Characterization of minipuberty in infants with prader-willi syndrome

Harry J. Hirsch*, Talia Eldar-Geva, Matityahu Erlichman, Yehuda Pollak, Varda Gross-Tsur

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

16 Scopus citations

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.

Objectives: 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.

Original languageEnglish
Pages (from-to)230-237
Number of pages8
JournalHormone Research in Paediatrics
Volume82
Issue number4
DOIs
StatePublished - 7 Nov 2014

Bibliographical note

Publisher Copyright:
© 2014 S. Karger AG, Basel.

Keywords

  • Infants
  • Minipuberty
  • Prader-willi syndrome

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