Defects in granulocyte function in various chromosome abnormalities (down's-, edwards'-, cri-du-chat syndrome)
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Summary
In five infants with autosomal aberrations and diminished resistance to infection (in spite of intact humoral and cellular immune mechanisms) several granulocyte functions (chemotaxis, phagocytosis, intracellular killing and metabolism of killing) were measured. A serum-dependent or a cell-dependent disturbance of phagocytosis of Candida albicans was found in two infants with cat-cry syndrome and one with trisomy 18. In one of these children there was an additional serum dependent defect of the killing of Candida albicans and of Staphylococcus aureus, serum levels of opsonins (IgG, IgM, CH50 and C3) being within normal range. An infant with trisomy 21 showed, in addition to a cellular defect of chemotaxis, a reduced cellular ability of the killing of Staphylococcus aureus and of Escherichia coli in autologous and AB-pool-serum. Phagocytosis of these bacteria remained normal.
Key words
Granulocytes Chemotaxis Phagocytosis Intracellular microbicidal activity Down's-, Edwards'-, Cri-du-chat SyndromeFunktionsdefekte der Granulocyten bei bestimmten Chromosomenanomalien (Down-, Edwards-, Cri-du-chat-Syndrom)
Zusammenfassung
Bei fünf Säuglingen mit Autosomenaberrationen, herabgesetzter Infektresistenz und intaktem humoralen und zellulären Immunapparat wurden die Granulocytenfunktionen Chemotaxis, Phagocytose, intrazelluläre Erregerabtötung und einige Stoffwechselvorgänge experimentell untersucht. Eine serumabhängige bzw. zelluläre Phagocytoseschwäche für Candida albicans bestand bei zwei Säuglingen mit Cri-du-chat-Syndrome und einem anderen mit Trisomie 18. Bei einem dieser Kinder bestand zusätzlich eine serumabhängige, verzögerte Erregerabtötung von Candida albicans sowie von Staphylococcus aureus, wobei die Serumspiegel der Opsonine IgG, IgM, CH 50 und C3 normal waren. Ein Säugling mit Trisomie 21 bot außer einem zellulären Chemotaxisdefekt ein verringertes zelluläres Abtötungsvermögen für Staphylococcus aureus sowie Escherichia coli im Eigen- und im Fremdserum. Die Phagocytose dieser Keime hingegen verlief ungestört.
Schlüsselwörter
Granulocyten Chemotaxis Phagocytose Intrazelluläre Keimabtötung Down-, Edwards, Cri-du-chat-SyndromPreview
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