One Year of Growth Hormone Treatment in Adults with Prader-Willi Syndrome Improves Body Composition: Results from a Randomized, Placebo-Controlled Study

Sode-Carlsen, Rasmus; Farholt, Stense; Rabben, Kai Fr.; Bollerslev, Jens; Schreiner, Thomas; Jurik, Anne Grethe; Christiansen, Jens Sandahl; Höybye, Charlotte

doi:10.1210/jc.2010-0907

Cited by 56 publications

(104 citation statements)

References 30 publications

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“…47 Several studies have documented the safety and efficacy of GH treatment in adults with PWS on body composition and quality of life. 48,49 One study found that the beneficial effects of GH therapy were unrelated to the pretreatment GH or serum IGF-1 levels. 49 Concern about the possible contribution of GH administration to unexpected death has been raised by reported deaths of individuals within a few months of starting GH therapy.…”

Section: Hypogonadismmentioning

confidence: 99%

“…48,49 One study found that the beneficial effects of GH therapy were unrelated to the pretreatment GH or serum IGF-1 levels. 49 Concern about the possible contribution of GH administration to unexpected death has been raised by reported deaths of individuals within a few months of starting GH therapy. [50][51][52][53][54][55] The few reported deaths were mostly in obese individuals who had preexisting respiratory or cardiac disorders with evidence of upper airway obstruction and uncorrected tonsillar and adenoidal hypertrophy.…”

Section: Hypogonadismmentioning

confidence: 99%

“…46,48,49 The conventional adult dose of GH is 20-25% of the dose recommended in children. Replacement of sex hormones at puberty produces further development of secondary sexual characteristics (personal observation).…”

Section: Adolescence and Adulthoodmentioning

confidence: 99%

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Prader-Willi syndrome

Cassidy

Schwartz

Miller

et al. 2012

Genetics in Medicine

1,114

1,193

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Section: Hypogonadismmentioning

confidence: 99%

Section: Hypogonadismmentioning

confidence: 99%

See 1 more Smart Citation

Prader-Willi syndrome

Cassidy

Schwartz

Miller

et al. 2012

Genetics in Medicine

1,114

1,193

View full text Add to dashboard Cite

“…Thigh fat mass and muscle mass were measured in one study. Five studies performed standard 75 g oral glucose tolerance tests (OGTT) [16][17][18][19][20] .…”

Section: Clinical Studies Investigating the Efficacy And Safety Of Rhmentioning

confidence: 99%

“…Changes in exercise capacity in response to rhGH therapy were examined in two studies 17,21) . Sode-Carlsen et al 17) performed a 10-metre walk test and found improvement (faster walking) in 6/18 rhGH-treated patients, but also in 2/16 control patients, and a repeat standing test in which 6/18 rhGH-treated and 4/16 controls had poorer scores compared with baseline.…”

Section: ) Exercise Capacitymentioning

confidence: 99%

Growth Hormone Therapy in Adults with Prader-Willi Syndrome

Cho¹

2015

Journal of mucopolysaccharidosis and rare disease

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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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Development and implementation of electronic growth charts for infants with Prader–Willi syndrome

Rosenbloom

Butler

2012

American J of Med Genetics Pt A

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Pediatric growth charts are used to monitor children's growth and development, and assist in healthcare delivery and supervision. Researchers have developed syndrome‐specific growth charts for several disorders in which affected children grow differently than unaffected children. Typical standardized growth curves have been recently reported in non‐growth hormone treated infants with Prader–Willi syndrome (PWS) of both genders from birth to 36 months of age to support monitoring growth and nutritional status during infancy, and to guide growth hormone treatment and follow up. Electronic health record (EHR) systems serving pediatric populations can now incorporate interactive automated growth charts to assist the healthcare providers in clinical settings to monitor growth. The American Academy of Pediatrics has recommended that EHR systems incorporate syndrome‐specific growth charts where feasible. This manuscript describes the experiences at two medical centers at different stages of incorporating electronic growth charts into the medical records on infants with rare disorders, using PWS as an illustration. The absence of readily available and accessible syndrome‐specific standardized growth charts in other disorders causes challenges for EHR personnel and for healthcare providers who care for these children particularly in the growing electronic medical record environment. © 2012 Wiley Periodicals, Inc.

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One Year of Growth Hormone Treatment in Adults with Prader-Willi Syndrome Improves Body Composition: Results from a Randomized, Placebo-Controlled Study

Abstract: Unrelated to the GH-IGF-I levels at baseline, our results showed that long-term treatment with GH effectively improved body composition and represents a safe, potential treatment option, relieving some of the negative consequences of PWS.

Cited by 56 publications

References 30 publications

Prader-Willi syndrome

Prader-Willi syndrome

Growth Hormone Therapy in Adults with Prader-Willi Syndrome

Development and implementation of electronic growth charts for infants with Prader–Willi syndrome

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