The role of hypogonadism in Klinefelter Syndrome
Klinefelter syndrome (KS) (47, XXY) is the most abundant sex-chromosome disorder, and is a common cause of infertility and hypogonadism in men. Most men with KS go through life without knowing the diagnosis, as only 25% are diagnosed and only a few of these before puberty. Apart from hypogonadism an...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2014-04-01
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| Series: | Asian Journal of Andrology |
| Subjects: | |
| Online Access: | http://www.ajandrology.com/article.asp?issn=1008-682X;year=2014;volume=16;issue=2;spage=185;epage=191;aulast=H%F8st |
| _version_ | 1811277375771508736 |
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| author | Christian Høst Anne Skakkeb'k Kristian A Groth Anders Bojesen |
| author_facet | Christian Høst Anne Skakkeb'k Kristian A Groth Anders Bojesen |
| author_sort | Christian Høst |
| collection | DOAJ |
| description | Klinefelter syndrome (KS) (47, XXY) is the most abundant sex-chromosome disorder, and is a common cause of infertility and hypogonadism in men. Most men with KS go through life without knowing the diagnosis, as only 25% are diagnosed and only a few of these before puberty. Apart from hypogonadism and azoospermia, most men with KS suffer from some degree of learning disability and may have various kinds of psychiatric problems. The effects of long-term hypogonadism may be diffi cult to discern from the gene dose effect of the extra X-chromosome. Whatever the cause, alterations in body composition, with more fat and less muscle mass and diminished bone mineral mass, as well as increased risk of metabolic consequences, such as type 2 diabetes and the metabolic syndrome are all common in KS. These fi ndings should be a concern as they are not simply laboratory fi ndings; epidemiological studies in KS populations show an increased risk of both hospitalization and death from various diseases. Testosterone treatment should be offered to KS patients from early puberty, to secure a proper masculine development, nonetheless the evidence is weak or nonexisting, since no randomized controlled trials have ever been published. Here, we will review the current knowledge of hypogonadism in KS and the rationale for testosterone treatment and try to give our best recommendations for surveillance of this rather common, but often ignored, syndrome. |
| first_indexed | 2024-04-13T00:14:31Z |
| format | Article |
| id | doaj.art-40b6f7a997634222a8740edb12e4ca7d |
| institution | Directory Open Access Journal |
| issn | 1008-682X 1745-7262 |
| language | English |
| last_indexed | 2024-04-13T00:14:31Z |
| publishDate | 2014-04-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Asian Journal of Andrology |
| spelling | doaj.art-40b6f7a997634222a8740edb12e4ca7d2022-12-22T03:10:58ZengWolters Kluwer Medknow PublicationsAsian Journal of Andrology1008-682X1745-72622014-04-0116218519110.4103/1008-682X.122201The role of hypogonadism in Klinefelter SyndromeChristian HøstAnne Skakkeb'kKristian A GrothAnders BojesenKlinefelter syndrome (KS) (47, XXY) is the most abundant sex-chromosome disorder, and is a common cause of infertility and hypogonadism in men. Most men with KS go through life without knowing the diagnosis, as only 25% are diagnosed and only a few of these before puberty. Apart from hypogonadism and azoospermia, most men with KS suffer from some degree of learning disability and may have various kinds of psychiatric problems. The effects of long-term hypogonadism may be diffi cult to discern from the gene dose effect of the extra X-chromosome. Whatever the cause, alterations in body composition, with more fat and less muscle mass and diminished bone mineral mass, as well as increased risk of metabolic consequences, such as type 2 diabetes and the metabolic syndrome are all common in KS. These fi ndings should be a concern as they are not simply laboratory fi ndings; epidemiological studies in KS populations show an increased risk of both hospitalization and death from various diseases. Testosterone treatment should be offered to KS patients from early puberty, to secure a proper masculine development, nonetheless the evidence is weak or nonexisting, since no randomized controlled trials have ever been published. Here, we will review the current knowledge of hypogonadism in KS and the rationale for testosterone treatment and try to give our best recommendations for surveillance of this rather common, but often ignored, syndrome.http://www.ajandrology.com/article.asp?issn=1008-682X;year=2014;volume=16;issue=2;spage=185;epage=191;aulast=H%F8standrogen receptorbody compositionbone densityhypogonadisminfertilityKlinefelter syndromelearning disordersmaletestosterone |
| spellingShingle | Christian Høst Anne Skakkeb'k Kristian A Groth Anders Bojesen The role of hypogonadism in Klinefelter Syndrome Asian Journal of Andrology androgen receptor body composition bone density hypogonadism infertility Klinefelter syndrome learning disorders male testosterone |
| title | The role of hypogonadism in Klinefelter Syndrome |
| title_full | The role of hypogonadism in Klinefelter Syndrome |
| title_fullStr | The role of hypogonadism in Klinefelter Syndrome |
| title_full_unstemmed | The role of hypogonadism in Klinefelter Syndrome |
| title_short | The role of hypogonadism in Klinefelter Syndrome |
| title_sort | role of hypogonadism in klinefelter syndrome |
| topic | androgen receptor body composition bone density hypogonadism infertility Klinefelter syndrome learning disorders male testosterone |
| url | http://www.ajandrology.com/article.asp?issn=1008-682X;year=2014;volume=16;issue=2;spage=185;epage=191;aulast=H%F8st |
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