Increased Phagocytic Cell Chemiluminescence in Patients With Cystic Fibrosis

Increased whole blood chemiluminescence in patients with Shwachman syndrome: therapy trial with thiamine and α-tocopherol

“…The differences may have been contributed by the differences between the techniques used. Furthermore, the mean age of the patients in the study of Roberts and Stiehm [27] was higher (23.6 years) than in that of Kemp et al [21] (15.2 years), in that of Graft et al [16] (15.3 years), or in the present study (6.3 years). It is possible that neutrophils from the older patients become "primed" by chronic infections and thereby are more easily activated as suggested by Roberts and Stiehm [27].…”

“…In cystic fibrosis, Kemp,al. [21] found FMLP-induced non-enhanced CL of pm lied neutrophils to be decreased, Graft et al [16] four OZP-induced luminol-enhanced CL of neutrophils to t normal, and Roberts and Stiehm [27] showed FMLP-i duced luminol-enhanced CL of purified neutrophils to be increased. In the present study we found both FMLPand OZP-induced luminol-enhanced CL of whole blood in the patients with cystic fibrosis to be much the same as that of the control subjects.…”

Virulence properties of Pseudomonas aeruginosa lacking the extreme-stress sigma factor AlgU (sigmaE)

“…The role of AlgU in resistance to superoxide and hypohalous ions is of potential relevance for the situation in the inflamed CF lung. The chronic infection in CF is notorious for the profuse infiltration of neutrophils which, among other noxious products, release enzymatically generated hypochlorous acid not only into phagosomes but also to the external environment (2,26,44). It is thus reasonable to assume that upregulation of algU-dependent systems may be beneficial under such conditions and could provide some selective advantage to P. aeruginosa.…”

“…The phenomena described in this work suggest a function for algU in P. aeruginosa pathogenesis besides its central role in alginate production. The role of AlgU in defense against reactive oxygen intermediates, which is independent of alginate production, as shown by the comparison of a wild-type parent and its algU and algD mutant derivatives, could play a significant protective role against such host products present in pathologically excessive amounts in the inflamed CF lung (38,44). In contrast, any contributions of such systems for the course of acute infection may be eclipsed by the ability of human phagocytic cells to control P. aeruginosa chiefly via oxygen-independent mechanisms (31, 37, 52) and by an apparent interference of algU expression with the factors or properties contributing to host mortality in systemic disease.…”