肺动脉高血压在输血依赖的沙拉西米亚
Vineeta Gupta1, Vishnu Vijayakumar2, Priyanka Aggarwal2
1Division of Pediatric Hematology Oncology, Department pf Pediatrics,Institute of Medical Sciences, Banaras Hindu University (BHU), Varanasi, Uttar Pradesh. Correspondence to: Prof. Vineeta Gupta, Division of Pediatric Hematology Oncology, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi. vineetaguptabhu@gmail.com.
肺动脉高血压 (PAH) 影响31.6%的输血依赖性沙拉西米亚 (TDT) 患者. 高龄和先前的脊髓切除术是TDT患者PAH的关键风险因素.
科学领域:
- 心脏病学 心脏病学
- 血液学 血液学 血液学
- 肺部病理学 肺部病理学
背景情况:
- 输血依赖性沙拉西米亚 (TDT) 患者面临肺动脉高血压 (PAH) 的风险,原因是慢性血液溶解,铁过载,高凝血和切除术.
- 在 TDT 中评估 PAH 患病率和预测因素对于早期干预和管理至关重要.
研究的目的:
- 确定肺动脉高血压 (PAH) 在患有输血依赖性沙拉西米亚 (TDT) 的儿童和青少年中的患病率.
- 在这个患者群体中确定PAH的预测因子.
主要方法:
- 包括61名年龄在6-18岁的TDT患者.
- 使用二维心声图进行肺动脉抽缩压 (PASP) 和左心室喷射率 (LVEF) 评估.
- 使用T2*MRI评估心脏铁过载,并测量N-终端-pro脑性尿素 (NT-pro BNP) 水平.
主要成果:
- 在19名参与者中 (31.6%) 确诊了PAH.
- 患有PAH的患者年龄明显较大 (12.2 vs 9.6年,P=0.016) 并且具有较高的NT-pro BNP水平 (63.80 vs 41.97,P=0.01).
- 年龄较大,脊髓切除术后的岁月,NT-pro BNP升高,心脏T2*MRI值<10毫秒被确定为PAH的显著预测因素.
结论:
- 在31.6%的TDT患者中,PAH是普遍存在的,年龄较大和脊髓切除术史是显著的风险因素.
- NT-pro BNP作为一种有价值的查工具,用于检测TDT患者的PAH.
- 早期查和风险因素识别对于在TDT中管理PAH至关重要.
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