ECE2015 Eposter Presentations Obesity and cardiovascular endocrinology (108 abstracts)
Therapeutic education contributes to minimise excess weight in Prader-Willi syndrome
Roxana Stefan 1 , Cristina Rusu 2 , Ioana Armasu 3 , Alina Belceanu 3 , Elena Braha 2 , Carmen Gorduza 3 , Bogdan Mihai 4 , Cristina Lacatusu 4 , Mihaela Negru 3 & Carmen Vulpoi 3
1Clinic Center Providenta, Iasi, Romania; 2Department of Genetics, Iasi, Romania; 3Department of Endocrinology, Iasi, Romania; 4Department of Diabetes, Nutrition and Metabolic Disease, Iasi, Romania.
Introduction: Prader-Willi syndrome (PWS) is a complex genetic disorder characterised by hyperphagia with progressive obesity, dysmorphic features, hypotonia, mental retardation, behavioural abnormalities and endocrine dysfunctions as hypogonadism and growth hormone (GH) deficiency. PWS is the most commonly identified genetic cause of obesity.
Methods: We reviewed five cases of confirmed PWS (three female and two male patients, aged between 8 and 32 years old) evaluated at the Endocrinology Department of Iaşi between January 2008 and July 2013. Clinical and hormonal data were documented.
Results: All patients had specific clinical features of PWS and genetic confirmation. They were all born in non-sanguine couples, four of them with low birth weight. After the first year of life they presented hyperfagia with rapid and important weight gain, except for one case where hyperphagia began in adolescence (at 13 years of age). The major weight gain was until late adolescence (>+6 S.D.). Nutritional counselling, with detailed information about diet, exercise, and subsequent adverse consequences of obesity, was offered to patients and family in order to ameliorate their eating behaviour. After that, the three patients that reached adulthood achieved a mean weight at +1.5 S.D. (under rigorous alimentation).
Paradoxally, all patients had a higher height than expected (mean+1.5 S.D.) in childhood and adolescence, even though three of them had low IGF1. Two patients had their first endocrinological examination in adulthood and presented a satisfactory final height (>155 cm).
Conclusions: Therapeutical education of the family, performed as early as possible, is an important determinant of the disease evolution and diminishes the potential weight gain. Although most patients with PWS have morbid obesity it seems that caregivers involvement in weight management may lead to favorable results.
Article tools
My recent searches
My recently viewed abstracts
Authors
Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
© Bioscientifica 2024 |
Privacy policy |
Cookie settings
BiosciAbstracts
Bioscientifica Abstracts is the gateway to a series of products that provide a permanent, citable record of abstracts for biomedical and life science conferences.
Find out more