[Liver abscess in chronic granulomatosis of childhood (author's transl)].
A thirteen-year-old boy with an abscess of the liver and recurrent infections since infancy is described. Aspergillus fumigatus could be isolated from this surgical treated abscess. Polymorphonuclear leukocytes (PMN) and monocytes from the patient show a normal phagocytosis, whereas the oxygen metabolism and the intracellular killing of kastalase-positive microorganismus (Staphylococcus aureus, Candida albicans) are strikingly decreased. In contrast, the catalase-negative Pneumococcus pneumoniae is rapidly killed. The iodination (131 J) of PMN is despite phagocytosis also markedly reduced. Myeloperoxidase could be detected in the phagocytes histochemically. No morphologic abnormalities of PMN have been found by electron microscopy. A defect of the humoral of cellular immunity has been excluded. The characterized dysfunction of PMN and monocytes as well as the clinical features of the patient are consistent with a diagnosis of chronic granulomatous disease of childhood.
D000293:Adolescent; D001228:Aspergillosis / Q000150:complications*; D001232:Aspergillus fumigatus; D006098:Granulocytes / Q000502:physiology / Q000648:ultrastructure; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D007109:Immunity; D008100:Liver Abscess / Q000150:complications*; D008297:Male; D008854:Microscopy, Electron; D009000:Monocytes / Q000502:physiology; D009504:Neutrophils / Q000502:physiology; D010587:Phagocytosis
Leberabszess bei chronischer Granulomatose des Kindesalters.