Literature DB >> 21269146

Specific treatment of Prader-Willi syndrome through cyclical rehabilitation programmes.

Emanuele Grolla1, Gilberto Andrighetto, Pietro Parmigiani, Uros Hladnik, Gabriela Ferrari, Roberta Bernardelle, Martina Dal Lago, Anna Albarello, Giuseppe Baschirotto, Giuseppe Filippi, Roberto Lovato, Diego Dolcetta.   

Abstract

PURPOSE: To evaluate retrospectively the efficiency of our rehabilitation programme for patients with Prader-Willi Syndrome. In total, 49 patients were examined, 21 female and 28 male, the youngest in their late teens. Prader-Willi syndrome is generally characterised by cognitive impairment, behavioural abnormalities, and hyperphagia. Patients are usually considerably adverse to any form of physical exercise, and despite hormonal therapy, weight control in adult patients can be difficult.
METHODS: Four times a year, disease-specific residential programmes were organised, each lasting 4 weeks. The patients were restricted to a 1500 Kcal diet. In addition, they were required to do 6.5 h of physical exercise daily, stamina being built up by using music therapy, psychomotor therapy, education and entertainment activities.
RESULTS: BMI decreased by 2.1 average points in every residential session. For three patients who attended our treatments regularly, a reduction of 8.9 points over 6 years was recorded. An attendance of at least three sessions per year seemed to be necessary to substantially reduce weight.
CONCLUSIONS: A multidisciplinary approach and a daily calorie-counted diet can lead to significant weight loss in teenage and adult PWS patients. This approach would also be suitable in treating patients with other obesity syndromes with mental retardation.

Entities:  

Mesh:

Year:  2011        PMID: 21269146     DOI: 10.3109/09638288.2010.549288

Source DB:  PubMed          Journal:  Disabil Rehabil        ISSN: 0963-8288            Impact factor:   3.033


  10 in total

1.  Insulin-driven translational capacity is impaired in primary fibroblasts of Prader Willi.

Authors:  Cristiana Meneghello; Daniela Segat; Elisabetta Fortunati
Journal:  Intractable Rare Dis Res       Date:  2016-02

2.  Physical activity and maximal oxygen uptake in adults with Prader-Willi syndrome.

Authors:  Itai Gross; Harry J Hirsch; Naama Constantini; Shachar Nice; Yehuda Pollak; Larry Genstil; Talia Eldar-Geva; Varda Gross Tsur
Journal:  Eat Weight Disord       Date:  2017-03-16       Impact factor: 4.652

Review 3.  Evaluation and Management of Early Onset Genetic Obesity in Childhood.

Authors:  Sonali Malhotra; Ramya Sivasubramanian; Gitanjali Srivastava
Journal:  J Pediatr Genet       Date:  2021-07-03

Review 4.  Prader-Willi Syndrome in Adults: An Update On Nutritional Treatment and Pharmacological Approach.

Authors:  Silvia Savastano; Giovanna Muscogiuri; Luigi Barrea; Claudia Vetrani; Danilo Fintini; Giulia de Alteriis; Filippo Maria Panfili; Sarah Bocchini; Ludovica Verde; Annamaria Colao
Journal:  Curr Obes Rep       Date:  2022-09-05

Review 5.  GrowthHormone Research Society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome.

Authors:  Cheri L Deal; Michèle Tony; Charlotte Höybye; David B Allen; Maïthé Tauber; Jens Sandahl Christiansen
Journal:  J Clin Endocrinol Metab       Date:  2013-03-29       Impact factor: 5.958

6.  Weight control and behavior rehabilitation in a patient suffering from Prader Willi syndrome.

Authors:  Rosaria Di Lorenzo; Sara Sberveglieri; Donatella Marrama; Giulia Landi; Paola Ferri
Journal:  BMC Res Notes       Date:  2016-04-01

7.  Mapping the evidence of multidimensional health approaches in treating individuals with Prader-Willi Syndrome: A scoping review protocol.

Authors:  Cila Umat; Azhar Bin Talib; Normah Che Din; Nor Haniza Abdul Wahat
Journal:  BMJ Open       Date:  2022-01-12       Impact factor: 2.692

8.  Health Problems in Individuals With PWS Are Associated With Lower Quality of Life for Their Parents: A Snapshot in the Brazilian Population.

Authors:  Alexandre Slowetzky Amaro; Daniela Andrea Rubin; Maria Cristina Triguero Veloz Teixeira; Arcenio José Ferreira; Graciele Massoli Rodrigues; Luiz Renato Rodrigues Carreiro
Journal:  Front Pediatr       Date:  2022-02-15       Impact factor: 3.418

9.  Changes of Body Weight and Body Composition in Obese Patients with Prader-Willi Syndrome at 3 and 6 Years of Follow-Up: A Retrospective Cohort Study.

Authors:  Giorgio Bedogni; Graziano Grugni; Sabrina Cicolini; Diana Caroli; Sofia Tamini; Alessandro Sartorio
Journal:  J Clin Med       Date:  2020-11-08       Impact factor: 4.241

10.  A 24-Week Physical Activity Intervention Increases Bone Mineral Content without Changes in Bone Markers in Youth with PWS.

Authors:  Daniela A Rubin; Kathleen S Wilson; Camila E Orsso; Erik R Gertz; Andrea M Haqq; Diobel M Castner; Marilyn Dumont-Driscoll
Journal:  Genes (Basel)       Date:  2020-08-24       Impact factor: 4.096

  10 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.