Blandón R, Leandro I M, Altafulla M, Díaz Fernández R
Servicio de Hematología, Hospital del Niño, Panamá.
Rev Med Panama. 1991 May;16(2):88-97.
One hundred and sixty four (164) patients were evaluated. Sixty (60) with Sickle cell disease (SSHg.) and ninety seven (97) with Trait (ASHg.); seventeen (17) were normal control group. The study confirmed that the incidence of cardiomyopathy in Trait (ASHg.) is greater than reported by other clinical investigations. Cardiac arrhythmia, atrial fibrillation, premature ventricular contractions, bundle branch blocks, and T and ST modifications with sub epicardial isquemia were most significant electrocardiographics changes. The possibility of myocardial infarction in SS patients with low or normal hemoglobin is significant. M-Mode and 2-D echo, demonstrated similar end diastolic volumes in AS and SS patients in which cardiomyopathy were diagnosticated. Patients with cardiac failure, treated with cardiotonics, diuretics and ACE were compensated most frequently. To prevent hemosiderosis, antioxydant (alfatocoferol and Ubiquinones) were used with satisfactory response.
对164例患者进行了评估。其中60例患有镰状细胞病(SSHg.),97例为性状携带者(ASHg.);17例为正常对照组。该研究证实,性状携带者(ASHg.)中心肌病的发病率高于其他临床研究报告的发病率。心律失常、心房颤动、室性早搏、束支传导阻滞以及伴有心外膜下缺血的T波和ST段改变是最显著的心电图变化。血红蛋白水平低或正常的SS患者发生心肌梗死的可能性很大。M型和二维超声心动图显示,被诊断为心肌病的AS和SS患者的舒张末期容积相似。心力衰竭患者使用强心剂、利尿剂和ACE进行治疗后,最常得到代偿。为预防含铁血黄素沉着症,使用了抗氧化剂(α-生育酚和泛醌),效果令人满意。