en POLSKI
eISSN: 2083-8441
ISSN: 2081-237X
Pediatric Endocrinology Diabetes and Metabolism
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SCImago Journal & Country Rank
 
1/2022
vol. 28
 
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abstract:
Review paper

Management of Prader-Labhart-Willi syndrome in children and in adults, with particular emphasis on the treatment with recombinant human growth hormone

Marta Drabik
1
,
Andrzej Lewiński
1, 2
,
Renata Stawerska
1, 3

1.
Department of Endocrinology and Metabolic Diseases, Polish Mother’s Memorial Hospital - Research Institute in Lodz, Poland
2.
Department of Endocrinology and Metabolic Diseases, Medical University of Lodz, Poland
3.
Department of Paediatric Endocrinology, Medical University of Lodz, Poland
Pediatr Endocrinol Diabetes Metab 2022; 28 (1): 64–74
Online publish date: 2022/03/03
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Introduction
Prader-Willi syndrome (PWS) is a genetically determined disease that manifests itself in a number of abnormalities resulting, among others, from dysfunction of the hypothalamic-pituitary system. Only integrated, multidisciplinary care gives patients the chance to significantly improve the quality of life and achieve a life expectancy that does not differ from the general population.

Aim
The aim of the study was to summarize the available literature on the management of patients suffering from PWS.

Conclusions
More and more reports based on clinical trials conducted around the world indicate the undeniable benefits of rhGH therapy in patients with PWS in childhood and after the end of growth period. They consist in improving the body composition, improving the lipid profile, increasing bone mineral density and improving the mental state and patients' quality of life.

keywords:

Prader-Willi syndrome, obesity, recombinant human growth hormone, metabolism, therapeutic program


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