血浆内啡肽和非对称二甲基精氨酸水平对重度β -地中海贫血儿童内皮和心功能的影响

Pub Date : 2022-04-01 DOI:10.4274/jcp.2022.88557
Utku Aygüneş, Ü. Can, M. Doğan, Merter Keçeli, Hatice Koçak Eker
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引用次数: 0

摘要

乙型地中海贫血(BTM)是一种常染色体隐性遗传病,以溶血性贫血为特征。频繁的红细胞输注导致体内铁负荷增加,导致内皮功能障碍和心血管并发症。高不对称二甲基精氨酸(ADMA)水平破坏内皮功能。内啡肽是在许多器官的血管内皮细胞中合成的可溶性蛋白多糖。内啡肽水平高表明内皮功能障碍。我们的目的是通过评估BTM患者组和健康对照组的循环ADMA和endocan水平来确定是否与心脏评估仪器相关,以及它们是否可以作为内皮功能障碍和心血管危险分层的预后标志物。材料与方法:共纳入39例BTM患者和39例年龄、性别匹配的健康儿童。记录所有患者的病史并进行体格检查。全血细胞计数,网织红细胞计数,血清铁蛋白和铁水平,肝肾功能检查,血浆ADMA和内啡肽,以及proBNP。由一名儿科心脏病专家对患者组和对照组的所有儿童的心脏检查结果进行评估。结果:BTM组平均ADMA水平高于对照组。BTM患者的内啡肽水平没有统计学差异。血清内啡肽和ADMA水平与心血管功能无关。结论:虽然BTM患者血清ADMA水平明显升高,但高血清内啡肽和ADMA水平与心血管功能无关。
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The Effect of Plasma Endocan and Asymmetric Dimethyl Arginine Levels on Endothelial and Cardiac Functions in Children with Beta-Thalassemia Major
Introduction: Beta-thalassemia major (BTM) is an autosomal recessive disease characterized by hemolytic anemia. Increased iron load accumulating in the body as a result of frequent erythrocyte transfusions leads to endothelial dysfunction and cardiovascular complications. High asymmetric dimethylarginine (ADMA) levels disrupt endothelial function. Endocan is a soluble proteoglycan synthesized in vascular endothelial cells in many organs. High levels of endocan indicate endothelial dysfunction. We aimed to determine whether there is a correlation with cardiac evaluation instruments by evaluating circulating ADMA and endocan levels in BTM patient group and healthy control group and whether they can be a prognostic marker in terms of endothelial dysfunction and cardiovascular risk stratification. Materials and Methods: A total of 39 patients with BTM and 39 age and gender-matched healthy children were enrolled in the study. Medical histories of all patients were recorded and physical examinations were performed. Complete blood count, reticulocyte count, serum ferritin and the iron level, liver, renal function tests, plasma ADMA and endocan, and proBNP. Cardiac examination results were evaluated on all children in both patient and control groups by a pediatric cardiologist. Results: Mean ADMA level in the BTM group is higher than in the control group. Endocan levels in patients with BTM were not found to be statistically different. Serum endocan and ADMA levels were not associated with cardiovascular functions. Conclusion: Although serum levels of ADMA were found to be significantly higher in BTM patients, high serum endocan and ADMA levels were not associated with cardiovascular functi-ons.
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