Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review
Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by various clinical features linked to hypothalamic/pituitary gland abnormalities. Growth hormone deficiency is a prominent feature of PWS that results in poor linear growth and delayed development. This review discusses the evalua...
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| Format: | Article |
| Language: | English |
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Sungkyunkwan University School of Medi
2023-09-01
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| Series: | Precision and Future Medicine |
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| Online Access: | http://www.pfmjournal.org/upload/pdf/pfm-2023-00079.pdf |
| _version_ | 1797653486534393856 |
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| author | Aram Yang |
| author_facet | Aram Yang |
| author_sort | Aram Yang |
| collection | DOAJ |
| description | Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by various clinical features linked to hypothalamic/pituitary gland abnormalities. Growth hormone deficiency is a prominent feature of PWS that results in poor linear growth and delayed development. This review discusses the evaluation and effects of growth hormone therapy (GHT) in PWS. Heterogeneity in growth hormone secretion patterns based on genotype and the potential for personalized GHT were explored. The benefits of GHT, including improvements in motor and cognitive development, growth, and body composition, are discussed in detail. Safety considerations for GHT initiation and response to GHT in adults with PWS are discussed, along with ongoing debates regarding the efficacy and safety. Although controversies persist, an evolving understanding of the long-term effects and safety of GHT underscores the need for comprehensive research in this field. |
| first_indexed | 2024-03-11T16:45:16Z |
| format | Article |
| id | doaj.art-61fc11b1db534528998a64f25a081b30 |
| institution | Directory Open Access Journal |
| issn | 2508-7940 2508-7959 |
| language | English |
| last_indexed | 2024-03-11T16:45:16Z |
| publishDate | 2023-09-01 |
| publisher | Sungkyunkwan University School of Medi |
| record_format | Article |
| series | Precision and Future Medicine |
| spelling | doaj.art-61fc11b1db534528998a64f25a081b302023-10-23T01:13:53ZengSungkyunkwan University School of MediPrecision and Future Medicine2508-79402508-79592023-09-017310711610.23838/pfm.2023.00079149Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A reviewAram Yang0 Department of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, KoreaPrader-Willi syndrome (PWS) is a rare genetic disorder characterized by various clinical features linked to hypothalamic/pituitary gland abnormalities. Growth hormone deficiency is a prominent feature of PWS that results in poor linear growth and delayed development. This review discusses the evaluation and effects of growth hormone therapy (GHT) in PWS. Heterogeneity in growth hormone secretion patterns based on genotype and the potential for personalized GHT were explored. The benefits of GHT, including improvements in motor and cognitive development, growth, and body composition, are discussed in detail. Safety considerations for GHT initiation and response to GHT in adults with PWS are discussed, along with ongoing debates regarding the efficacy and safety. Although controversies persist, an evolving understanding of the long-term effects and safety of GHT underscores the need for comprehensive research in this field.http://www.pfmjournal.org/upload/pdf/pfm-2023-00079.pdfgrowth hormonehuman growth hormonehypothalamuspituitary glandprader-willi syndrome |
| spellingShingle | Aram Yang Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review Precision and Future Medicine growth hormone human growth hormone hypothalamus pituitary gland prader-willi syndrome |
| title | Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review |
| title_full | Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review |
| title_fullStr | Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review |
| title_full_unstemmed | Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review |
| title_short | Prader-Willi syndrome and growth hormone therapy: exploring the precise management of hypothalamic short stature: A review |
| title_sort | prader willi syndrome and growth hormone therapy exploring the precise management of hypothalamic short stature a review |
| topic | growth hormone human growth hormone hypothalamus pituitary gland prader-willi syndrome |
| url | http://www.pfmjournal.org/upload/pdf/pfm-2023-00079.pdf |
| work_keys_str_mv | AT aramyang praderwillisyndromeandgrowthhormonetherapyexploringtheprecisemanagementofhypothalamicshortstatureareview |