Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH.
Blood. 2022 Sep 15;140(11):1322-1324. doi: 10.1182/blood.2021015303.
Cardiovascular disease is a major cause of mortality in patients with sickle cell disease (SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed universal myocardial fibrosis, which they correlated with increased extracellular volume fraction (ECV). In the current study, they compared patients with SCD who were treated with hydroxyurea or transfusion at age <6 years to a group of patients with SCD without therapy. They documented that patients treated early had ECV levels comparable to normal controls, potentially preventing subsequent myocardial fibrosis.
心血管疾病是镰状细胞病（SCD）患者死亡的主要原因。Niss 等人此前曾报告称，对 25 名患者进行的心脏磁共振检查显示普遍存在心肌纤维化，他们将其与细胞外容积分数（ECV）增加相关联。在当前的研究中，他们将 6 岁前接受羟基脲或输血治疗的 SCD 患者与一组未接受治疗的 SCD 患者进行了比较。他们记录到早期接受治疗的患者的 ECV 水平与正常对照组相当，这可能防止了随后的心肌纤维化。
