生玉米淀粉治疗肝糖原累积病并发严重痛风性关节炎一例  

Treatment of Glycogen Storage Disease Combined with Severe Gout with Uncooked Cornstarch: A Case Report

作　　者：朱丽晔 沈山梅[2] 毕艳[2] 冯文焕[2] 金玺[2] 石欢[2] 韩小娟[2] 王静[3] 朱大龙[2] 

机构地区：[1]溧水区人民医院内分泌科,江苏省南京市211200 [2]南京大学医学院附属鼓楼医院内分泌科 [3]南京大学医学院附属鼓楼医院临床营养科

出　　处：《中国全科医学》2013年第30期3626-3629,共4页Chinese General Practice

摘　　要：为探讨肝糖原累积病的临床特征及诊治策略,本文回顾性分析了本院2011-03-18收治的1例并发严重痛风性关节炎的肝糖原累积病患者的临床资料。患者为20岁男性,以多发肢体关节剧烈疼痛为主要症状就诊,经常鼻出血。实验室检查发现血尿酸水平明显升高、空腹血糖水平降低、三酰甘油水平升高、肝功能异常等;腹部B超及上腹部CT检查示肝弥漫性病变不伴脾肿大;空腹肾上腺刺激试验阳性。给予患者以生玉米淀粉悬液为主要的营养治疗后,其一般情况改善,关节肿痛好转,各项代谢指标好转,肝脏体积逐渐恢复正常,体质量及身高增加,治疗效果明显。In order to investigate the clinical characteristics, strategy of diagnosis and treatment of glycogen storage disease, the clinical data of a patient with glycogen storage disease combined with severe gout was reviewed retrospectively. The 20 years old male patient was hospitalized on 18 March 2011, the main symptom was intensive pain of multiply joints, and nose bleeds happened frequently. Laboratory tests showed elevated levels of uric acid and triglyceride, decreasing levels of fasting blood glucose, as well as dysfunction of liver. The results of abdominal B type uhrasonography and upper abdomen CT showed the dif- fuse lesions of liver without splenomegaly. Fasting epinephrine test result showed positive. Intensive dietary management with un- cooked cornstarch mixture was given to the patient, after the above treatment, the patient＇s general condition improved, pain in joints disappeared, metabolic indexes improved, and liver size normalized. At the same time, the weight and height of the patient increased. In conclusion, the effect of uncooked cornstarch treatment is obvious.

关 键 词：糖原贮积病I型 痛风 葡糖-6-磷酸酶 玉米 淀粉 

分 类 号：R589.1[医药卫生—内分泌]