Segal B H, DeCarlo E S, Kwon-Chung K J, Malech H L, Gallin J I, Holland S M
Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892-1886, USA.
Medicine (Baltimore). 1998 Sep;77(5):345-54. doi: 10.1097/00005792-199809000-00004.
Chronic granulomatous disease (CGD) is a rare inherited disorder of the NADPH oxidase complex in which phagocytes are defective in generating reactive oxidants. As a result, patients with CGD suffer from recurrent bacterial and fungal infections. The most common fungal infections are caused by Aspergillus species. Aspergillus nidulans is a rare pathogen in most patient populations with quantitative or qualitative neutrophil defects. We have reviewed all cases in which A. nidulans was isolated from patients at the National Institutes of Health (Bethesda, MD) between 1976 and 1997. A. nidulans infection occurred in 6 patients with CGD, but was not a pathogen in any other patient group. Aspergillus fumigatus was a more common pathogen in CGD compared with A. nidulans, but A. nidulans was more virulent. A. nidulans was significantly more likely to result in death compared with A. fumigatus, to involve adjacent bone, and to cause disseminated disease. Patients with A. nidulans received longer courses of amphotericin B therapy than patients with A. fumigatus, and were treated with surgery more often. In contrast to A. fumigatus, A. nidulans was generally refractory to intensive antifungal therapy, suggesting that early surgery may be important. These data show that A. nidulans is a distinct pathogen in CGD and its isolation carries more severe implications than that of A. fumigatus.
慢性肉芽肿病（CGD）是一种罕见的遗传性NADPH氧化酶复合物疾病，其中吞噬细胞在产生活性氧化剂方面存在缺陷。因此，CGD患者会反复遭受细菌和真菌感染。最常见的真菌感染由曲霉菌引起。在大多数有定量或定性中性粒细胞缺陷的患者群体中，构巢曲霉是一种罕见的病原体。我们回顾了1976年至1997年间在美国国立卫生研究院（马里兰州贝塞斯达）从患者中分离出构巢曲霉的所有病例。6例CGD患者发生了构巢曲霉感染，但在任何其他患者群体中它都不是病原体。与构巢曲霉相比，烟曲霉在CGD中是更常见的病原体，但构巢曲霉的毒性更强。与烟曲霉相比，构巢曲霉导致死亡、累及相邻骨骼和引起播散性疾病的可能性显著更高。感染构巢曲霉的患者接受两性霉素B治疗的疗程比感染烟曲霉的患者更长，且更常接受手术治疗。与烟曲霉不同，构巢曲霉通常对强化抗真菌治疗耐药，这表明早期手术可能很重要。这些数据表明，构巢曲霉在CGD中是一种独特的病原体，其分离所带来的影响比烟曲霉更为严重。
