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 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 i...

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Authors: Pellikaan, Karlijn|||0000-0002-0738-0275, Ben Brahim, Yassine, Rosenberg, Anna G. W|||0000-0002-8333-1512, Davidse, Kirsten, Poitou, Christine|||0000-0001-7769-6331, Coupaye, Muriel|||0000-0002-8988-6260, Goldstone, Anthony|||0000-0001-8179-7071, Høybye, Charlotte|||0000-0002-3980-1927, Markovic, Tania|||0000-0003-2521-0357, Grugni, Graziano|||0000-0003-4708-2544, Crinò, Antonino|||0000-0003-3548-7114, Caixàs i Pedragós, Assumpta|||0000-0001-8472-9189, Eldar-Geva, Talia, Hirsch, Harry|||0000-0002-7499-4023, Gross-Tsur, Varda, Butler, Merlin|||0000-0002-2911-0524, Miller, Jennifer L., van den Berg, Sjoerd A. A., van der Lely, Aart J|||0000-0002-1059-0126, de Graaff, Laura|||0000-0002-0295-7063
Format: article
Publication Date:2021
Country:España
Institution:Universitat Autònoma de Barcelona
Repository:Dipòsit Digital de Documents de la UAB
Language:English
OAI Identifier:oai:ddd.uab.cat:283308
Online Access:https://ddd.uab.cat/record/283308
https://dx.doi.org/urn:doi:10.3390/jcm10194361
Access Level:Open access
Keyword:Hypogonadism
Obesity
Pituitary gland
Prader-Willi syndrome
Puberty
Testosterone
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spelling 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 DiscussionPellikaan, Karlijn|||0000-0002-0738-0275Ben Brahim, YassineRosenberg, Anna G. W|||0000-0002-8333-1512Davidse, KirstenPoitou, Christine|||0000-0001-7769-6331Coupaye, Muriel|||0000-0002-8988-6260Goldstone, Anthony|||0000-0001-8179-7071Høybye, Charlotte|||0000-0002-3980-1927Markovic, Tania|||0000-0003-2521-0357Grugni, Graziano|||0000-0003-4708-2544Crinò, Antonino|||0000-0003-3548-7114Caixàs i Pedragós, Assumpta|||0000-0001-8472-9189Eldar-Geva, TaliaHirsch, Harry|||0000-0002-7499-4023Gross-Tsur, VardaButler, Merlin|||0000-0002-2911-0524Miller, Jennifer L.van den Berg, Sjoerd A. A.van der Lely, Aart J|||0000-0002-1059-0126de Graaff, Laura|||0000-0002-0295-7063HypogonadismObesityPituitary glandPrader-Willi syndromePubertyTestosteronePrader-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.Universitat Autònoma de Barcelona 22021-01-0120212021-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/283308https://dx.doi.org/urn:doi:10.3390/jcm10194361reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest 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.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:2833082026-06-06T12:50:31Z
dc.title.none.fl_str_mv 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
title 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
spellingShingle 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|||0000-0002-0738-0275
Hypogonadism
Obesity
Pituitary gland
Prader-Willi syndrome
Puberty
Testosterone
title_short 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_sort 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
dc.creator.none.fl_str_mv Pellikaan, Karlijn|||0000-0002-0738-0275
Ben Brahim, Yassine
Rosenberg, Anna G. W|||0000-0002-8333-1512
Davidse, Kirsten
Poitou, Christine|||0000-0001-7769-6331
Coupaye, Muriel|||0000-0002-8988-6260
Goldstone, Anthony|||0000-0001-8179-7071
Høybye, Charlotte|||0000-0002-3980-1927
Markovic, Tania|||0000-0003-2521-0357
Grugni, Graziano|||0000-0003-4708-2544
Crinò, Antonino|||0000-0003-3548-7114
Caixàs i Pedragós, Assumpta|||0000-0001-8472-9189
Eldar-Geva, Talia
Hirsch, Harry|||0000-0002-7499-4023
Gross-Tsur, Varda
Butler, Merlin|||0000-0002-2911-0524
Miller, Jennifer L.
van den Berg, Sjoerd A. A.
van der Lely, Aart J|||0000-0002-1059-0126
de Graaff, Laura|||0000-0002-0295-7063
author Pellikaan, Karlijn|||0000-0002-0738-0275
author_facet Pellikaan, Karlijn|||0000-0002-0738-0275
Ben Brahim, Yassine
Rosenberg, Anna G. W|||0000-0002-8333-1512
Davidse, Kirsten
Poitou, Christine|||0000-0001-7769-6331
Coupaye, Muriel|||0000-0002-8988-6260
Goldstone, Anthony|||0000-0001-8179-7071
Høybye, Charlotte|||0000-0002-3980-1927
Markovic, Tania|||0000-0003-2521-0357
Grugni, Graziano|||0000-0003-4708-2544
Crinò, Antonino|||0000-0003-3548-7114
Caixàs i Pedragós, Assumpta|||0000-0001-8472-9189
Eldar-Geva, Talia
Hirsch, Harry|||0000-0002-7499-4023
Gross-Tsur, Varda
Butler, Merlin|||0000-0002-2911-0524
Miller, Jennifer L.
van den Berg, Sjoerd A. A.
van der Lely, Aart J|||0000-0002-1059-0126
de Graaff, Laura|||0000-0002-0295-7063
author_role author
author2 Ben Brahim, Yassine
Rosenberg, Anna G. W|||0000-0002-8333-1512
Davidse, Kirsten
Poitou, Christine|||0000-0001-7769-6331
Coupaye, Muriel|||0000-0002-8988-6260
Goldstone, Anthony|||0000-0001-8179-7071
Høybye, Charlotte|||0000-0002-3980-1927
Markovic, Tania|||0000-0003-2521-0357
Grugni, Graziano|||0000-0003-4708-2544
Crinò, Antonino|||0000-0003-3548-7114
Caixàs i Pedragós, Assumpta|||0000-0001-8472-9189
Eldar-Geva, Talia
Hirsch, Harry|||0000-0002-7499-4023
Gross-Tsur, Varda
Butler, Merlin|||0000-0002-2911-0524
Miller, Jennifer L.
van den Berg, Sjoerd A. A.
van der Lely, Aart J|||0000-0002-1059-0126
de Graaff, Laura|||0000-0002-0295-7063
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Universitat Autònoma de Barcelona
dc.subject.none.fl_str_mv Hypogonadism
Obesity
Pituitary gland
Prader-Willi syndrome
Puberty
Testosterone
topic Hypogonadism
Obesity
Pituitary gland
Prader-Willi syndrome
Puberty
Testosterone
description 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.
publishDate 2021
dc.date.none.fl_str_mv 2
2021-01-01
2021
2021-01-01
dc.type.none.fl_str_mv Article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://ddd.uab.cat/record/283308
https://dx.doi.org/urn:doi:10.3390/jcm10194361
url https://ddd.uab.cat/record/283308
https://dx.doi.org/urn:doi:10.3390/jcm10194361
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Dipòsit Digital de Documents de la UAB
instname:Universitat Autònoma de Barcelona
instname_str Universitat Autònoma de Barcelona
reponame_str Dipòsit Digital de Documents de la UAB
collection Dipòsit Digital de Documents de la UAB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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