U Karagol | PubMed Health

PubMed1986

Neutrophil function in Duchenne muscular dystrophy.

Spontaneous migration, chemotactic and bactericidal activity and nitroblue-tetrazolium (NBT) reduction by neutrophils from patients with Duchenne muscular dystrophy were evaluated using quantitative techniques. Significant reductions in spontaneous migration and chemotaxis were found in the DMD group when compared with a control group of subjects. No significant differences were found in bactericidal activity or NBT reduction. The findings suggest that there is a defect in the contractile system or cell membrane function of neutrophils in DMD and that the genetic defect is expressed in leucocytes as well as other tissues.

Adolescent↗

PubMed1983

Lymphocyte capping in myotonic dystrophy.

Lymphocyte capping with antihuman immunoglobulin was studied in 16 cases of myotonic dystrophy, including two cases with the congenital form. Percentage capping after 1 hour incubation was reduced and the time course of the capping sequence was apparently delayed. The significance of these findings remains to be determined.

Adolescent↗

PubMed1983

Immunological studies in myotonic dystrophy.

Abnormalities of cellular and humoral immunity have been recognised in myotonic dystrophy but their significance is uncertain. To throw further light on this, a group of 22 patients with myotonic dystrophy was investigated, looking specifically for evidence of abnormalities of B lymphocyte function and of disturbed immunoregulation. The previously reported reduction in serum IgG levels was confirmed and, in addition, serum IgA levels were significantly reduced. Autoantibodies to muscle and non-muscle antigens were not found and there was no increase in autoimmune disease or malignancy, suggesting that immunoregulatory mechanisms are not disturbed. The proportions of T and B cells were not significantly different in patients and controls. B-lymphocyte capping with FITC- antihuman IgG was quantitatively altered, the percentage of capped cells after incubation being reduced and the time-course of the capping process being apparently prolonged in the myotonic dystrophy subjects. While there are a number of possible explanations for these findings, they may reflect an intrinsic abnormality of the lymphocyte membrane. This could be part of a more generalised membrane defect which has been suggested to underlie the myotonia and certain other manifestations of this multisystemic disorder.

Adolescent↗