[普拉德-威利综合征肥胖症的治疗方法]

[Therapeutical approach of obesity in Prader-Willi Syndrome].

作者信息

Carvalho Daniel F de, Cercato Cíntia, Almeida Madson Q, Mancini Marcio C, Halpern Alfredo

机构信息

Grupo de Obesidade e Síndrome Metabólica, Divisão de Endocrinologia, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, SP.

出版信息

Arq Bras Endocrinol Metabol. 2007 Aug;51(6):913-9. doi: 10.1590/s0004-27302007000600004.

DOI:10.1590/s0004-27302007000600004

PMID:17934657

Abstract

Prader-Willi Syndrome (PWS) is a multisystemic genetic disease characterized by hypotonia, mental retardation, characteristic facial appearance, hyperphagia, and compulsive eating due to hypothalamic dysfunction. PWS is caused by loss of function of genes located in chromosome 15q11-q13, an area subject to genomic imprinting. Obesity is a major cause of increased morbidity and mortality among patients with PWS. The objective of this study was to analyze the therapeutic options available for the treatment of the obesity in PWS including pharmacological and surgical strategies.

摘要

普拉德-威利综合征（PWS）是一种多系统遗传性疾病，其特征为肌张力减退、智力障碍、特殊面容、食欲亢进以及由于下丘脑功能障碍导致的强迫性进食。PWS是由位于15号染色体q11-q13区域的基因功能丧失引起的，该区域存在基因组印记。肥胖是PWS患者发病率和死亡率增加的主要原因。本研究的目的是分析可用于治疗PWS肥胖的治疗选择，包括药物和手术策略。