Section of Immunology, Allergy and Rheumatology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston.
Vanderbilt Vaccine Research Program and Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt University School of Medicine and Medical Center, Nashville, Tennessee.
J Pediatric Infect Dis Soc. 2018 May 9;7(suppl_1):S2-S5. doi: 10.1093/jpids/piy008.
Chronic Granulomatous Disease is one of the classic primary immunodeficiencies of childhood. While the incidence and severity of bacterial and fungal infections have been greatly reduced in this patient population, much remains to be learned about the pathophysiology of the disease, particularly for autoinflammatory manifestations. In this review, we examine the epidemiology, pathophysiology, and genetic basis for CGD.
慢性肉芽肿病是儿童期经典的原发性免疫缺陷病之一。虽然该患者人群的细菌和真菌感染的发生率和严重程度已经大大降低,但对于该疾病的病理生理学,特别是自身炎症表现,仍有许多需要了解。在这篇综述中,我们研究了 CGD 的流行病学、病理生理学和遗传基础。