title: Beneficial effects of growth hormone treatment on cognition in children with Prader-Willi syndrome: a randomized controlled trial and longitudinal study.
abstract: BACKGROUND
  Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited.
  
  
  METHODS
  Fifty prepubertal children aged 3.5 to 14 yr were studied in a randomized controlled GH trial during 2 yr, followed by a longitudinal study during 4 yr of GH treatment. Cognitive functioning was measured biennially by short forms of the WPPSI-R or WISC-R, depending on age. Total IQ (TIQ) score was estimated based on two subtest scores.
  
  
  RESULTS
  During the randomized controlled trial, mean sd scores of all subtests and mean TIQ score remained similar compared to baseline in GH-treated children with PWS, whereas in untreated controls mean subtest sd scores and mean TIQ score decreased and became lower compared to baseline. This decline was significant for the Similarities (P = 0.04) and Vocabulary (P = 0.03) subtests. After 4 yr of GH treatment, mean sd scores on the Similarities and Block design subtests were significantly higher than at baseline (P = 0.01 and P = 0.03, respectively), and scores on Vocabulary and TIQ remained similar compared to baseline. At baseline, children with a maternal uniparental disomy had a significantly lower score on the Block design subtest (P = 0.01) but a larger increment on this subtest during 4 yr of GH treatment than children with a deletion. Lower baseline scores correlated significantly with higher increases in Similarities (P = 0.04) and Block design (P < 0.0001) sd scores.
  
  
  CONCLUSIONS
  Our study shows that GH treatment prevents deterioration of certain cognitive skills in children with PWS on the short term and significantly improves abstract reasoning and visuospatial skills during 4 yr of GH treatment. Furthermore, children with a greater deficit had more benefit from GH treatment.
authors.affiliation: Dutch Growth Research Foundation/Erasmus MC Rotterdam, Westzeedijk 106, 3016 AH Rotterdam, The Netherlands. e.siemensma@kindengroei.nl
authors.forename: Elbrich P C
authors.initials: EP
authors.lastname: Siemensma
authors.forename: Roderick F A
authors.initials: RF
authors.lastname: Tummers-de Lind van Wijngaarden
authors.forename: Dederieke A M
authors.initials: DA
authors.lastname: Festen
authors.forename: Zyrhea C E
authors.initials: ZC
authors.lastname: Troeman
authors.forename: A A E M Janielle
authors.initials: AA
authors.lastname: van Alfen-van der Velden
authors.forename: Barto J
authors.initials: BJ
authors.lastname: Otten
authors.forename: Joost
authors.initials: J
authors.lastname: Rotteveel
authors.forename: Roelof J H
authors.initials: RJ
authors.lastname: Odink
authors.forename: G C B Karen
authors.initials: GC
authors.lastname: Bindels-de Heus
authors.forename: Mariette
authors.initials: M
authors.lastname: van Leeuwen
authors.forename: Danny A J P
authors.initials: DA
authors.lastname: Haring
authors.forename: Wilma
authors.initials: W
authors.lastname: Oostdijk
authors.forename: Gianni
authors.initials: G
authors.lastname: Bocca
authors.forename: E C A
authors.initials: EC
authors.lastname: Mieke Houdijk
authors.forename: A S Paul
authors.initials: AS
authors.lastname: van Trotsenburg
authors.forename: J J Gera
authors.initials: JJ
authors.lastname: Hoorweg-Nijman
authors.forename: Hester
authors.initials: H
authors.lastname: van Wieringen
authors.forename: René C F M
authors.initials: RC
authors.lastname: Vreuls
authors.forename: Petr E
authors.initials: PE
authors.lastname: Jira
authors.forename: Eelco J
authors.initials: EJ
authors.lastname: Schroor
authors.forename: Evelyn
authors.initials: E
authors.lastname: van Pinxteren-Nagler
authors.forename: Jan
authors.initials: J
authors.lastname: Willem Pilon
authors.forename: L Bert
authors.initials: LB
authors.lastname: Lunshof
authors.forename: Anita C S
authors.initials: AC
authors.lastname: Hokken-Koelega
mesh_terms: D000293:Adolescent; D002648:Child; D002657:Child Development / Q000187:drug effects / Q000502:physiology; D002675:Child, Preschool; D003071:Cognition / Q000187:drug effects* / Q000502:physiology; D005260:Female; D019382:Human Growth Hormone / Q000494:pharmacology* / Q000627:therapeutic use*; D006801:Humans; D007361:Intelligence Tests; D008137:Longitudinal Studies; D008297:Male; D011218:Prader-Willi Syndrome / Q000188:drug therapy* / Q000503:physiopathology / Q000523:psychology; D012107:Research Design; D013997:Time Factors
chemical_list: D019382:Human Growth Hormone