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

Prader–Willi syndrome (PWS) is a complex genetic syndrome characterized by hyper-phagia, 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... Mehr ...

Verfasser: Pellikaan, Karlijn
Brahim, Yassine Ben
Rosenberg, Anna G.W.
Davidse, Kirsten
Poitou, Christine
Coupaye, Muriel
Goldstone, Anthony P.
Høybye, Charlotte
Markovic, Tania P.
Grugni, Graziano
Crinò, Antonino
Caixàs, Assumpta
Eldar-Geva, Talia
Hirsch, Harry J.
Gross-Tsur, Varda
Butler, Merlin G.
Miller, Jennifer L.
van den Berg, Sjoerd A.A.
van der Lely, Aart J.
de Graaff, Laura C.G.
Dokumenttyp: Artikel
Erscheinungsdatum: 2021
Reihe/Periodikum: Pellikaan , K , Brahim , Y B , Rosenberg , A G W , Davidse , K , Poitou , C , Coupaye , M , Goldstone , A P , Høybye , C , Markovic , T P , Grugni , G , Crinò , A , Caixàs , A , Eldar-Geva , T , Hirsch , H J , Gross-Tsur , V , Butler , M G , Miller , J L , van den Berg , S A A , van der Lely , A J & de Graaff , L C G 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 ' , Journal of Clinical Medicine , vol. 10 , no. 19 , 4361 . https://doi.org/10.3390/jcm10194361
Schlagwörter: /dk/atira/pure/sustainabledevelopmentgoals/good_health_and_well_being / SDG 3 - Good Health and Well-being
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-27073069
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://pure.eur.nl/en/publications/247328b8-6fc0-4b28-9488-fa5141bc85f7

Prader–Willi syndrome (PWS) is a complex genetic syndrome characterized by hyper-phagia, 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.