Preserved diffusion capacity in children with cystic fibrosis.

Abstract:

:Early detection of progressive lung disease in cystic fibrosis (CF) may lead to better treatment and prognosis. Routine lung function indices may be relatively insensitive markers of peripheral airway obstruction and alveolar collapse. We hypothesized that the single-breath diffusion capacity of the lung for carbon monoxide (DLCO) would change before tests of airway function in patients with CF. We assessed lung function longitudinally in 53 children with CF during a mean period of 3.8 years to determine whether the diffusion capacity of the lung becomes abnormal before more conventional indices of lung function do. Within patients, DLCO was slightly elevated and remained stable, while forced expired volume in 1 sec (FEV1) and forced vital capacity (FVC) declined progressively (mean individual decline, -1.8% and -0.8% of predicted). Cross-sectionally, this decline was faster (mean group decline -3.8% and -2.8% of predicted), indicating an additional cohort effect. Normalized diffusion capacity at an early stage of CF is slightly elevated and is preserved in spite of progressive obstructive lung disease. This can be attributed to alterations in pulmonary and bronchial circulation due to loss of function and/or number of alveolar units. Diffusion capacity at rest does not appear to be a suitable early marker of progressive deterioration of CF lung disease.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Merkus PJ,Govaere ES,Hop WH,Stam H,Tiddens HA,de Jongste JC

doi

10.1002/ppul.10357

subject

Has Abstract

pub_date

2004-01-01 00:00:00

pages

56-60

issue

1

eissn

8755-6863

issn

1099-0496

journal_volume

37

pub_type

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