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Published ahead of print on July 21, 2004, doi:10.1164/rccm.200405-575OC

Am. J. Respir. Crit. Care Med., Volume 170, Number 9, November 2004, 1000-1005

A more recent version of this article appeared on November 1, 2004
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Submitted on May 10, 2004
Accepted on July 16, 2004

Pulmonary Surfactant, Lung Function and Endobronchial Inflammation in Cystic Fibrosis

Matthias Griese1*, Robert Essl1, Reinhold Schmidt1, Ernst Rietschel1, Felix Ratjen1, Manfred Ballmann1, and Karl Paul1

1 Department of Pediatrics, Ludwig-Maximilians-University, Munich, Germany

* To whom correspondence should be addressed. E-mail: griese{at}pk-i.med.uni-muenchen.de.

Cystic fibrosis (CF) lung disease is primarily a disease of the small airways. We hypothesized that even in patients with normal lung function, a reduced surfactant function would be present and favor small airway obstruction. Bronchoalveolar lavages from 76 CF patients (5-31 years, median 11) with well conserved lung function (FEV1 94% pred. (range 78-121) and from 10 healthy control subjects were investigated. The deviation of the biophysical surfactant performance from normal, assessed in a bubble surfactometer, was small, however the ability of the surfactant to maintain the patency of a narrow airway (% open) was significantly reduced. Surfactant protein (SP)-C level were increased, SP-B and SP-D were unchanged, whereas SP-A was decreased. Among the CF-patients neutrophilic inflammation was modestly related to a poorer surfactant activity, but not to lung function. SP-D was reduced in proportion to the degree of inflammation and in the presence of bacteria. These findings in a large cohort of CF patients with normal lung function show that the endobronchial airway inflammation is linked to early perturbations of the biophysical properties and immunological components of pulmonary surfactant and opens fields for novel therapeutic interventions.


Key words: surface activity, pulsating bubble, capillary surfactometer, bronchoalveolar lavage, airway inflammation




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