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KMID : 1146120150010020049
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Journal of Mucopolysaccharidosis and Rare Diseases
2015 Volume.1 No. 2 p.49 ~ p.53
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Growth Hormone Therapy in Adults with Prader-Willi Syndrom
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Cho Sung-Yoon
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Abstract
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Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. Many features of PWS indicate a deficiency in growth hormone (GH) production, and these findings provide a rationale for GH therapy in PWS. It is possible that rhGH therapy could have beneficial effects in adults with PWS, similar to those in adults with GH deficiency (GHD) of non-syndromic cause. However, there is a paucity of data on the use of GH in adults with PWS. Here, the previous studies about efficacy and safety of rhGH therapy in PWS adults are summarized. Briefly, rhGH therapy in PWS adults may improve body composition, leading to increased lean body mass and decreased fat mass, as well as decreased subcutaneous and visceral adiposity without overall changes in body mass index. There may be at least transient deterioration in glucose homoeostasis in some PWS patients on rhGH therapy, which requires further study. In addition, clinical care guidelines for rhGH therapy in adults with PWS were suggested.
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KEYWORD
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Prader-Willi syndrome, Growth hormone deficiency, Growth hormone therapy
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