منابع مشابه
Pulmonary surfactant in cystic fibrosis.
Cystic fibrosis (CF) leads to a chronic inflammation of the airways with significant air flow limitations developing early in the course of the disease. As a well-functioning pulmonary surfactant is necessary to keep the alveoli and the small conducting airways open during expiration, we hypothesized that the biochemical and biophysical properties of surfactant may be impaired in CF. Bronchoalv...
متن کاملFunctional heterogeneity of pulmonary surfactant protein-D in cystic fibrosis.
Pulmonary surfactant protein-D (SP-D) is a soluble collagenous C-type lectin with important anti-microbial and anti-inflammatory properties. Although it is subject to functionally relevant modification by common polymorphisms and unregulated inflammation, the functional status of SP-D in cystic fibrosis (CF) remains unclear. Given the importance of infection and inflammation in CF lung patholog...
متن کاملPulmonary surfactant, lung function, and endobronchial inflammation in cystic fibrosis.
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 patients with CF (5-31 years, median 11) with well-conserved lung function (FEV1 94% predicted, range 78-121) and from 10 healthy control ...
متن کاملSurfactant Protein A in Cystic Fibrosis: Supratrimeric Structure and Pulmonary Outcome
BACKGROUND The state of oligomerization of surfactant associated protein-A (SP-A) monomers differs between individuals. This likely affects SP-A's functional properties and could thereby influence clinical status in patients with lung diseases. In this study we focus on SP-A structure in cystic fibrosis (CF) compared to both healthy subjects and disease controls. METHODS SP-A composition and ...
متن کاملPulmonary complications of cystic fibrosis.
Earlier diagnosis, treatment of exacerbations, and the use of long-term therapies have all improved the lifespan of patients with cystic fibrosis (CF). However, the natural history of CF airways disease remains one of worsening bronchiectasis and obstructive airways impairment. The progression of airways disease leads to eventual respiratory failure, but some will suffer other acute respiratory...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 1997
ISSN: 0000-0000,0903-1936
DOI: 10.1183/09031936.97.10091983