The FSH-inhibin axis in prader-willi syndrome: heterogeneity of gonadal dysfunction.

Reproductive Biology and Endocrinology : RB&E
Varda Gross-TsurTalia Eldar-Geva

Abstract

We characterized the spectrum and etiology of hypogonadism in a cohort of Prader-Willi syndrome (PWS) adolescents and adults. Reproductive hormonal profiles and physical examination were performed on 19 males and 16 females ages 16-34 years with PWS. Gonadotropins, sex-steroids, inhibin B (INB) and anti-Mullerian hormone (AMH) were measured. We defined 4 groups according to the relative contribution of central and gonadal dysfunction based on FSH and INB levels: Group A: primary hypogonadism (FSH >15 IU/l and undetectable INB (<10 pg/ml); Group B: central hypogonadism (FSH <0.5 IU/l, INB <10 pg/ml); Group C: partial gonadal & central dysfunction (FSH 1.5-15 IU/l, INB >20 pg/ml); Group D: mild central and severe gonadal dysfunction (FSH 1.5-15 IU/l, INB < 10 pg/ml. There were 10, 8, 9 and 8 individuals in Groups A-D respectively; significantly more males in group A (9, 4, 4 and 2; P = 0.04). Significant differences between the groups were found in mean testosterone (P = 0.04), AMH (P = 0.003) and pubic hair (P = 0.04) in males and mean LH (P = 0.003) and breast development (P = 0.04) in females. Mean age, height, weight, BMI and the distribution of genetic subtypes were similar within the groups. Analysis of FSH and inhibin B re...Continue Reading

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Citations

Sep 17, 2013·American Journal of Medical Genetics. Part a·Talia Eldar-GevaVarda Gross-Tsur
Jan 11, 2016·Clinical Anatomy : Official Journal of the American Association of Clinical Anatomists & the British Association of Clinical Anatomists·Bradley J Hurren, Natasha A M S Flack
May 3, 2016·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Manuel NistalMiguel Reyes-Múgica
Jul 14, 2018·Molecular Genetics & Genomic Medicine·Talia Eldar-GevaRachel Eiges
Jul 25, 2019·Current Pediatric Reviews·Merlin G ButlerJanice L Forster
Sep 23, 2020·Open Biology·Michael S ChungGordon G Carmichael
Mar 1, 2019·Endocrinology, Diabetes & Metabolism·Satoko MatsuyamaAtsushi Mizokami
May 9, 2019·Reviews in Endocrine & Metabolic Disorders·Giovanna MuscogiuriUNKNOWN RESTARE
May 26, 2017·European Psychiatry : the Journal of the Association of European Psychiatrists·L Shriki-TalF Benarroch
May 2, 2019·Journal of the Endocrine Society·Luciana Pinto ValadaresAdriana Lofrano-Porto
Mar 7, 2021·International Journal of Molecular Sciences·Luigi NapolitanoMassimiliano Creta
Apr 4, 2021·International Journal of Molecular Sciences·Cees NoordamUrs Eiholzer
Jul 25, 2021·Journal of Clinical Medicine·Agnieszka Lecka-Ambroziak On Behalf Of The Polish Coordination Group For rhGH Treatment
Sep 25, 2021·Seminars in Reproductive Medicine·Aviva B SopherSelma F Witchel

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Methods Mentioned

BETA
hormone replacement therapy
ELISAs
contraception

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