Prader-Willi综合征合并糖尿病1例病例报道  

Report a case of Prader-Willi syndrome complicated with diabetes mellitus

作　　者：杨涛 宫晴[2] 王世东[2] 啜文静 李佳玥[2] 王格[2] YANG Tao GONG qing WANG Shidong CHUO Wenjing LI Jiayue WANG Ge(The First Clinical Medical College, Beijing University of Chinese Medicine, Beijing 100029,China Department of Endocrinology of Renal Disease, Dong Zhimen Hospital, Beijing 100700, China Beijing First Hospital of Traditional Chinese Medicine and Western Medicine, Beijing 100026,China)

机构地区：[1]北京中医药大学第一临床医学院,北京100029 [2]北京中医药大学东直门医院肾病内分泌科,北京100700 [3]北京市第一中西医结合医院,北京100026

出　　处：《中国医药导报》2017年第29期170-173,共4页China Medical Herald

摘　　要：本文报告1例Prader-Willi综合征(PWS)合并糖尿病患者,本例患者于1996年诊断为糖尿病,2015年行基因检查明确诊断为PWS。本次入院主要表现为多食、肥胖和难以控制的高血糖。治疗方面,严格控制饮食并结合运动,起始降糖方案为口服降糖药物联合胰岛素,治疗效果不明显,后加用GPL-1类似物,血糖水平较前有明显改善。PWS是一种比较少见的遗传性肥胖综合征,因相关基因表达缺失导致下丘脑功能不全,引起神经内分泌功能紊乱进而出现多食、肥胖、生长发育不良等复杂的临床表现,早期诊断和及时干预对本病的预后至关重要。This paper reports a case of Prader-Willi syndrome （PWS） with diabetes mellitus, the patient was diagnosed with diabetes in 1996 and was diagnosed with PWS by genetic examination in 2015, the major clinical manifestations are overeating, obesity, and uncontrollable hyperglycemia. In terms of treatment, strict diet control and exercise, the initial hypoglycemic program for oral hypoglyeemic drugs combined with insulin, but the efficacy of this treatment is not obvious, and after the use of GPL-1 analogues, the patient＇s blood glucose levels improved significantly. PWS is a rare syndrome of genetic obesity, due to lack of related gene expression of hypothalamic dysfunction eaused by neu- roendocrine dysfunction and polyphagia, obesity, poor growth and development of such complicated clinical manifestations. The early diagnosis and timely intervention is important for prognosis of the disease.

关 键 词：PRADER-WILLI综合征 肥胖 糖尿病 GLP-1类似物 

分 类 号：R58[医药卫生—内分泌]