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Effect of non-pharmacological intervention on the nutritional status of patients with Prader Willi Syndrome.
Ceccomancini, Romina; Gerk, Ayla; Stegmann, Jorgelina.
Afiliação
  • Ceccomancini R; Department of Nutrition, SPINE Foundation, Buenos Aires, Argentina.
  • Gerk A; Department of Clinical Research, SPINE Foundation, Buenos Aires, Argentina; Department of Clinical Medicine, SPINE Foundation, Buenos Aires, Argentina.
  • Stegmann J; Department of Clinical Research, SPINE Foundation, Buenos Aires, Argentina; Department of Clinical Medicine, SPINE Foundation, Buenos Aires, Argentina. Electronic address: coordinacioninvestigacion@spine.org.ar.
Clin Nutr ESPEN ; 59: 149-153, 2024 02.
Article em En | MEDLINE | ID: mdl-38220368
ABSTRACT

INTRODUCTION:

Obesity is highly prevalent in patients with Prader-Willi syndrome (PWS), particularly among adults. This condition, which can be morbid in many cases, is multifactorial and has a complex management. The purpose of our study was to describe the feasibility of achieving a better nutritional status, including normal weight in individuals diagnosed with PWS, through specific nutritional interventions within the framework of a transdisciplinary treatment and without resorting to pharmacological treatments or growth hormone (GH).

METHODOLOGY:

This observational study included patients with confirmed genetic diagnosis of PWS, receiving transdisciplinary treatment in a specialized rare diseases institution. Patients under treatment with GH and those under pharmacological treatment with nutritional objectives were excluded from the study. All patients attended our institution regularly on a weekly or fortnightly basis. Anthropometric records, including weight, height, and body mass index (BMI) were evaluated in each visit from treatment onset until the last check-up.

RESULTS:

We included 24 patients with confirmed genetic diagnosis of PWS. At baseline, 9 patients (38 %) had obesity grade III, 1 (4 %) of obesity grade II, 10 (42 %) of obesity grade I, 2 (8 %) of overweight, and 2 patients (8 %) with normal baseline weight. After a median duration of 52 months (interquartile range 23-116 months) of transdisciplinary nutritional treatment, we identified a significant reduction in BMI (baseline 40.2 ± 15.7 kg/m2 vs. follow-up 28.3 ± 6.7 kg/m2, p < 0.0001), without significant differences regarding height (baseline 1.45 ± 0.1 m vs. follow-up 1.48 ± 0.1 m, p = 0.09).

CONCLUSION:

In this study, we demonstrated that nutritional nonpharmacologic interventions immersed in a transdisciplinary treatment enabled a consistent and sustainable improvement in BMI and nutritional status among patients with PWS.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome de Prader-Willi / Hormônio do Crescimento Humano Tipo de estudo: Observational_studies Limite: Adult / Humans Idioma: En Revista: Clin Nutr ESPEN Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Argentina País de publicação: Reino Unido

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome de Prader-Willi / Hormônio do Crescimento Humano Tipo de estudo: Observational_studies Limite: Adult / Humans Idioma: En Revista: Clin Nutr ESPEN Ano de publicação: 2024 Tipo de documento: Article País de afiliação: Argentina País de publicação: Reino Unido