原发性及继发性血色病对心脏结构及功能影响的对比研究  

Involvement of cardiac structure and function between primary and secondary hemochromatosis:a comparative study

作　　者：王婉溦 武亭 王昱[2] 李柯鑫 刘立伟 欧晓娟[2] 贾继东[2] 赵新颜[2] WANG Wan-wei;WU Ting;WANG Yu;LI Ke-xin;LIU Li-wei;OU Xiao-juan;JIA Ji-dong;ZHAO Xin-yan(Beijing No.6 Hospital;Liver Research Center,Beijing Friendship Hospital,Capital Medical University,National Clinical Research Center for Digestive Disease,Beijing 100050,China)

机构地区：[1]北京市第六医院,100007 [2]首都医科大学附属北京友谊医院肝病中心,国家消化系统疾病临床医学研究中心

出　　处：《肝脏》2021年第5期542-546,共5页Chinese Hepatology

基　　金：北京市医院管理局消化内科学科协同发展中心专项经费资助(XXZ0301)。

摘　　要：目的比较原发性血色病与继发性血色病对心脏结构及功能的影响。方法回顾分析2008年1月至2018年12月在首都医科大学附属北京友谊医院诊断为铁过载的住院患者,收集患者临床表现及实验室指标、心电图及超声心动图心脏结构参数并进行对比分析。结果原发性血色病20例,平均年龄45岁,男性14例;继发性血色病20例,平均年龄47岁,男性15例。与继发性血色病患者比较,原发性血色病患者铁蛋白显著性升高(P<0.05),肝外表现更为常见。继发性血色病和原发性血色病患者中合并心律失常分别为1例和4例,差异无统计学意义(χ^(2)=2.057,P=0.342);两者在心电图及心脏结构主要参数方面差异无统计学意义(P>0.05)。原发性血色病2例心律失常患者中,1例房颤,1例频发室早,2例死于心力衰竭。结论原发性血色病患者铁过载程度高于继发性血色病患者,且心脏受累更为多见,包括心律失常,心力衰竭等,心脏受累往往提示预后不良。Objective To compare the characteristics of cardiac involvement between primary and secondary hemochromatosis.Methods Patients with hemochromatosis from January 2008 to December 2018 in Beijing Friendship Hospital were retrospectively analyzed.The clinical manifestation,laboratory parameters,electrocardiogram and echocardiographic structural parameters of the patients were collected and compared.Results There were 20 cases of primary hemochromatosis(mean age 45.0 years,male 70.0%)and 20 cases of secondary hemochromatosis(mean age 47 years,male 75%).Patients with primary hemochromatosis had a significant higher level of ferritin(P<0.05)and more frequent extrahepatic manifestations than patients with secondary hemochromatosis.Cardiac arrhythmias were more common in patients with primary haemochromatosis than in patients with secondary hemochromatosis,but the difference was not statistically significant(20%vs.5%,χ^(2)=2.057,P=0.342).There were no significant differences in ECG and cardiac structure between two groups(P>0.05).Primary hemochromatosis included 2 patients with arrhythmias,one patient with atrial fibrillation,another one with frequent ventricular premature beats and 2 patients died of cardiac failure.Conclusion The degree of iron overload in primary hemochromatosis was significantly higher than that of secondary hemochromatosis.Cardiac involvement is more common in patients with primary hemochromatosis,including arrhythmias and heart failure.Heart involvement of hemochromatosis often indicates a poor prognosis and warrants attention of hepatologists.

关 键 词：原发性血色病 继发性血色病 心律失常 心功能衰竭 

分 类 号：R54[医药卫生—心血管疾病]