儿童Mauriac综合征2例并文献复习  

Two Cases of Mauriac Syndrome in Children and Literature Review

作　　者：韩蕊 张改秀[2] HAN Rui;ZHANG Gaixiu(Department of Pediatrics,Shanxi Medical University,Taiyuan 030001,China;Department of Endocrine and Genetic Metabolism,Shanxi Children′s Hospital,Taiyuan 030001,China)

机构地区：[1]山西医科大学儿科医学系,太原030001 [2]山西省儿童医院内分泌遗传代谢科,太原030001

出　　处：《医学综述》2023年第7期1449-1452,F0003,共5页Medical Recapitulate

摘　　要：目的探讨儿童Mauriac综合征的临床特点及治疗方法。方法回顾性分析2019年6月至2020年2月山西省儿童医院收治的2例儿童Mauriac综合征患儿(分别于3岁时、8岁时确诊为1型糖尿病,于2019年6月、2020年2月确诊为Mauriac综合征)的临床表现、实验室检查及治疗效果。结果2例患儿入院后均给予皮下注射胰岛素控制血糖,住院期间患儿肝脏均回缩。出院后随访1年,2例患儿血糖控制均不佳,身高增长缓慢,均低于同年龄、同性别儿童的3个标准差,且在随访过程中有肝肿大和丙氨酸转氨酶、天冬氨酸转氨酶升高。结论糖尿病患儿长期高血糖状态是Mauriac综合征发生的主要因素,患儿血糖控制不佳可造成糖尿病性肝损伤,引起肝功能异常及肝大,且会导致患儿出现生长迟缓及肥胖等表现。故对于糖尿病患儿要严格控制血糖,减少Mauriac综合征的发生,以提高患儿的生活质量。Objective To investigate the clinical characteristics and treatment of Mauriac syndrome in children.Methods Two children with Mauriac syndrome admitted to Shanxi Children′s Hospital from Jun.2019 to Feb.2020(diagnosed with type 1 diabetes at age 3 years and 8 years,and diagnosed with Mauriac syndrome in Jun.2019 and Feb.2020,respectively)were selected to conduct retrospective analysis on clinical manifestations,laboratory tests,and treatment effect.Results The two patients were given subcutaneous injection of insulin to control blood glucose after admission.During the hospitalization,the liver of both patients retracted.One year of follow-up after discharge was done,and the blood glucose control of the two patients was poor.The height growth of the two patients was slow,which was lower than the three standard deviations of children of the same age and sex.During the follow-up,there were hepatomegaly and alanine aminotransferase,aspartate aminotransferase rise.Conclusion Long term hyperglycemia in children with diabetes is the main factor leading to Mauriac syndrome.Poor glycemic control in children can cause diabetic liver injury,abnormal liver function and hepatomegaly,and lead to growth retardation and obesity.Therefore,for patients with diabetes,it is necessary to strictly control blood sugar,reduce the occurrence of the disease,in order to improve the quality of life of the patients.

关 键 词：儿童Mauriac综合征 临床特点 发病机制 

分 类 号：R725.8[医药卫生—儿科]