Abé E, Belohradsky B H, Fink U, Wüst I
Fortschr Med. 1978 Oct 12;96(38):1910-2.
A 17-year-old boy demonstrated clinical and immunological signs of exogenic allergic alveolitis (farmer's lung). The patient grew up in the country and suffered from recurrent "pneumonia." In his serum precipitins were detected against Micropolyspora faeni, Aspergillus fumigatus and hay. Transbronchially obtained lung tissue showed histologically an inflammation of the epitheloid-cell-granulomatous type with fibrosis. A congenital defect of the neutrophilic function was suspected in view of the recurrent infections with significant polyclonal hypergammaglobulinemia, accompanied by leucocytosis and neutrophilia. In vitro studies of isolated patient's neutrophils showed that he suffered from chronic granulomatous disease. CGD should be considered in adolescents and adults with a history of severe infections and unexplained pulmonary fibrosis or pulmonary fibrosis simulating exogen allergic alveolitis.
一名17岁男孩表现出外源性过敏性肺泡炎(农民肺)的临床和免疫学症状。该患者在农村长大,患有复发性“肺炎”。在他的血清中检测到针对嗜热放线菌、烟曲霉和干草的沉淀素。经支气管获取的肺组织在组织学上显示为上皮样细胞肉芽肿型炎症伴纤维化。鉴于反复感染伴显著的多克隆高球蛋白血症,伴有白细胞增多和中性粒细胞增多,怀疑存在中性粒细胞功能先天性缺陷。对分离出的患者中性粒细胞进行的体外研究表明,他患有慢性肉芽肿病。对于有严重感染史以及不明原因的肺纤维化或类似外源性过敏性肺泡炎的肺纤维化的青少年和成年人,应考虑慢性肉芽肿病。