Wildfeuer A, Krawinkel M, Lange C E, Voigt W H
Klin Padiatr. 1980 May;192(3):241-8. doi: 10.1055/s-2008-1035588.
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.
本文描述了一名13岁男孩，自婴儿期起就患有肝脓肿并反复感染。从该手术治疗的脓肿中分离出烟曲霉。患者的多形核白细胞（PMN）和单核细胞表现出正常的吞噬作用，而氧代谢以及对过氧化氢酶阳性微生物（金黄色葡萄球菌、白色念珠菌）的细胞内杀伤作用显著降低。相比之下，过氧化氢酶阴性的肺炎链球菌被迅速杀死。尽管PMN发生了吞噬作用，但其碘化（131J）也明显减少。通过组织化学方法可在吞噬细胞中检测到髓过氧化物酶。电子显微镜检查未发现PMN有形态学异常。已排除体液或细胞免疫缺陷。PMN和单核细胞的特征性功能障碍以及患者的临床特征与儿童慢性肉芽肿病的诊断相符。
