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

Abstract

Neutrophils purified from peripheral blood of patients with the Shwachman syndrome show enhanced chemiluminescence (CL) and depressed chemotaxis. Here we present data showing that the increased CL response can be demonstrated by using a whole blood CL assay. This assay is well-suited for studies in infants, because the blood sample volumes needed are small. Increase in CL was most distinct in the initial (1 min) activation induced by N-formyl-methionyl-leucyl-phenylalanine. The 1-min response is considered to derive from extracellular production of oxygen radicals. Such an extracellular oxygen radical production may render the patients susceptible to undue oxidant stress. We therefore treated the patients with two antioxidants, thiamine and alpha-tocopherol, for 3 months. This supplementation, however, failed to exert any significant effect on either whole blood CL or migration of the patients' neutrophils under agarose.

Effect of Stobadine on Leukocyte Free Radical Generation in Streptozotocin-Diabetic Rats: Comparison with Vitamin E

“…These observations are in agreement with the finding that the CL was not affected in premature infants and adults after oral vitamin E supplementation [8]. In another study [9], ·-tocopherol supplementation for 3 months failed to exert any significant effect on either whole blood CL or migration of the patients' neutrophils. On the other hand, a decrease in the CL has also been reported in isolated leukocytes after oral supplementation with vitamin E in volunteers [10].…”

Reactive oxygen species of neutrophils from patients with monosomy 7 in the bone marrow: Contradictory chemiluminescence activity by whole blood or by purified cells

“…The results of the present study show that in monosomy 7 the determination of the production of ROS of neutrophils by whole blood or by purified neutrophils gives contradictory results. Our modification of CL by whole blood has previously given results similar to those obtained using purified neutrophils, when we have studied patients with seronegative spondyloarthropathies (1 8) or neutropenic patients with the Schwachman syndrome (19). The present study provides further evidence that the CL response of healthy neutrophils is not exaggerated even at low neutrophil counts by the whole blood assay that we have used.…”

Neutrophil free oxygen radical production and blood total antioxidant capacity in patients with coronary heart disease using various medicationsNote

“…Furthermore, the effect of antioxidant supplementation can be paradoxical with respect to the production of ROS. In our earlier study, the use of vitamin E and thiamine increased rather than decreased the production of ROS by neutrophils in whole blood (15).…”

“…the degree of both generation and scavenging of phagocytederived ROS in vivo. In this setting, the use of whole blood assays in evaluation of ROS production is crucial because phagocyte purification procedures potentially distort the results (15,16) by causing cell loss and contamination of the cells by ubiquitous bacterial lipopolysaccharides which can alter phagocyte function. The TRAP/ neutrophil chemiluminescence ratio used in the present study as a marker of the redox balance represents a composite parameter which is related to cellular ROS production capacity and composite antioxidant capacity of the cells and plasma.…”