Maioli Maria Christina Paixão, Soares Andrea Ribeiro, Bedirian Ricardo, Alves Ursula David, de Lima Marinho Cirlene, Lopes Agnaldo José
Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Universidade do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.
Rev Bras Hematol Hemoter. 2016 Jan-Feb;38(1):21-7. doi: 10.1016/j.bjhh.2015.11.001. Epub 2015 Dec 11.
To evaluate the association between clinical, pulmonary, and cardiovascular findings in patients with sickle cell disease and, secondarily, to compare these findings between sickle cell anemia patients and those with other sickle cell diseases.
Fifty-nine adults were included in this cross-sectional study; 47 had sickle cell anemia, and 12 had other sickle cell diseases. All patients underwent pulmonary function tests, chest computed tomography, and echocardiography.
Abnormalities on computed tomography, echocardiography, and pulmonary function tests were observed in 93.5%, 75.0%; and 70.2% of patients, respectively. A higher frequency of restrictive abnormalities was observed in patients with a history of acute chest syndrome (85% vs. 21.6%; p-value<0.0001) and among patients with increased left ventricle size (48.2% vs. 22.2%; p-value=0.036), and a higher frequency of reduced respiratory muscle strength was observed in patients with a ground-glass pattern (33.3% vs. 4.3%; p-value=0.016). Moreover, a higher frequency of mosaic attenuation was observed in patients with elevated tricuspid regurgitation velocity (61.1% vs. 24%; p-value=0.014). Compared to patients with other sickle cell diseases, sickle cell anemia patients had suffered increased frequencies of acute pain episodes, and acute chest syndrome, and exhibited mosaic attenuation on computed tomography, and abnormalities on echocardiography.
A significant interrelation between abnormalities of the pulmonary and cardiovascular systems was observed in sickle cell disease patients. Furthermore, the severity of the cardiopulmonary parameters among patients with sickle cell anemia was greater than that of patients with other sickle cell diseases.
评估镰状细胞病患者的临床、肺部和心血管检查结果之间的关联，其次，比较镰状细胞贫血患者与其他镰状细胞病患者的这些检查结果。
59名成年人纳入了这项横断面研究；47名患有镰状细胞贫血，12名患有其他镰状细胞病。所有患者均接受了肺功能测试、胸部计算机断层扫描和超声心动图检查。
计算机断层扫描、超声心动图和肺功能测试异常分别在93.5%、75.0%和70.2%的患者中观察到。有急性胸综合征病史的患者（85%对21.6%；p值<0.0001）和左心室增大的患者（48.2%对22.2%；p值=0.036）中观察到限制性异常的频率更高，磨玻璃样改变的患者中观察到呼吸肌力量减弱的频率更高（33.3%对4.3%；p值=0.016）。此外，三尖瓣反流速度升高的患者中观察到马赛克样衰减的频率更高（61.1%对24%；p值=0.014）。与其他镰状细胞病患者相比，镰状细胞贫血患者急性疼痛发作、急性胸综合征的发生率更高，计算机断层扫描显示马赛克样衰减，超声心动图检查有异常。
在镰状细胞病患者中观察到肺部和心血管系统异常之间存在显著的相互关系。此外，镰状细胞贫血患者的心肺参数严重程度高于其他镰状细胞病患者。
