Review of growth hormone therapy in adolescents and young adults with Prader–Willi syndrome

Lucas‐Herald, Angela K; Perry, Colin; Shaikh, M Guftar

doi:10.1586/17446651.2015.1007126

Cited by 3 publications

(3 citation statements)

References 66 publications

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“…Höybye (2004) suggested that GH treatment should be combined with lifestyle changes and exercise, in order to reduce the negative outcomes on glucose tolerance 30 . This further supports previous recommendations that transition to adult services should be supported by a multidisciplinary team, including a dietitian 13 …”

Section: Discussionsupporting

confidence: 54%

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The use of growth hormone therapy in adults with Prader‐Willi syndrome: A systematic review

Frixou

Vlek

Lucas‐Herald

et al. 2020

Clinical Endocrinology

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Objective Despite clear benefits in the management of children with Prader–Willi syndrome (PWS), the role of growth hormone (GH) in adults is unclear. The aim of this study was to conduct a systematic review to evaluate the effects of GH on body composition, bone health and cardiovascular health in adults with PWS. Design A systematic computerized literature search of the PubMed database was conducted by two independent reviewers. Inclusion criteria were individuals over the age of 16 years with a genetic diagnosis of PWS who had received GH therapy, together with assessment of body composition, bone health or cardiovascular health. Results Twenty full‐text papers met the inclusion criteria, encompassing 364 unique patients. No differences in body mass index (BMI) were noted, although 2 studies reported increased BMI after GH cessation. Data demonstrated statistically significant increases in lean body mass and reductions in percentage fat mass. Studies reported inconsistent effects of GH on cholesterol and echocardiography parameters. No studies reported differences in bone mineral density, although one reported improved bone geometry. Minor adverse events including pretibial oedema, headache and transient impaired glucose tolerance were reported in 7 studies. Conclusions These data suggest that GH is safe and well tolerated in adults with PWS, with evidence of improvement in body composition. Further longitudinal studies are still required to investigate the effects of GH on bone and cardiovascular health. Where GH is used in adults with PWS, this should be managed by a specialist multidisciplinary team with regular monitoring initiated.

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Section: Discussionsupporting

confidence: 54%

“…A meta‐analysis published in 2012 demonstrated that GH may improve body composition in adults with PWS 12 . In addition, a previous review by our group suggested that continuous GH use is beneficial in adolescents 13 . However, currently GH use in adulthood is not licensed unless there is confirmed GHD.…”

Section: Introductionmentioning

confidence: 99%

The use of growth hormone therapy in adults with Prader‐Willi syndrome: A systematic review

Frixou

Vlek

Lucas‐Herald

et al. 2020

Clinical Endocrinology

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“…From the age of two years the clinical features are dominated by increasing weight gain, hyperphagia, and an insatiable appetite. Other features include short stature, learning difficulties, dysmorphic facial features, low lean body mass, hypogonadism, and sleep and behavioural disturbance [ 2 , 7 ]. The endocrine features of PWS are well recognised and can include Growth Hormone Deficiency (GHD) or insufficiency, cryptorchidism and hypogonadism, hypothyroidism, impaired glucose tolerance and diabetes mellitus [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Pituitary-Adrenal Axis in Prader Willi Syndrome

2016

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Purpose: Prader Willi syndrome (PWS) is a rare genetic condition that has concurrent endocrinological insufficiencies. The presence of growth hormone deficiency has been well documented, but adrenal insufficiency (AI) is not widely reported. A review was conducted to investigate its prevalence and relevance in PWS in both adults and children. Methodology: A literature review was performed with the search terms “Prader-Willi syndrome” and “adrenal insufficiency”. Results: The review found studies disagree on the prevalence and method of investigation of AI in PWS. Case studies demonstrate that patients with PWS are at risk of premature death, often secondary to respiratory infections. The possibility that this may be the result of the inability to mount an effective cortisol response has been studied, with some evidence confirming AI in PWS patients. Most reports agreed AI is present in PWS, however, Farholt et al. showed no HPA axis dysfunction in adults, suggesting that perhaps it is rare in adults, and children should be the focus of further studies. Conclusion: AI is present in some patients with PWS. Further research is required to ensure optimal treatment can be implemented and to prevent premature deaths related to adrenal insufficiency. Clinicians should have a low threshold for testing the adrenal axis and considering treatment for adrenal insufficiency in PWS patients.

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Laboratory evaluation of short stature in children

Winter

2021

Handbook of Diagnostic Endocrinology

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Review of growth hormone therapy in adolescents and young adults with Prader–Willi syndrome

Cited by 3 publications

References 66 publications

The use of growth hormone therapy in adults with Prader‐Willi syndrome: A systematic review

The use of growth hormone therapy in adults with Prader‐Willi syndrome: A systematic review

Pituitary-Adrenal Axis in Prader Willi Syndrome

Laboratory evaluation of short stature in children

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