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PubMed · 6429434

Immune functions in methylmalonicaciduria.

J A Church·R Koch·K N Shaw·C A Nye·G N Donnell
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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 50 mg/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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AcidosisChildChild, PreschoolFemaleHumansImmunity, CellularImmunoglobulin AImmunoglobulin MImmunologic Deficiency SyndromesIntellectual DisabilityLeukocyte CountMaleMalonatesMethylmalonic AcidPhagocytosisJournal ArticleResearch Support, Non-U.S. Gov'tResearch Support, U.S. Gov't, Non-P.H.S.

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J A Church, R Koch, K N Shaw, C A Nye, G N Donnell. 1984. Immune functions in methylmalonicaciduria.. https://doi.org/10.1007/bf01805612

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