title: Characterization of minipuberty in infants with Prader-Willi syndrome.
abstract: BACKGROUND
  Minipuberty describes transient activation of the hypothalamic-pituitary-gonadal axis occurring during the first few months of life. Hormone levels during minipuberty were described in only a few Prader-Willi syndrome (PWS) infant boys and have not been reported in PWS infant girls.
  
  
  OBJECTIVE
  To measure gonadotropins and gonadal hormones in PWS male and female infants and assess gender-specific patterns of hormone secretion.
  
  
  METHODS
  Hormone levels in 14 (9 male, 5 female) PWS infants ages 1-3 months were compared with reference ranges for normal infants and in 44 prepubertal PWS children (27 female, 17 male).
  
  
  RESULTS
  Compared to prepubertal boys, hormone levels (median and range) for PWS infant boys were increased: LH 2.8 mIU/ml (1.2-6.2), FSH 4.4 mIU/ml (1.0-19.5), testosterone 4.0 nmol/l (3.0-7.0), inhibin B 219 pg/ml (141-325), and AMH 79 ng/ml (45-157). Hormone levels in infant girls were not significantly different from levels in prepubertal girls. LH, inhibin B, and AMH were higher in male infants than in female infants. LH/FSH ratios were 0.56 (0.24-1.77) in boys versus 0.09 (0.04-0.17) in girls (p = 0.003).
  
  
  CONCLUSIONS
  Hormone levels in PWS infant boys are in the expected minipuberty range. By contrast, reproductive hormones in most PWS infant girls did not differ from levels in prepubertal girls.
authors.affiliation: Neuropediatric Unit, Shaare Zedek Medical Center, The Hebrew University, Jerusalem, Israel.
authors.forename: Harry J
authors.initials: HJ
authors.lastname: Hirsch
authors.forename: Talia
authors.initials: T
authors.lastname: Eldar-Geva
authors.forename: Matityahu
authors.initials: M
authors.lastname: Erlichman
authors.forename: Yehuda
authors.initials: Y
authors.lastname: Pollak
authors.forename: Varda
authors.initials: V
authors.lastname: Gross-Tsur
mesh_terms: D000375:Aging / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D012739:Gonadal Steroid Hormones / Q000378:metabolism; D006801:Humans; D007006:Hypogonadism / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011218:Prader-Willi Syndrome / Q000150:complications*; D011629:Puberty, Precocious; D012727:Sex Characteristics
chemical_list: D012739:Gonadal Steroid Hormones