Growth hormone use in prader-willi syndrome - Experience of a dedicated paediatric clinic

2017 ◽  
Author(s):  
Mikaela Frixou ◽  
Georgia Irene Neophytou ◽  
M. Guftar Shaikh ◽  
Andreas Kyriakou
2011 ◽  
Vol 14 (7) ◽  
pp. A328 ◽  
Author(s):  
M. Tony ◽  
M.M. Goetghebeur ◽  
H. Khoury ◽  
M. Wagner ◽  
C.L. Deal ◽  
...  

2021 ◽  
Vol 10 (15) ◽  
pp. 3250
Author(s):  
Karlijn Pellikaan ◽  
Anna G. W. Rosenberg ◽  
Kirsten Davidse ◽  
Anja A. Kattentidt-Mouravieva ◽  
Rogier Kersseboom ◽  
...  

Prader-Willi syndrome (PWS) is a complex hypothalamic disorder. Features of PWS include hyperphagia, hypotonia, intellectual disability, and pituitary hormone deficiencies. The combination of growth hormone treatment and multidisciplinary care (GHMDc) has greatly improved the health of children with PWS. Little is known about the effects of childhood GHMDc on health outcomes in adulthood. We retrospectively collected clinical data of 109 adults with PWS. Thirty-nine had received GHMDc during childhood and adolescence (GHMDc+ group) and sixty-three had never received growth hormone treatment (GHt) nor multidisciplinary care (GHMDc− group). Our systematic screening revealed fewer undetected health problems in the GHMDc+ group (10%) than in the GHMDc− group (84%). All health problems revealed in the GHMDc+ group had developed between the last visit to the paediatric and the first visit to the adult clinic and/or did not require treatment. Mean BMI and the prevalence of diabetes mellitus type 2 were significantly lower in the GHMDc+ group compared to the GHMDc− group. As all patients who received GHt were treated in a multidisciplinary setting, it is unknown which effects are the result of GHt and which are the result of multidisciplinary care. However, our data clearly show that the combination of both has beneficial effects. Therefore, we recommend continuing GHMDc after patients with PWS have reached adult age.


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.


2018 ◽  
Vol 39 ◽  
pp. 29-33 ◽  
Author(s):  
Merlin G. Butler ◽  
Waheeda Hossain ◽  
Maaz Hassan ◽  
Ann M. Manzardo

Author(s):  
Giménez-Palop Olga ◽  
Laia Casamitjana ◽  
Raquel Corripio ◽  
Pareja Rocío ◽  
Joan Carles Oliva ◽  
...  

2011 ◽  
Vol 79 (2) ◽  
pp. 238-243 ◽  
Author(s):  
Nidhi Maheshwari ◽  
Naveen K. Uli ◽  
Sumana Narasimhan ◽  
Leona Cuttler

2013 ◽  
Vol 23 (3) ◽  
pp. 81-87 ◽  
Author(s):  
M.G. Butler ◽  
B.K. Smith ◽  
J. Lee ◽  
C. Gibson ◽  
C. Schmoll ◽  
...  

2016 ◽  
Vol 60 (6) ◽  
pp. 596-600 ◽  
Author(s):  
Natasha G. Ludwig ◽  
Rafael F. Radaeli ◽  
Mariana M. X. Silva ◽  
Camila M. Romero ◽  
Alexandre J. F. Carrilho ◽  
...  

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