Abstract
Prader-Willi syndrome (PWS) is characterized by hyperphagia, obesity if food intake is not strictly controlled, abnormal body composition with decreased lean body mass and increased fat mass, decreased basal metabolic rate, short stature, low muscle tone, cognitive disability, and hypogonadism. In addition to improvements in linear growth, the benefits of growth hormone therapy on body composition and motor function in children with PWS are well established. Evidence is now emerging on the benefits of growth hormone therapy in adults with PWS. This review summarizes the current literature on growth hormone status and the use of growth hormone therapy in adults with PWS. The benefits of growth hormone therapy on body composition, muscle strength, exercise capacity, certain measures of sleep-disordered breathing, metabolic parameters, quality of life, and cognition are covered in detail along with potential adverse effects and guidelines for initiating and monitoring therapy.
Highlights
Prader-Willi syndrome (PWS) is a complex genetic disorder caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13
Current evidence supports that adults with PWS have lower baseline insulin-like growth factor 1 (IGF-1) levels and a diminished response to growth hormone (GH) provocative testing when compared to obese controls
Depending on the cohort studied and the provocative agents used, 8%–55% of adults with PWS met criteria for severe GH deficiency based on body mass index (BMI) specific cut-offs
Summary
Prader-Willi syndrome (PWS) is a complex genetic disorder caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13. Clinical manifestations include hyperphagia, obesity if food intake is not strictly controlled, decreased basal metabolic rate, sleepdisordered breathing, cognitive disability, short stature, and hypogonadism. The benefits of recombinant growth hormone therapy (hGH) on body composition and motor function, in addition to linear growth, in children with PWS are well established [2,3]. Evidence is emerging on the benefits of growth hormone therapy in adults with PWS as well. We discuss the benefits of growth hormone therapy on body composition, muscle strength, exercise capacity, certain measures of sleep-disordered breathing, metabolic parameters, quality of life, and cognition. We discuss potential adverse effects and guidelines for initiating and monitoring therapy
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