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
Pellikaan, Karlijn (Erasmus University Medical Center Rotterdam)
Ben Brahim, Yassine (Erasmus University Medical Center Rotterdam)
Rosenberg, Anna G. W (Erasmus University Medical Center Rotterdam)
Davidse, Kirsten (Erasmus University Medical Center Rotterdam)
Poitou, Christine (European Reference Network)
Coupaye, Muriel (European Reference Network)
Goldstone, Anthony (Imperial College Healthcare National Health Service Trust (Londres, Regne Unit))
Høybye, Charlotte (Karolinska University Hospital and Karolinska Institutet (Suecia))
Markovic, Tania (University of Sydney, Sydney)
Grugni, Graziano (Istituto Auxologico Italiano)
Crinò, Antonino (Bambino Gesù Hospital (Palidoro, Itàlia))
Caixàs i Pedragós, Assumpta (Parc Taulí Hospital Universitari. Institut d'Investigació i Innovació Parc Taulí (I3PT))
Eldar-Geva, Talia (Hebrew University School of Medicine)
Hirsch, Harry (Shaare Zedek Medical Center)
Gross-Tsur, Varda (Shaare Zedek Medical Center)
Butler, Merlin (University of Kansas Medical Center)
Miller, Jennifer L. (University of Florida)
van den Berg, Sjoerd A. A. (University Medical Center Rotterdam)
van der Lely, Aart J (Erasmus University Medical Center Rotterdam)
de Graaff, Laura (European Reference Network)
Universitat Autònoma de Barcelona

Data:	2021
Resum:	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.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	Hypogonadism ; Obesity ; Pituitary gland ; Prader-Willi syndrome ; Puberty ; Testosterone
Publicat a:	Journal of clinical medicine, Vol. 10 (september 2021) , ISSN 2077-0383

DOI: 10.3390/jcm10194361
PMID: 34640379

28 p, 2.7 MB

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Documents de recerca > Documents dels grups de recerca de la UAB > Centres i grups de recerca (producció científica) > Ciències de la salut i biociències > Institut d’Investigació i Innovació Parc Taulí (I3PT)
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