title: Clinical characteristics and effects of GH replacement therapy in adults with childhood-onset craniopharyngioma compared with those in adults with other causes of childhood-onset hypothalamic-pituitary dysfunction.
abstract: OBJECTIVE
  Adults with childhood-onset (CO) craniopharyngioma (COCP) have poor quality of life (QoL) and clinical outcomes, but few studies have compared these patients with adults with other causes of CO hypothalamic-pituitary dysfunction. In this study, we compared baseline clinical characteristics and patient-reported outcomes before starting GH replacement therapy in adults with GH deficiency (GHD) due to COCP with those of adults either with CO idiopathic/congenital hypopituitarism (COH) or with CO extrasellar (COE) tumours, and evaluated the 1- and 5-year effects of GH replacement therapy.
  
  
  METHODS
  Retrospective analysis of the data recorded in KIMS (Pfizer International Metabolic Database) was carried out. Patients with COCP, COH and COE tumours were evaluated at baseline, and after 1 and 5 years of therapy.
  
  
  RESULTS
  Compared with COH and COE patients, more COCP patients underwent surgery, had greater abnormalities of body composition and higher prevalence of pituitary hormone deficits (all P<0.001), but comparable fasting glucose, HbA1c, total cholesterol and LDL-cholesterol levels, marital status, parenthood, living arrangements, education, employment and annual sick-leave days. After 1 and 5 years of GH replacement therapy, similar changes were evident with regard to body composition, fasting glucose and HbA1c levels, QoL, and the level of and satisfaction with physical activity across the three groups.
  
  
  CONCLUSIONS
  Adults with untreated COCP with GHD at baseline demonstrated more co-morbidities including greater abnormalities of body composition, pituitary hormone deficits and visual field defects. Overall, adults with COCP, COH and COE tumours responded comparably to short- and long-term GH replacement therapy, suggesting that patients with GHD due to COCP benefited from GH replacement therapy to a similar degree as those with other causes of CO hypothalamic-pituitary dysfunction did.
authors.affiliation: Division of Endocrinology, Diabetes and Clinical Nutrition, Department of Medicine, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Mailcode: L607, Portland, Oregon 97239-3098, USA.
authors.forename: Kevin C J
authors.initials: KC
authors.lastname: Yuen
authors.forename: Maria
authors.initials: M
authors.lastname: Koltowska-Häggström
authors.forename: David M
authors.initials: DM
authors.lastname: Cook
authors.forename: Janet L
authors.initials: JL
authors.lastname: Fox
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Jönsson
authors.forename: Mitchell E
authors.initials: ME
authors.lastname: Geffner
authors.forename: Roger
authors.initials: R
authors.lastname: Abs
mesh_terms: D000236:Adenoma / Q000150:complications / Q000601:surgery / Q000628:therapy; D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D003397:Craniopharyngioma / Q000150:complications* / Q000601:surgery / Q000628:therapy; D005260:Female; D019382:Human Growth Hormone / Q000172:deficiency / Q000627:therapeutic use*; D006801:Humans; D007018:Hypopituitarism / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D010911:Pituitary Neoplasms / Q000150:complications* / Q000601:surgery / Q000628:therapy; D011788:Quality of Life; D012189:Retrospective Studies; D012919:Social Behavior; D055815:Young Adult
chemical_list: D019382:Human Growth Hormone