在患有肺部低成形症的儿童中长期肺功能结果
Alexander I Gipsman1, Enrico Danzer2, Annaliese Aarthun2
1Division of Pulmonary and Sleep Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA.
The Journal of pediatrics
|May 30, 2025
概括
患有先天性隔膜 (CDH),先天性肺形和巨型肺的儿童在童年后期经历了持续的异常肺功能,每个情况都有不同的模式.
科学领域:
- 儿科肺病学 儿科肺病学
- 胸部外科手术 胸部外科手术
- 新生儿科学 新生儿科学
背景情况:
- 肺部缺血症是一种肺部发育不完全的情况,可以由各种先天性异常引起.
- 先天性腹膜 (CDH),早期干预先天性肺形 (EICLM) 和巨型腹膜 (GO) 是与肺部缺血症相关的疾病.
- 了解长期肺功能轨迹对于管理这些复杂的儿科疾病至关重要.
研究的目的:
- 研究和比较8-13岁儿童的肺功能轨迹,这些儿童患有CDH,EICLM和GO.
- 为了确定与肺部低成形的根本原因相关的肺功能异常的特定模式.
主要方法:
- 对81名被诊断患有CDH,EICLM或GO的儿童 (8-13岁) 进行了螺旋测量和囊造影.
- 进行了心声图以评估心脏功能,并对右心室功能进行了回顾性定量分析.
- 收集了临床,人口和医疗记录数据.
主要成果:
- 在51.9%的研究队列中观察到异常的肺功能.
- 患有CDH和EICLM的患者主要表现出阻塞性肺病模式.
- 患有GO的儿童经常表现出限制性肺病模式. 胸壁异常和胸内肝位置等因素与CDH患者的异常肺功能有关.
结论:
- 在患有CDH,EICLM和GO的个体中,异常的肺功能持续到童年后期.
- 每种潜在疾病都与不同的肺功能特征有关,这表明肺生长和重塑途径各不相同.
- 这些发现凸显了特定疾病管理对于这些儿科患者的长期呼吸系统健康的重要性.
相关概念视频
Pulmonary Function Tests
438
Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
438
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
2.6K
Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
Medical History
Medical History
2.6K
Chronic Obstructive Pulmonary Disease-I: Introduction
3.0K
Chronic Obstructive Pulmonary Disease (COPD) is a long-lasting respiratory condition requiring continuous attention and care. It is a progressive lung disease that leads to breathing challenges due to airflow obstruction. It manifests as persistent respiratory symptoms and restricted airflow resulting from abnormalities in the airways and alveoli, usually due to long-term exposure to harmful particles or gases. COPD mainly consists of two primary conditions: emphysema and chronic bronchitis.
3.0K
Pulmonary Cycle: Exhalation
1.8K
In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...
1.8K
Pulmonary Hypertension: Classification and Pathogenesis
300
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
300
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
3.1K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
3.1K


