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血管内皮生长因子与儿童重型β地中海贫血相关肺动脉高压。

Vascular endothelial growth factor and pulmonary hypertension in children with beta thalassemia major.

机构信息

Zagazig University, Faculty of Medicine, Department of Pediatrics, Kassala, Egypt.

Al-Azhar University, Faculty of Medicine, Department of Pediatrics (for girls), Cairo, Egypt.

出版信息

J Pediatr (Rio J). 2019 Sep-Oct;95(5):593-599. doi: 10.1016/j.jped.2018.05.003. Epub 2018 Jun 1.

Abstract

OBJECTIVE

The purpose of this study was to illustrate the association between vascular endothelial growth factor level and pulmonary artery hypertension in children with β-thalassemia major.

METHOD

This case-control study was conducted on 116 children with β-thalassemia major; 58 of them had pulmonary artery hypertension. They were compared to 58 healthy children who were age and sex-matched (control group). Serum levels of vascular endothelial growth factor and echocardiographic assessment were done for all children.

RESULTS

Vascular endothelial growth factor serum level was significantly higher in children with β-thalassemia major with pulmonary artery hypertension than in those without pulmonary artery hypertension, as well as in control groups (p<0.001). Vascular endothelial growth factor serum level had a significant positive correlation with pulmonary artery pressure and serum ferritin, as well as a significant negative correlation with the duration of chelation therapy. Logistic regression analysis revealed that elevated vascular endothelial growth factor (Odd Ratio=1.5; 95% Confidence Interval, 1.137-2.065; p=0.005) was an independent risk factor of pulmonary artery hypertension in such children. Vascular endothelial growth factor serum level at a cutoff point of >169pg/mL had 93.1% sensitivity and 93.1% specificity for the presence of pulmonary artery hypertension in children with β-thalassemia major.

CONCLUSION

Elevated vascular endothelial growth factor serum level is associated with pulmonary artery hypertension in children with β-thalassemia.

摘要

目的

本研究旨在阐明血管内皮生长因子水平与重型β地中海贫血儿童肺动脉高压之间的关系。

方法

本病例对照研究纳入了 116 例重型β地中海贫血患儿,其中 58 例合并肺动脉高压,将其与 58 名年龄和性别相匹配的健康儿童(对照组)进行比较。所有儿童均进行了血管内皮生长因子血清水平检测和超声心动图评估。

结果

与无肺动脉高压组以及对照组相比,合并肺动脉高压的重型β地中海贫血患儿的血管内皮生长因子血清水平显著升高(均 P<0.001)。血管内皮生长因子血清水平与肺动脉压和血清铁蛋白呈显著正相关,与螯合治疗持续时间呈显著负相关。Logistic 回归分析显示,血管内皮生长因子升高(比值比=1.5;95%置信区间,1.137-2.065;P=0.005)是此类患儿肺动脉高压的独立危险因素。血管内皮生长因子血清水平截断值>169pg/mL 时,对重型β地中海贫血患儿肺动脉高压的检出具有 93.1%的敏感性和 93.1%的特异性。

结论

血管内皮生长因子血清水平升高与重型β地中海贫血儿童的肺动脉高压有关。

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