open access

Vol 69, No 4 (2018)
Guidelines
Submitted: 2018-08-21
Accepted: 2018-08-21
Get Citation

Management of Prader-Willi Syndrome (PWS) in adults — what an endocrinologist needs to know. Recommendations of the Polish Society of Endocrinology and the Polish Society of Paediatric Endocrinology and Diabetology

Magdalena Góralska, Tomasz Bednarczuk, Marek Rosłon, Maria Libura, Mieczysław Szalecki, Maciej Hilczer, Renata Stawerska, Joanna Smyczyńska, Małgorzata Karbownik-Lewińska, Mieczysław Walczak, Andrzej Lewiński
DOI: 10.5603/EP.2018.0047
·
Endokrynologia Polska 2018;69(4):345-364.

open access

Vol 69, No 4 (2018)
Guidelines
Submitted: 2018-08-21
Accepted: 2018-08-21

Abstract

Prader-Willi syndrome (PWS) is a complex genetic disorder characterised by a set of phenotypic traits, which include infantile hypotonia, short stature, and morbid obesity. Over the last 12 years, visible progress has been made in medical care management of PWS patients in Poland. Increasing awareness of the disorder in neonatal and paediatric care has led to early identification of the condition in neonates, followed by the institution of an appropriate dietary regime, introduction of physiotherapy, and early-onset recombinant human growth hormone (rhGH) treatment. Growth hormone (GH) therapy in Poland is conducted within the nationwide framework of the Therapeutic Programme: “Treatment of Prader-Willi Syndrome”. The therapeutic interventions initiated in the paediatric centres need to be continued in multidisciplinary adult care settings. The main aim of PWS clinical management in adulthood is prevention of obesity and its comor­bidities, treatment of hormonal disorders, mental health stabilisation, nutritional guidance, as well as on-going physiotherapy. Integrated multidisciplinary therapeutic intervention is necessary if patients with such a complex genetic condition as PWS are to not only achieve an average life expectancy but also to enjoy higher quality of life.

Abstract

Prader-Willi syndrome (PWS) is a complex genetic disorder characterised by a set of phenotypic traits, which include infantile hypotonia, short stature, and morbid obesity. Over the last 12 years, visible progress has been made in medical care management of PWS patients in Poland. Increasing awareness of the disorder in neonatal and paediatric care has led to early identification of the condition in neonates, followed by the institution of an appropriate dietary regime, introduction of physiotherapy, and early-onset recombinant human growth hormone (rhGH) treatment. Growth hormone (GH) therapy in Poland is conducted within the nationwide framework of the Therapeutic Programme: “Treatment of Prader-Willi Syndrome”. The therapeutic interventions initiated in the paediatric centres need to be continued in multidisciplinary adult care settings. The main aim of PWS clinical management in adulthood is prevention of obesity and its comor­bidities, treatment of hormonal disorders, mental health stabilisation, nutritional guidance, as well as on-going physiotherapy. Integrated multidisciplinary therapeutic intervention is necessary if patients with such a complex genetic condition as PWS are to not only achieve an average life expectancy but also to enjoy higher quality of life.

Get Citation

Keywords

Prader-Willi Syndrome (PWS), recombinant human growth hormone (rhGH), integrated therapy, rare diseases

About this article
Title

Management of Prader-Willi Syndrome (PWS) in adults — what an endocrinologist needs to know. Recommendations of the Polish Society of Endocrinology and the Polish Society of Paediatric Endocrinology and Diabetology

Journal

Endokrynologia Polska

Issue

Vol 69, No 4 (2018)

Pages

345-364

DOI

10.5603/EP.2018.0047

Bibliographic record

Endokrynologia Polska 2018;69(4):345-364.

Keywords

Prader-Willi Syndrome (PWS)
recombinant human growth hormone (rhGH)
integrated therapy
rare diseases

Authors

Magdalena Góralska
Tomasz Bednarczuk
Marek Rosłon
Maria Libura
Mieczysław Szalecki
Maciej Hilczer
Renata Stawerska
Joanna Smyczyńska
Małgorzata Karbownik-Lewińska
Mieczysław Walczak
Andrzej Lewiński

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Via MedicaWydawcą serwisu jest  "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail:  viamedica@viamedica.pl