Abstract
A variety of phagocytic cell and lymphocyte assays were employed to evaluate the immune status of four patients with methylmalonicaciduria. One patient had a depressed absolute granulocyte count and two patients had depressed neutrophil and monocyte chemotactic responses. All subjects had normal neutrophil phagocytic and bactericidal activities. One patient had a decreased T-cell number; blastogenic responses to phytohaemagglutinin and pokeweed mitogen were normal in all subjects. B lymphocyte measurements were variably abnormal; two children had decreased B-cell numbers; two had marginally decreased IgG levels; a third had an undetectable rubella titre; and two had elevated serum IgE concentrations.In vitro exposure of normal cells to methylmalonic acid concentrations up to 50mg/100 ml did not affect chemotactic or lymphoproliferative responses.
In conclusion, although B-cell function may be affected, no consistent abnormality of lymphocyte or phagocytic cell functions could be attributed to the metabolic disorder.
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Church, J.A., Koch, R., Shaw, K.N.F. et al. Immune functions in methylmalonicaciduria. J Inherit Metab Dis 7, 12–14 (1984). https://doi.org/10.1007/BF01805612
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DOI: https://doi.org/10.1007/BF01805612