scholarly journals Three years of growth hormone treatment in young adults with Prader-Willi syndrome: sustained positive effects on body composition

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Layla Damen ◽  
Stephany H. Donze ◽  
Renske J. Kuppens ◽  
Nienke E. Bakker ◽  
Laura C. G. de Graaff ◽  
...  
2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Harry J. Hirsch ◽  
Varda Gross-Tsur

AbstractGrowth hormone treatment for children with Prader Willi syndrome (PWS) has shown proven benefits not only in increasing final height but also with positive effects on body composition and motor development. In a recent letter to the editor, Hoybye and colleagues recommend growth hormone treatment for adults with PWS based exclusively on the genetic diagnosis and without regard for growth hormone secretory status. We question whether the benefits of growth hormone treatment in PWS adults, mainly improvement in body composition, are significant enough to justify the as yet unkown consequences of long-term treatment in an adult population. Morbidity and mortality in PWS are mainly due to complications of obesity, and growth hormone treatment does not result in a decrease in BMI or waist circumference. Increases in insulin-like factor-1 as a result of growth hormone treatment over the course of several decades in PWS adults raises concern over possible increase risk of cancer. Compliance with daily injections is likely to be poor. We suggest that efforts to provide appropriate dietary and exercise regimens may be more beneficial and cost-effective than advocating for growth hormone treatment for adults with PWS.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Charlotte Höybye ◽  
◽  
Anthony J. Holland ◽  
Daniel J. Driscoll

AbstractPrader-Willi syndrome (PWS) is a complex, multi-system, neurodevelopmental disorder characterised by neonatal muscular hypotonia, short stature, high risk of obesity, hypogonadism, intellectual disabilities, distinct behavioural/psychiatric problems and abnormal body composition with increased body fat and a deficit of lean body mass. Growth hormone (GH) deficiency and other hormone deficiencies are common due to hypothalamic dysfunction. In children with PWS GH treatment has been widely demonstrated to improve body composition, normalise height and improve psychomotor development. In adults with PWS, GH’s main effects are to maintain normal body structure and metabolism. The positive effects of GH treatment on body composition, physical fitness and beneficial effects on cardiovascular risk markers, behaviour and quality of life in adults with PWS are also well established from several studies. GH treatment is approved for treatment of children with PWS in many countries, but until recently not as a treatment in young adults in the transition period or for adults in general. In this commentary we want to draw attention to the uneven global use of GH treatment, specifically in adults with PWS, and advocate for GH treatment to be approved internationally, not just for children, but also for adults with PWS and based only on the diagnosis of genetically confirmed PWS.


1997 ◽  
Vol 86 (S423) ◽  
pp. 60-62 ◽  
Author(s):  
AC Lindgren ◽  
L. Hagenäs ◽  
J. Müller ◽  
S. Blichfeldt ◽  
M. Rosenborg ◽  
...  

2004 ◽  
Vol 39 (Supplement 1) ◽  
pp. S28-S29
Author(s):  
A. O. Scheimann ◽  
W. J. Klish ◽  
B. Hayslett ◽  
N. Lafuente ◽  
D. Orellana ◽  
...  

2013 ◽  
Vol 98 (10) ◽  
pp. 4013-4022 ◽  
Author(s):  
N. E. Bakker ◽  
R. J. Kuppens ◽  
E. P. C. Siemensma ◽  
R. F. A. Tummers-de Lind van Wijngaarden ◽  
D. A. M. Festen ◽  
...  

2007 ◽  
Vol 87 (1) ◽  
pp. 28-31 ◽  
Author(s):  
AC Lindgren ◽  
L Hagenäs ◽  
J Müller ◽  
S Blichfeldt ◽  
M Rosenborg ◽  
...  

2003 ◽  
Vol 58 (5) ◽  
pp. 653-661 ◽  
Author(s):  
Charlotte Höybye ◽  
Agneta Hilding ◽  
Hans Jacobsson ◽  
Marja Thorén

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