title: Pubarche and serum dehydroepiandrosterone sulphate levels in children with Prader-Willi syndrome.
abstract: BACKGROUND
  Premature pubarche (PP) is reported in children with Prader-Willi Syndrome (PWS). Pubarche is preceded by adrenarche - an increase in serum levels of adrenal androgens, most specifically dehydroepiandrosterone sulphate (DHEAS).
  
  
  OBJECTIVE
  To assess DHEAS levels, the age at and progression of pubarche and the prevalence of PP in children with PWS.
  
  
  METHODS
  In the Dutch PWS Cohort Study, 120 children (6 months-17 years) are prospectively followed. Their age at onset of pubarche and various pubic hair stages and prevalence of PP were determined. Serum DHEAS levels were assessed in 97 children.
  
  
  RESULTS
  Median serum DHEAS levels were significantly higher in children with PWS than in healthy age-matched controls at ages 3-6 years (girls: P = 0·004 and boys: P = 0·010) and 6-10 years (girls: P = 0·045 and boys: P = 0·001). Age and gender significantly influenced DHEAS levels in children with PWS. The median [P10-P90] age at onset of pubarche in children with PWS was significantly younger than in healthy peers, 9·04[6·75-11·84] years in PWS girls (P < 0·0001) and 10·31 [8·65-12·29] years in PWS boys (P = 0·003). The prevalence of PP in children with PWS was 30·0% in girls and 16·1% in boys.
  
  
  CONCLUSIONS
  Compared to healthy children, children with PWS have significantly higher DHEAS levels from 3 to 10 years of age. They are younger at onset of pubarche and have a higher prevalence of premature pubarche. DHEAS levels in PWS are influenced by age and gender. Our findings indicate earlier maturation of the zona reticularis of the adrenal glands in children with PWS.
authors.affiliation: Dutch Growth Research Foundation, RotterdamDepartment of Pediatrics, Division of Endocrinology, Erasmus University Medical Center Rotterdam/Sophia's Children's Hospital, RotterdamDepartment of Pediatric Endocrinology, Radboud University Medical Center, NijmegenDepartment of Internal Medicine, Erasmus University Medical Center, Rotterdam, the Netherlands.
authors.forename: Elbrich P C
authors.initials: EP
authors.lastname: Siemensma
authors.forename: Roderick F A
authors.initials: RF
authors.lastname: de Lind van Wijngaarden
authors.forename: Barto J
authors.initials: BJ
authors.lastname: Otten
authors.forename: Frank H
authors.initials: FH
authors.lastname: de Jong
authors.forename: Anita C S
authors.initials: AC
authors.lastname: Hokken-Koelega
mesh_terms: D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D011218:Prader-Willi Syndrome / Q000097:blood* / Q000453:epidemiology; D011627:Puberty / Q000097:blood*; D011629:Puberty, Precocious / Q000097:blood* / Q000453:epidemiology; D016032:Randomized Controlled Trials as Topic
chemical_list: D019314:Dehydroepiandrosterone Sulfate