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无症状重型β地中海贫血患者的心肺功能障碍

Cardiac and pulmonary dysfunction in asymptomatic beta-thalassanemia major.

作者信息

Noori Noor Mohammad, Keshavarz Kambiz, Shahriar Mosaeib

机构信息

Pediatric Cardiology, Children and Adolescent Health Research Center, Zahedan University of Medical Sciences, Zahedan, Iran.

出版信息

Asian Cardiovasc Thorac Ann. 2012 Oct;20(5):555-9. doi: 10.1177/0218492312439706.

DOI:10.1177/0218492312439706
PMID:23087299
Abstract

This study assessed cardiac and pulmonary dysfunction in 26 asymptomatic patients with beta-thalassemia major. This investigation was a case-controlled study considering 10-20-year-old patients with beta-thalassemia major and no cardiac or pulmonary symptoms. Healthy individuals matched for age and sex were used as controls. At 48-72 h after blood transfusion, the patients underwent echocardiography and spirometry by a cardiologist and a pulmonologist. The results were compared to those of the control group. The right and left myocardial performance index, preejection period/ejection time ratio, ejection fraction, acceleration time, isovolumic contraction time, and bilateral isovolumic relaxation times in the study group demonstrated significant differences from the data of the controls. Right deceleration time was significantly different between the 2 groups. Myocardial performance index, peak early velocity of the right heart, and peak atrial velocity-to-peak early velocity ratio of the right side by Doppler tissue imaging were also significantly different between the 2 groups. Spirometry showed a significant difference in forced expiratory volume in 1 s/forced vital capacity between groups. Based on spirometry, pulmonary involvement in the patients was 77% restrictive. These findings show that systolic and diastolic dysfunction of the heart and pulmonary disturbances are unavoidable in patients with beta-thalassemia major.

摘要

本研究评估了26例无症状重型β地中海贫血患者的心脏和肺功能障碍。本调查为病例对照研究,纳入了10至20岁、无心脏或肺部症状的重型β地中海贫血患者。年龄和性别匹配的健康个体作为对照。输血后48至72小时,由心脏病专家和肺科医生对患者进行超声心动图和肺活量测定。将结果与对照组进行比较。研究组的右心室和左心室心肌性能指数、射血前期/射血时间比值、射血分数、加速时间、等容收缩时间以及双侧等容舒张时间与对照组数据存在显著差异。两组之间的右心室减速时间也存在显著差异。两组之间通过多普勒组织成像得出的心肌性能指数、右心室早期峰值速度以及右侧心房峰值速度与早期峰值速度比值也存在显著差异。肺活量测定显示两组之间第1秒用力呼气量/用力肺活量存在显著差异。基于肺活量测定,患者肺部受累77%为限制性。这些发现表明,重型β地中海贫血患者不可避免地会出现心脏收缩和舒张功能障碍以及肺部紊乱。

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