Growth hormone treatment of Prader-Willi syndrome has long-term, positive effects on body composition

2015 ◽  
Vol 104 (4) ◽  
pp. 422-427 ◽  
Author(s):  
Charlotte Höybye
2004 ◽  
Vol 39 (Supplement 1) ◽  
pp. S28-S29
Author(s):  
A. O. Scheimann ◽  
W. J. Klish ◽  
B. Hayslett ◽  
N. Lafuente ◽  
D. Orellana ◽  
...  

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 ◽  
...  

2020 ◽  
Vol 182 (4) ◽  
pp. 405-411
Author(s):  
S H Donze ◽  
L Damen ◽  
E F Mahabier ◽  
A C S Hokken-Koelega

Objective Children with Prader–Willi syndrome (PWS) have mild to moderate cognitive impairment. Short-term studies showed positive effects of growth hormone (GH) on cognitive development. This study investigated the effects of 8 years of GH on cognitive development in children with PWS. We also investigated whether starting GH during infancy results in higher cognitive functioning after 8 years of GH. Design Longitudinal study in 43 children with PWS during 8 years of GH (median age at GH start 8.1 years). Cognitive functioning after 8 years was compared to another group of 22 children with PWS (median age at GH start 1.4 years). Methods Cognitive functioning was measured by Wechsler Intelligence Scale for Children. Vocabulary, Similarities and Block Design subtests were expressed as standard deviation scores (SDS) and total IQ (TIQ) calculated. Results Estimated mean (95%CI) Block Design SDS changed from −2.2 (−2.6; −1.8) at GH start to −1.8 (−2.2; −1.4) after 8 years of GH (P = 0.18), similarly SDS from −1.5 (−2.1; −0.9) to −1.3 (−1.9; −0.7, P = 0.66) and TIQ from 66 (60; 72) to 69 (63; 75, P = 0.57). Vocabulary SDS remained similar, being −1.9 (−2.3; −1.4) at GH start and −1.9 (−2.4; −1.5) after 8 years (P = 0.85). After 8 years of GH Vocabulary, SDS and TIQ were higher in the children who started GH during infancy, compared to those who started GH later in childhood (P < 0.01, P = 0.04, respectively). Conclusions Cognitive functioning in children with PWS remains similar during long-term GH and develops at the same pace as healthy peers.


Author(s):  
Sin T. Lo ◽  
Dederieke A. M. Festen ◽  
Roderick F. A. Tummers-de Lind van Wijngaarden ◽  
Philippe J. L. Collin ◽  
Anita C. S. Hokken-Koelega

Abstract The aim of this study was to investigate the effect of growth hormone treatment on adaptive functioning in children with Prader-Willi syndrome. Vineland Adaptive Behavior Scale (VABS) was assessed during a randomized controlled trial (RCT) and after 7 years of growth hormone treatment. In the RCT, 75 children (42 infants and 33 prepubertal children) with Prader-Willi syndrome were included. Subsequently, 53 children were treated with long-term growth hormone. Our study demonstrates a marked delay in adaptive functioning in infants and children with Prader-Willi syndrome, which was associated with older age and lower intelligence. Results of the repeated measurements show that the earlier growth hormone treatment was started during infancy, the better the adaptive skills were on the long-term.


2009 ◽  
Vol 94 (11) ◽  
pp. 4205-4215 ◽  
Author(s):  
Roderick F. A. de Lind van Wijngaarden ◽  
Elbrich P. C. Siemensma ◽  
Dederieke A. M. Festen ◽  
Barto J. Otten ◽  
Edgar G. A. H. van Mil ◽  
...  

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 ◽  
...  

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