.001, and 11.2 ± 4.1 vs. 8.3 ± 3.8, P = 0.04, respectively); severity was not significantly different between groups (3.8 ± 1.5 vs. 3.0 ± 1.3, P = 0.072). After adjustment for demographic variables and BMI z-score, total and behaviour subscale scores remained significantly different between groups, suggesting food-seeking activity, rather than preoccupation with food may be the main hyperphagic feature among patients with BBS.
  
  
  CONCLUSIONS
  Appetite dysregulation may contribute to obesity in BBS.
authors.affiliation: Section on Growth and Obesity (SGO), Program in Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), DHHS, Bethesda, MD, USA.
authors.forename: R
authors.initials: R
authors.lastname: Sherafat-Kazemzadeh
authors.forename: L
authors.initials: L
authors.lastname: Ivey
authors.forename: S R
authors.initials: SR
authors.lastname: Kahn
authors.forename: J C
authors.initials: JC
authors.lastname: Sapp
authors.forename: M D
authors.initials: MD
authors.lastname: Hicks
authors.forename: R C
authors.initials: RC
authors.lastname: Kim
authors.forename: A J
authors.initials: AJ
authors.lastname: Krause
authors.forename: L B
authors.initials: LB
authors.lastname: Shomaker
authors.forename: L G
authors.initials: LG
authors.lastname: Biesecker
authors.forename: J C
authors.initials: JC
authors.lastname: Han
authors.forename: J A
authors.initials: JA
authors.lastname: Yanovski
mesh_terms: D017668:Age of Onset; D020788:Bardet-Biedl Syndrome / Q000150:complications* / Q000378:metabolism / Q000523:psychology; D001823:Body Composition; D015992:Body Mass Index; D002648:Child; D004734:Energy Metabolism; D005260:Female; D006801:Humans; D006963:Hyperphagia / Q000150:complications* / Q000378:metabolism / Q000523:psychology; D008297:Male; D009765:Obesity / Q000209:etiology* / Q000378:metabolism / Q000523:psychology; D010290:Parents; D011795:Surveys and Questionnaires
keywords: Bardet-Biedl syndrome; hyperphagia; obesity syndromes; polyphagia