发育医学电子杂志Issue(4)：224-227,4.
14例糖原累积病临床、病理特征及治疗
Clinical manifestation, pathological feature and treatment of 14 cases of glycogen storage disease
陈小宁 1牛焕红 2晏伟 3何娜 4成胜权5
作者信息
- 1. 海南省屯昌县人民医院 儿科，海南 屯昌 571600
- 2. 第四军医大学西京医院 儿科
- 3. 第四军医大学西京医院 儿科
- 4. 第四军医大学 病理科，西安 710032
- 5. 海南省海口市妇幼保健院 儿科，海口 570203
- 折叠
摘要
Abstract
ObjectiveTo retrospectively analyze clinical manifestation, pathological feature,follow-up and treatment of 14 cases of glycogen storage disease (GSD), in order to gain deep insight into the disease. Method Fourteen cases of children patients diagnosed with GSD in Xijing Hospital during the year of 2008 and 2014 were retrospectively analyzed in term of clinical and pathological characteristics, and treatment. Result The initial symptoms of 14 children patients with GSD were all hepatomegaly and hepatic dysfunction, some cases among them were concomitant with hypoglycemia and hyperlipidemia. 5 cases of children GSD were given treatment and follow-up. After treatment of raw corn starch [1.75-2.5 g/(kg·time), 4 times /day], the levels of blood glucose and blood lipid in 3 cases turned to normality, hepatomegaly in 2 cases turned to normality with in half a year.ConclusionIn terms of inexplicable hepatomegaly, hepatic dysfunction, hypoglycemia and hyperlipidemia in children cases, the possibility of GSD should be considered. Raw corn starch is an effective diet therapy for some Children cases.关键词
糖原累积病/儿童/肝脏活检Key words
Glycogen storage disease/Children/Liver biopsy引用本文复制引用
陈小宁,牛焕红,晏伟,何娜,成胜权..14例糖原累积病临床、病理特征及治疗[J].发育医学电子杂志,2014,(4):224-227,4.
