间质性肺疾病患儿肺功能改变的特征

Characteristics of pulmonary function changes in children with interstitial lung diseases

间质性肺疾病(interstitial lung diseases,ILD)是以肺实质扩张性降低所致的限制性通气功能障碍为特征的一类疾病。ILD患者病理生理特征是气体交换异常和通气功能障碍。其气体交换异常的出现往往先于呼吸力学的改变,这一特点可以提高肺功能检查诊断ILD的敏感性。静息肺功能和运动性低氧血症有助于判断ILD的预后,肺活量和弥散能力有助于评估患者对治疗的反应。较之基线测量,不同肺功能参数的趋势变化能够更好地判断预后。呼吸力学改变和气体交换异常是大多数ILD的共同特点,ILD的肺功能异常是典型的,但缺乏特异性。

Interstitial lung diseases(ILD) are functionally characterized by a restrictive ventilatory defect due to a reduced distensibility of the lung parenchyma.ILD patients also show a reduced exercise tolerance,and the main factors limiting exercise capacity are ventilatory and gas exchange abnormalities.The change of gas exchange often precedes the change of respiratory mechanics,which can improve the sensitivity of diagnosis of ILD.Resting pulmonary function and exercise-induced hypoxemia can aid to define the prognosis of ILD,and vital capacity and diffusing capacity are useful parameters to monitor the response of patients to therapy.To determine prognosis,identifying the trend of different parameters of lung function is a better method than baseline measurement.Respiratory mechanical changes and abnormal gas exchange are common characteristics of most ILD,and functional abnormalities in ILDs are typical,but not specific.