原发性免疫缺陷儿童的非感染性肺部并发症。

IF 1.7 Q2 PEDIATRICS Clinical Medicine Insights-Pediatrics Pub Date : 2023-09-08 eCollection Date: 2023-01-01 DOI:10.1177/11795565231196431
Wimwipa Mongkonsritragoon, Ruma Srivastava, Divya Seth, Aishwarya Navalpakam, Pavadee Poowuttikul
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摘要

原发性免疫缺陷(PID)是一大类疾病,其特点是免疫功能缺陷,导致反复感染和免疫调节失调。每种疾病的临床表现、严重程度和并发症都不尽相同,这取决于受影响的免疫系统组成部分。当 PID 患者出现呼吸道症状时,应首先怀疑、检查并及时处理感染。然而,PID 的非感染性并发症也经常发生,并可能导致严重的发病率和死亡率。非感染性并发症可累及上呼吸道和下呼吸道系统,导致各种类似感染性疾病的表现。因此,临床医生应能够发现这些病症,并适当转诊至免疫科和肺科医生进行进一步治疗。在本文中,我们以病例为基础,回顾了原发性免疫缺陷患者非感染性肺部并发症的鉴别诊断、调查和多学科治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Non-infectious Pulmonary Complications in Children with Primary Immunodeficiency.

Primary immune deficiency (PID) is a large group of diseases characterized by defective immune function, leading to recurrent infections, and immune dysregulation. Clinical presentations, severity, and complications differ for each disease, based on the components of the immune system that are impacted. When patients with PID present with respiratory symptoms, infections should be initially suspected, investigated, and promptly managed. However, non-infectious complications of PID also frequently occur and can lead to significant morbidity and mortality. They can involve both the upper and lower respiratory systems, resulting in various presentations that mimic infectious diseases. Thus, clinicians should be able to detect these conditions and make an appropriate referral to an immunologist and a pulmonologist for further management. In this article, we use case-based scenarios to review the differential diagnosis, investigation, and multidisciplinary treatment of non-infectious pulmonary complications in patients with primary immune deficiencies.

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