在发育期肺部疾病中对肺高血压的跨学科共识方法
Nidhy P Varghese1, Eric D Austin2, Csaba Galambos3
1Department of Pediatrics, Division of Pulmonology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA paulose@bcm.edu.
遗传性呼吸系统疾病可以导致婴儿严重的肺高血压 (PH). 本综述解决了在发育性肺病 (DEVLD) 中诊断和管理PH的挑战,以改善婴儿的结果.
科学领域:
- 儿科肺病学 儿科肺病学
- 新生儿科学 新生儿科学
- 医学遗传学 医学遗传学
背景情况:
- 多种遗传性呼吸系统疾病在新生儿和幼儿中表现为严重的肺高血压 (PH).
- 对于长期结果的最佳诊断和管理策略仍在争论中.
- 发育性肺病 (DEVLD) 是婴儿PH的一个重要背景.
研究的目的:
- 在DEVLD.设置中定义PH的特征.
- 突出当前的挑战和临床方法的建议.
- 提供关于家庭咨询和支持的指导.
主要方法:
- 建立了一个多学科的专家临床小组.
- 对患有DEVLD/DEVLD-PH.婴儿的临床特征进行了审查.
- 确定了诊断和管理中的决策挑战.
主要成果:
- 患有DEVLD相关PH的婴儿的临床特征.
- 突出了遗传评估,肺活检,成像和治疗方面的挑战.
- 强调需要跨学科的共识.
结论:
- 在DEVLD中婴儿PH的最佳管理需要多学科的方法.
- 加强沟通和家庭咨询至关重要.
- 解决诊断和治疗的不确定性是改善长期结果的关键.
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