Abstract
We previously demonstrated abnormal opsonized zymosan (OZ) and concanavalin A (con-A) stimulated chemiluminescence (CL) in purified suspensionsof neutrophils (PMN), PMN membranes, erythrocytes (RBC), RBC membranes, and T-lymphocytes from patients with chronic granulomatous disease (CGD), suggesting generalization of the PMN membrane defect to other cell types (Ped. Res. 17: 1022, 1983). Duchenne muscular dystrophy (DMD) resembles CGD in its X-linked transmission, carrier mosaicism, membrane localization of the metabolic defect, and similarity to the rare McLeod phenotype, a state of weakened antigenicity of Kell RBC antigens and absence of leukocyte and RBC Kx antigen. We therefore studied the CL responses of intact and membrane preparations from PMN and RBC, to OZ and con-A stimulation, in 10 males with DMD. Results are expressed as peak stimulated response in counts per minute ± standard error of the mean × 10−3:
The statistically significant*, stimulus restricted defect in RBC membrane CL is consistent with other reported abnormalities in the RBC of DMD subjects and may relate to disruption of membrane respiratory metabolism, analagous to CGD.
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Peerless, A., Stiehm, E. ERYTHROCYTE CHEMILUMINESCENCE DEFECT IN DUCHENNE MUSCULAR DYSTROPHY AND CHRONIC GRANULOMATOUS DISEASE. Pediatr Res 18 (Suppl 4), 381 (1984). https://doi.org/10.1203/00006450-198404001-01730
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DOI: https://doi.org/10.1203/00006450-198404001-01730