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慢性肉芽肿病(CGD):常见相关病原体、诊断与治疗

Chronic Granulomatous Disease (CGD): Commonly Associated Pathogens, Diagnosis and Treatment.

作者信息

Justiz-Vaillant Angel A, Williams-Persad Arlene Faye-Ann, Arozarena-Fundora Rodolfo, Gopaul Darren, Soodeen Sachin, Asin-Milan Odalis, Thompson Reinand, Unakal Chandrashekhar, Akpaka Patrick Eberechi

机构信息

Department of Paraclinical Sciences, Faculty of Medical Sciences, The University of the West Indies, St. Augustine, Trinidad and Tobago.

Eric Williams Medical Sciences Complex, North Central Regional Health Authority, Champs Fleurs, Trinidad and Tobago.

出版信息

Microorganisms. 2023 Sep 5;11(9):2233. doi: 10.3390/microorganisms11092233.

Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by a defect in the phagocytic function of the innate immune system owing to mutations in genes encoding the five subunits of the nicotinamide adenine dinucleotide phosphatase (NADPH) oxidase enzyme complex. This review aimed to provide a comprehensive approach to the pathogens associated with chronic granulomatous disease (CGD) and its management. Patients with CGD, often children, have recurrent life-threatening infections and may develop infectious or inflammatory complications. The most common microorganisms observed in the patients with CGD are , spp., spp., spp., spp., spp., and spp. Antibacterial prophylaxis with trimethoprim-sulfamethoxazole, antifungal prophylaxis usually with itraconazole, and interferon gamma immunotherapy have been successfully used in reducing infection in CGD. Haematopoietic stem cell transplantation (HCT) have been successfully proven to be the treatment of choice in patients with CGD.

摘要

慢性肉芽肿病(CGD)是一种原发性免疫缺陷病,由先天性免疫系统吞噬功能缺陷引起,这是由于编码烟酰胺腺嘌呤二核苷酸磷酸酶(NADPH)氧化酶复合物五个亚基的基因突变所致。本综述旨在提供一种全面的方法来应对与慢性肉芽肿病(CGD)相关的病原体及其管理。CGD患者,通常为儿童,会反复发生危及生命的感染,并可能出现感染性或炎症性并发症。在CGD患者中观察到的最常见微生物是金黄色葡萄球菌、曲霉菌属、念珠菌属、伯克霍尔德菌属、诺卡菌属、沙门菌属和分枝杆菌属。用甲氧苄啶-磺胺甲恶唑进行抗菌预防,通常用伊曲康唑进行抗真菌预防,以及干扰素γ免疫疗法已成功用于减少CGD患者的感染。造血干细胞移植(HCT)已被成功证明是CGD患者的首选治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a69e/10534792/8843df8949fa/microorganisms-11-02233-g001.jpg

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