Cardiovascular adaptations to transfusion/chelation therapy of homozygote sickle cell anemia.
Cardiovascular adaptations to transfusion/chelation therapy of homozygote sickle cell anemia.
复制标题
心血管对纯合子镰状细胞贫血症的输血/螯合疗法的适应。
DOI：
10.1016/0002-9149(88)91376-8
复制
发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Gersony,WM
中科院分区：
文献类型：
--
作者：
Gaffney,JW;Bierman,FZ;Donnelly,CM;Sutton,M;Piomelli,S;Gersony,WM
输血/螯合治疗对患有纯合子镰状细胞病的儿童和年轻成人患者对慢性贫血的心血管适应性的影响尚不确定。本研究比较了未输血和输血的纯合子镰状细胞病患者的左心室（LV）功能指数和胸腹主动脉收缩期和舒张期血流。研究人群包括29例未输血的纯合子镰状细胞病患者，年龄为0.4至20.9岁（第1组）和11例慢性输血/螯合患者，年龄为4.0至21.8岁（第2组）。第2组的平均总血红蛋白浓度比第1组高28%。第2组中输血/螯合治疗的平均持续时间为3.7年。在两组中，预测的LV舒张末期和收缩末期尺寸的百分比显著大于相应的对照组。两组间预测的LV舒张末期尺寸百分比（第1组，120 ± 12%;第2组，120 ± 12%）或预测的LV收缩末期尺寸百分比（第1组，120 ± 12%;第2组，117 ± 8）无显著差异。研究组和对照组受试者的左心室缩短分数百分比相似。组1（2，426 ± 841）和组2（2，374 ± 1，004）的主动脉收缩期血流量（cc/min/m2）显著大于相应的对照值（分别为1，683 ± 442和1，736 ± 430）。第1组（699 ± 313 vs 488 ± 212）和第2组（1，080 ± 607 vs 588 ± 219）的主动脉舒张期血流量显著大于相应的对照值。这些结果表明，纯合子镰状细胞性贫血的中枢神经系统缺血的输血/螯合不会改变输血前心血管适应慢性贫血。
The effect of transfusion/chelation therapy on the cardiovascular adaptations to chronic anemia in pediatric and young adult patients with homozygous sickle cell disease is uncertain. This study compares left ventricular (LV) function indexes and thoracoabdominal aortic systolic and diastolic blood flow in nontransfused and transfused patients with homozygous sickle cell disease. The study population consisted of 29 nontransfused patients with homozygous sickle cell disease, ages 0.4 to 20.9 years (group 1) and 11 chronically transfused/chelated patients, ages 4.0 to 21.8 years (group 2). The mean total hemoglobin concentration in group 2 was 28% greater than that in group 1. The mean duration of transfusion/chelation therapy in group 2 was 3.7 years. The percent of predicted LV end-diastolic and end-systolic dimensions were significantly greater than the respective controls in both groups. There was no significant difference in percent of predicted LV end-diastolic dimension (group 1, 120 ± 12%; group 2, 120 ± 12%) or percent of predicted LV end-systolic dimension (group 1, 120 ± 12%; group 2, 117 ± 8) between the groups. The percent of LV shortening fraction was similar in study groups and control subjects. Aortic systolic blood flow (cc/min/m2) for group 1 (2,426 ± 841) and 2 (2,374 ± 1,004) were significantly greater than corresponding control values (1,683 ± 442, 1,736 ± 430, respectively). Aortic diastolic blood flow was significantly greater than corresponding control values for both group 1 (699 ± 313 vs 488 ± 212) and group 2 (1,080 ± 607 vs 588 ± 219). These findings suggest that transfusion/chelation of homozygote sickle cell anemia for central nervous system ischemia does not alter pretransfusion cardiovascular adaptations to chronic anemia.