Article Dans Une Revue Journal of Clinical Medicine Année : 2021

Hypogonadism in Adult Males with Prader-Willi Syndrome—Clinical Recommendations Based on a Dutch Cohort Study, Review of the Literature and an International Expert Panel Discussion

Karlijn Pellikaan
Yassine Ben Brahim
  • Fonction : Auteur
Anna Rosenberg
Kirsten Davidse
  • Fonction : Auteur
Muriel Coupaye
Anthony Goldstone
Charlotte Høybye
Tania Markovic
Graziano Grugni
Antonino Crinò
  • Fonction : Auteur
Assumpta Caixàs
Talia Eldar-Geva
  • Fonction : Auteur
Harry Hirsch
Varda Gross-Tsur
  • Fonction : Auteur
Merlin Butler
Jennifer Miller
  • Fonction : Auteur
Sjoerd van den Berg
  • Fonction : Auteur
Aart van der Lely
Laura de Graaff
  • Fonction : Auteur

Résumé

Prader-Willi syndrome (PWS) is a complex genetic syndrome characterized by hyperphagia, intellectual disability, hypotonia and hypothalamic dysfunction. Adults with PWS often have hormone deficiencies, hypogonadism being the most common. Untreated male hypogonadism can aggravate PWS-related health issues including muscle weakness, obesity, osteoporosis, and fatigue. Therefore, timely diagnosis and treatment of male hypogonadism is important. In this article, we share our experience with hypogonadism and its treatment in adult males with PWS and present a review of the literature. In order to report the prevalence and type of hypogonadism, treatment regimen and behavioral issues, we retrospectively collected data on medical interviews, physical examinations, biochemical measurements and testosterone replacement therapy (TRT) in 57 Dutch men with PWS. Fifty-six (98%) of the patients had either primary, central or combined hypogonadism. Untreated hypogonadism was associated with higher body mass index and lower hemoglobin concentrations. TRT was complicated by behavioral challenges in one third of the patients. Undertreatment was common and normal serum testosterone levels were achieved in only 30% of the patients. Based on the Dutch cohort data, review of the literature and an international expert panel discussion, we provide a practical algorithm for TRT in adult males with PWS in order to prevent undertreatment and related adverse health outcomes.

Dates et versions

hal-04011917 , version 1 (02-03-2023)

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Citer

Karlijn Pellikaan, Yassine Ben Brahim, Anna Rosenberg, Kirsten Davidse, Christine Poitou, et al.. Hypogonadism in Adult Males with Prader-Willi Syndrome—Clinical Recommendations Based on a Dutch Cohort Study, Review of the Literature and an International Expert Panel Discussion. Journal of Clinical Medicine, 2021, 10 (19), pp.4361. ⟨10.3390/jcm10194361⟩. ⟨hal-04011917⟩
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