Defective neutrophil function in myotonic dystrophy.
Polymorphonuclear leukocytes (PMNs), obtained from 10 patients with myotonic dystrophy and 39 age-matched controls, were tested for chemotactic activity, chemiluminescence (CL), and phagocytosis. PMNs from 8 of the 10 patients had depressed chemotactic indexes (30 +/- 23) as compared to controls (61 +/- 18) (P less than 0.0003), and 6 of the 10 patients' PMNs produced lower peaks in CL (less than 90%) than those of controls. Phagocytosis and bactericidal activity were evaluated in 7 patients and were normal. Control PMNs incubated in patient serum produced normal chemotactic activity and CL. These results indicate defects in 2 PMN membrane-associated activities in myotonic dystrophy and suggest that the PMN is affected by the generalized membrane abnormality underlying myotonic dystrophy.
D000293:Adolescent; D000328:Adult; D002634:Chemotaxis, Leukocyte; D005260:Female; D006801:Humans; D008163:Luminescent Measurements; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000097:blood*; D009504:Neutrophils / Q000187:drug effects / Q000502:physiology*; D010587:Phagocytosis; D015054:Zymosan / Q000494:pharmacology
D015054:Zymosan