Cystic fibrosis ciliary dyskinesia substances and pulmonary disease. Effects of ciliary dyskinesia substances on neutrophil movement in vitro.

Abstract

A B S T R A C T Cultured mononuclear cells (MNC) from individuals homozygous or heterozygous for the defective gene causing the inherited disease cystic fibrosis (CF) synthesize three unusual "mediators" termed ciliary dyskinesia substances (CDS), which markedly affect tracheal mucociliary systems in vitro. MNC cultures from normal healthy controls do not accumulate any CDS, whereas MNC cultures from non-CF patient controls with pulmonary disease synthesize at least one CDS. The possible involvement of the CDS in pulmonary disease is being investigated. In this study, we sought to determine whether the CDS could be chemoattractants for polymorphonuclear neutrophils (PMN), since they have characteristics in common with known chemoattractants generated by alveolar macrophages. Our

Imbalance between Polymorphonuclear Leukocyte Proteases and Antiproteases in Chronic Pyogenic Infections and Its Relation to the Proteolysis of Complement Component C3

“…These substances were later shown to be small cleavage products of C3 and C5 with chemotactic activity [82] and obviously, their presence in bronchial secretions may lead to the accumulation of large numbers of PMNL. A report from Cabrini [54] presents evidence that excessive degranulation of CF neutrophils may occur as a consequence of increased intracellular free calcium levels in these cells.…”