July 29, 2003 -- Bethesda, MD -- Cystic Fibrosis (CF) is one of the most frequent lethal chromosomal hereditary disorders in Caucasian populations and occurs in approximately one in every 3,500 births. Caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, a defective cAMP-dependent chloride ion conductance occurs. In patients with CF, lung disease is the major cause of sickness and death with the progressive decline of pulmonary function attributed to a vicious cycle of airway infection and inflammation. There is now evidence that inflammation plays a pivotal role and may be present very early in life, even before the onset of respiratory manifestations.

Background

The inflammatory process in the CF lung is dominated by a polymorphonuclear neutrophil (white blood cell) influx. Accumulation of neutrophils in the airways is associated with high concentrations of neutrophil-derived mediators, in particular pro-inflammatory cytokines (proteins) such as IL-8 and TNF- . Neutrophils also release numerous toxic agents, e.g., proteases and reactive oxygen species, which contribute to the damage of lung tissue. If the consequences of the neutrophil-dominated inflammation in CF with an altered repair of the respiratory structures can be explained by an overwhelming neutrophil toxicity, the mechanisms leading to neutrophil accumulation and activation in the CF airways are poorly understood. One possibility is that pro-inflammatory and anti-inflammatory imbalance with excessive concentrations of the neutrophil chemotactic cytokine IL-8 certainly plays an important role in the influx of neutrophils in the inflamed airways. Locally, bacterial toxins and inflammatory mediators can directly activate the neutrophils to carry out their cytotoxic activities.

Another cause may be that in CF patients, impaired neutrophil functions may contribute to an abnormal release of inflammatory mediators. One recent research effort provided data su
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Contact: Donna Krupa
djkrupa1@aol.com
703-527-7357
American Physiological Society
29-Jul-2003

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