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Lung function in children and adolescents with idiopathic interstitial pulmonary fibrosis
Author(s) -
Zapletal A.,
Houštěk J.,
Šamánek M.,
Čopová M.,
Paul T.
Publication year - 1985
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.1950010307
Subject(s) - medicine , idiopathic pulmonary fibrosis , pulmonary function testing , lung function , cystic fibrosis , lung , pulmonary fibrosis , intensive care medicine
Lung function of 65 patients who had idiopathic interstitial pulmonary fibrosis (IIPF) that had been treated with prednisone was evaluated by tests of ventilatory function, lung mechanics, and gas exchange at rest and during exercise. Ages on initial investigation ranged from 5 to 20 years. In 35 of 65 patients the studies were repeated an average of four times over a period of 1 to 9 years. Results of the first testing were as follows: 1) vital capacity (VC)—significantly reduced in all patients; 2) inspiratory capacity (IC)—significantly reduced in all patients; 3) total lung capacity (TLC)—reduced in 91%; 4) functional residual capacity (FRC)—reduced in 31%; 5) residual volume (RV)—reduced in 6%; 6) elastic recoil of the lungs (P st/ )—significantly increased in 97% at 100% TLC, significantly increased in 52% at 90% TLC, reduced in 68% at 60% TLC; 7) static compliance (C st )—reduced in 83%; 8) dynamic compliance (C dyn )—reduced in 88%; 9) specific airway conductance at FRC level (G aw /TGVex)—significantly increased in 50%; 10) maximum expiratory flow rates at 60% TLC (V max 60% TLC, in TLC/s)—significantly reduced in 33%; 11) upstream airway conductance (G us 60% TLC, in TLC/s/cm H 2 O)—reduced in 32%; 12) diffusing capacity of the lungs for carbon monoxide (D LCO ) related to body‐surface area—abnormal in 58% (when corrected for lung size, i.e., D LCO /TLC, abnormal in only 8%); 13) Pa O 2 at rest and after 6 minutes submaximal exercise—reduced in 25% and 63%, respectively. Changes in lung function that occurred with growth were assessed in terms of percentages of predicted values. Results showed that the VC and IC remained significantly reduced. An actual reduction of TLC, FRC, RV, breathing frequency, D L CO , and P st/ at 100% and 90% TLC was observed. Increases were seen in P st/ at 60% TLC, G aw /TGVex, V̇ max , and C st/ . Indices of lung elasticity suggested that regions of fibrosis and emphysema had become present. Smaller patients were also noted to have stiffer lungs.