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镰状细胞贫血中的舒张功能障碍和肺动脉高压:L-肉碱治疗是否起作用?

Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment?

作者信息

El-Beshlawy A, Abd El Raouf E, Mostafa F, Talaat M, Isma'eel H, Aoun E, Hoffbrand A V, Taher A

机构信息

Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.

出版信息

Acta Haematol. 2006;115(1-2):91-6. doi: 10.1159/000089472.

Abstract

Clinical manifestations of cardiovascular abnormalities in patients with sickle cell (SC) anemia are well documented. Many variables were assessed in our study before and after administration of L-carnitine to randomly selected 37 SC disease (SCD) children for a period of 6 months. Variables such as weight, height, serum ferritin levels, units of blood transfused and the number of veno-occlusive crises all showed significant improvement after the 6 months of therapy with L-carnitine. Our study also showed that cardiac diastolic function and pulmonary hypertension are common in pediatric SCD patients. These two disorders showed some improvement after L-carnitine administration. Therefore, L-carnitine deserves a rigorous large-scale randomized clinical trial to evaluate its potential benefits as treatment for SCD patients with cardiac complications.

摘要

镰状细胞(SC)贫血患者心血管异常的临床表现已有充分记录。在我们的研究中,对随机选取的37名SC病(SCD)儿童给予左旋肉碱治疗6个月,治疗前后评估了许多变量。体重、身高、血清铁蛋白水平、输血单位数和静脉闭塞性危机次数等变量在接受左旋肉碱治疗6个月后均有显著改善。我们的研究还表明,心脏舒张功能和肺动脉高压在儿科SCD患者中很常见。给予左旋肉碱后,这两种疾病有所改善。因此,左旋肉碱值得进行严格的大规模随机临床试验,以评估其作为治疗有心脏并发症的SCD患者的潜在益处。

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