免疫和侵入性真菌感染的先天性错误:呈现和管理
Olivier Paccoud1, Adilia Warris2, Anne Puel3,4,5
1Université Paris Cité, Department of Infectious Diseases and Tropical Medicine, Necker - Enfants Malades University Hospital, Assistance Publique - Hôpitaux de Paris (AP-HP), IHU Imagine, Paris, France.
Current opinion in infectious diseases
|September 11, 2024
概括
免疫的先天性错误增加了对侵入性真菌感染的易感性,如阿斯伯吉洛症. 管理需要量身定制的抗真菌治疗,预防和免疫调节,在严重病例中考虑干细胞移植.
科学领域:
- 免疫学 免疫学 免疫学
- 传染性疾病 传染性疾病
- 遗传学 是一个遗传学.
背景情况:
- 免疫的先天性错误 (IEI) 会使个体易于感染.
- 侵入性真菌感染 (IFI) 对免疫功能低下的患者构成重大风险.
- 特定的IEI与真菌病原体有独特的关联.
研究的目的:
- 在IEI患者中审查IFI的临床表现.
- 概述这一群体中IFI的管理策略,包括抗真菌疗法,预防和免疫调节.
主要方法:
- 在选定的IEI中对IFI的临床表现和管理进行文献综述.
- 对特定IEI-真菌病原体关联的分析.
- 对治疗结果的评估,包括抗真菌使用和造血干细胞移植 (HSCT).
主要成果:
- 慢性颗粒状体疾病 (CGD) 和信号传感器和转录3激活器 (STAT3) 缺乏与阿斯伯吉洛症有关;建议采用模具活性预防.
- 在STAT1功能增益突变中,侵入性真菌感染是罕见的.
- 与酶相关的招募域含蛋白9 (CARD9) 缺乏症与各种真菌感染有关;经常需要长时间的抗真菌治疗.
- 对于CGD来说,考虑HSCT,但对于其他IEI与活跃的IFI来说,结果有所不同.
结论:
- 具有明显的临床和放射性特征的IEI对IFI产生敏感性.
- 在IEI患者中管理IFI是具有挑战性的,需要预防,抗真菌药物和免疫调节的综合方法.
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