糖原累积病Ⅲ型的心脏表现  

Cardiac manifestations of the glycogen storage disease type Ⅲ

作　　者：郭潇潇[1] 田庄[1] 郭立琳[1] 张抒扬[1] 朱文玲[1] 

机构地区：[1]北京协和医院心内科,北京100730

出　　处：《临床心血管病杂志》2011年第2期134-137,共4页Journal of Clinical Cardiology

摘　　要：目的:总结糖原累积病Ⅲ型(GSDⅢ型)患者心脏改变的临床特点。方法:收集46例GSDⅢ型患者的临床资料,分析其心电图(ECG)和超声心动图(Echo)结果。结果:45例(97.8%)无明显心脏相关症状,10例心电图异常,分别为左室肥厚3例,PR间期延长3例,T波改变2例,双心室肥厚伴QT间期延长、单纯QT间期延长各1例。Echo异常15例,其中心室肥厚5例,单纯左房增大4例,并发先天性心脏病、少量心包积液各3例。心脏异常与肝功能和肌酸激酶水平无关(P>0.05)。结论:GSDⅢ型患者可能并发多种心脏损害,但起病隐匿,需提高警惕并定期行ECG和Echo检查,如出现心脏功能障碍,应进行相应药物治疗。Objective：To summarize the cardiac manifestations of glycogen storage disease（GSD） type Ⅲ.Method：The clinical data of 46 GSD type Ⅲ patients were collected.Their electrocardiography（ECG） and echocardiography（Echo） features were analyzed.Result：All the patients except one did not have cardiac symptoms.Ten patients had abnormalities with ECG.The abnormal electrocardiographic findings were ventricular hypertrophy（3 cases）,prolonged PR intervals（3 cases）,hypertrophy of both left and right ventricles with prolonged QT intervals（1 case） and T wave changes（1 case） respectively.Abnormalities of echo were found in 15 cases.Five cases had ventricular hypertrophy.Left atrial enlargement was observed in 4 cases.Congenital heart disease and pericardial effusion were found in three cases respectively.The cardiac abnormalities had no relationship with the level of liver function or creatine kinase（P0.05）.Conclusion：GSD type Ⅲ patients may present silent cardiac complications such as hypertrophic myocadiopathy.However,the onset was uneasy to detect.Therefore,electrocardiogram and echocardiography should be investigated regularly in respect of patients＇ clinical symptoms or muscle status and proper treatment should be given.

关 键 词：糖原累积病Ⅲ型 心肌病变 心电图异常 

分 类 号：R596.1[医药卫生—内科学]