Pulmonary function testing in children's interstitial lung disease

Abstract

The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulness of PFTs is still undetermined, despite widespread use in adult interstitial lung disease. A literature review was initiated by the COST/Enter chILD working group aiming to describe published studies, to identify gaps in knowledge and to propose future research goals in regard to spirometry, whole-body plethysmography, infant and pre-school PFTs, measurement of diffusing capacity, multiple breath washout and cardiopulmonary exercise tests in chILD. The search revealed a limited number of papers published in the past three decades, of which the majority were descriptive and did not report pulmonary function as the main outcome.PFTs may be useful in different stages of management of children with suspected or confirmed chILD, but the chILD spectrum is diverse and includes a heterogeneous patient group in all ages. Research studies in well-defined patient cohorts are needed to establish which PFT and outcomes are most relevant for diagnosis, evaluation of disease severity and course, and monitoring individual conditions both for improvement in clinical care and as end-points in future randomised controlled trials.

Pulmonary function in children with persistent tachypnea of infancy

“…Still, there are few studies assessing DL CO in a heterogeneous group of patients with different ILDs. Moreover, there have been no reported DL CO measurements in PTI patients 14 . Our study showed that most of the children with PTI (90.9%) had abnormally high DL CO .…”

“…S cond and S acin primarily reflect heterogeneity in the conductive (airway generations 8-16) and acinar (airway generations 17-23+) lung zones, respectively. 14 Like IOS, most data on N2MBW comes from children with asthma or cystic fibrosis. There have been few publications presenting the results of N2MBW in adult ILD.…”

“…Although pulmonary function testing represents a cornerstone in the evaluation of respiratory diseases, there is only limited data on pulmonary dysfunction in children diagnosed with PTI. This scarcity of data was elegantly shown in a recently published review by Ring et al, 14 in which the majority of the reviewed papers were descriptive and did not report pulmonary function as the primary outcome. This scarcity especially applies to PTI cases diagnosed in early childhood.…”

“…In addition, spirometry, body plethysmography, and techniques enabling the evaluation of infant pulmonary testing under moderate sedation were most widely used in these studies. [14][15][16][17] New types of PFTs, namely impulse oscillometry (IOS) and nitrogen multiple breath washout (N2MBW), have become available in recent years. 18,19 These techniques are particularly useful in the evaluation of peripheral airways and are less demanding in terms of cooperation, which suggests new diagnostic possibilities in children.…”

The improved clinical course of persistent tachypnea of infancy with inhaled bronchodilators and corticosteroids

“…16,17 Data on pulmonary function tests (PFTs) in children with PTI are sparse. 18 The most common abnormalities found are peripheral airway obstruction and hyperinflation. 12,16,19,20 Bronchial obstruction reversibility testing is usually negative.…”