Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Department of Health and Human Services, Bethesda, MD 20892-1684, USA.
Clin Rev Allergy Immunol. 2010 Feb;38(1):3-10. doi: 10.1007/s12016-009-8136-z.
Chronic granulomatous disease (CGD) was first described in the 1950s and has become a paradigm for genetic neutrophil diseases. It is characterized by recurrent infections with a narrow spectrum of bacteria and fungi as well as a common set of inflammatory complications most notably including inflammatory bowel disease. Over the last half century major advances in management have profoundly altered the major clinical issues and the life expectancy of CGD. With X-linked and autosomal recessive forms, it has been an important disease for the development of bone marrow transplantation and gene therapy. Some of the recent developments in infectious syndromes, inflammatory complications, and curative approaches are discussed in this review.
慢性肉芽肿病(CGD)于 20 世纪 50 年代首次被描述,现已成为遗传性中性粒细胞疾病的范例。其特征是反复发生特定谱的细菌和真菌感染,以及一组常见的炎症并发症,其中最显著的是炎症性肠病。在过去的半个世纪中,管理方面的重大进展极大地改变了 CGD 的主要临床问题和预期寿命。X 连锁和常染色体隐性遗传形式使 CGD 成为骨髓移植和基因治疗发展的重要疾病。本文就感染综合征、炎症并发症和治疗方法的一些最新进展进行了讨论。