title: Bone mineral density in children and adolescents with Prader-Willi syndrome: a longitudinal study during puberty and 9 years of growth hormone treatment.
abstract: BACKGROUND
  Longitudinal data on bone mineral density (BMD) in children and adolescents with Prader-Willi Syndrome (PWS) during long-term GH treatment are not available.
  
  
  OBJECTIVE
  This study aimed to determine effects of long-term GH treatment and puberty on BMD of total body (BMDTB), lumbar spine (BMDLS), and bone mineral apparent density of the lumbar spine (BMADLS) in children with PWS.
  
  
  METHODS
  This was a prospective longitudinal study of a Dutch PWS cohort.
  
  
  METHODS
  Seventy-seven children with PWS who remained prepubertal during GH treatment for 4 years and 64 children with PWS who received GH treatment for 9 years participated in the study.
  
  
  METHODS
  The children received GH treatment, 1 mg/m(2)/day (≅ 0.035 mg/kg/d).
  
  
  METHODS
  BMDTB, BMDLS, and BMADLS was measured by using the same dual-energy x-ray absorptiometry machine for all annual measurements.
  
  
  RESULTS
  In the prepubertal group, BMDTB standard deviation score (SDS) and BMDLSSDS significantly increased during 4 years of GH treatment whereas BMADLSSDS remained stable. During adolescence, BMDTBSDS and BMADLSSDS decreased significantly, in girls from the age of 11 years and in boys from the ages of 14 and 16 years, respectively, but all BMD parameters remained within the normal range. Higher Tanner stages tended to be associated with lower BMDTBSDS (P = .083) and a significantly lower BMADLSSDS (P = .016). After 9 years of GH treatment, lean body mass SDS was the most powerful predictor of BMDTBSDS and BMDLSSDS in adolescents with PWS.
  
  
  CONCLUSIONS
  This long-term GH study demonstrates that BMDTB, BMDLS, and BMADLS remain stable in prepubertal children with PWS but decreases during adolescence, parallel to incomplete pubertal development. Based on our findings, clinicians should start sex hormone therapy from the age of 11 years in girls and 14 years in boys unless there is a normal progression of puberty.
authors: Dutch Growth Research Foundation (N.E.B., R.J.K., E.P.C.S., R.F.A.T.d. L.v.W., D.A.M.F., A.C.S.H.-K), 3000 CA Rotterdam, The Netherlands; Children's Hospital Erasmus MC-Sophia (N.E.B., R.J.K., E.P.C.S., G.C.B.B.d.H., A.C.S.H.-K.), 3000 CA Rotterdam, The Netherlands; University of Groningen (G.B.), University Medical Center Groningen/Beatrix Children's Hospital, Department of Pediatrics, 9713 GZ Groningen, The Netherlands; Diaconessen Hospital (D.A.J.P.), 2334 CK Leiden, The Netherlands; St. Antonius Hospital (J.J.G.H.-N., H.V.W.), 3430 EM Nieuwegein, The Netherlands; Haga Hospitals/Juliana Children's Hospital (E.C.A.M.H.), 2566 MJ The Hague, The Netherlands; Department of Pediatrics (P.E.J.), Jeroen Bosch Hospital, 5200 ME's-Hertogenbosch, The Netherlands; Gelre Hospitals (L.L.), 7300 SD Apeldoorn, The Netherlands; St. Catharina Hospital (R.J.O.), 5623 EJ Eindhoven, The Netherlands; Department of Pediatrics (W.O.), Leiden University Medical Center, 2333 ZA Leiden, The Netherlands; VU University Medical Center (J.R.), 1081 HV Amsterdam, The Netherlands; Radboud University Nijmegen Medical Center (A.A.E.M.V.A.); 6500 HB Nijmegen, The Netherlands; St. Jansdal Hospital (M.V.L.), 3844 DG Harderwijk, The Netherlands; Medical Center Twente (M.E.J.W.B.), 7511 JX Enschede, The Netherlands; Academic Medical Center (N.Z.-S.), University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
authors: N E
authors: NE
authors: Bakker
authors: R J
authors: RJ
authors: Kuppens
authors: E P C
authors: EP
authors: Siemensma
authors: R F A
authors: RF
authors: Tummers-de Lind van Wijngaarden
authors: D A M
authors: DA
authors: Festen
authors: G C B
authors: GC
authors: Bindels-de Heus
authors: G
authors: G
authors: Bocca
authors: D A J P
authors: DA
authors: Haring
authors: J J G
authors: JJ
authors: Hoorweg-Nijman
authors: E C A M
authors: EC
authors: Houdijk
authors: P E
authors: PE
authors: Jira
authors: L
authors: L
authors: Lunshof
authors: R J
authors: RJ
authors: Odink
authors: W
authors: W
authors: Oostdijk
authors: J
authors: J
authors: Rotteveel
authors: A A E M
authors: AA
authors: Van Alfen
authors: M
authors: M
authors: Van Leeuwen
authors: H
authors: H
authors: Van Wieringen
authors: M E J
authors: ME
authors: Wegdam-den Boer
authors: N
authors: N
authors: Zwaveling-Soonawala
authors: A C S
authors: AC
authors: Hokken-Koelega
mesh_terms: D000293:Adolescent; D001823:Body Composition / Q000187:drug effects; D015519:Bone Density / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D012739:Gonadal Steroid Hormones / Q000627:therapeutic use; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009426:Netherlands; D011218:Prader-Willi Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D011627:Puberty / Q000187:drug effects / Q000502:physiology; D013997:Time Factors
chemical_list: D012739:Gonadal Steroid Hormones; D019382:Human Growth Hormone