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体液原发性免疫缺陷病:成人患者的临床概述及胸部高分辨率计算机断层扫描(HRCT)特征

Humoral primary immunodeficiency diseases: clinical overview and chest high-resolution computed tomography (HRCT) features in the adult population.

作者信息

Cereser L, Girometti R, d'Angelo P, De Carli M, De Pellegrin A, Zuiani C

机构信息

Institute of Diagnostic Radiology, Azienda Sanitaria Universitaria Integrata di Udine, Udine, Italy.

Institute of Diagnostic Radiology, Department of Medicine, University of Udine, Udine, Italy.

出版信息

Clin Radiol. 2017 Jul;72(7):534-542. doi: 10.1016/j.crad.2017.03.018. Epub 2017 Apr 19.

Abstract

Humoral primary immunodeficiency diseases (hPIDs) are a heterogeneous group of hereditary disorders resulting in abnormal susceptibility to infections of the sinopulmonary tract. Some of these conditions (e.g., common variable immunodeficiency disorders [CVID]) imply a number of non-infectious thoracic complications such as non-infectious airway disorders, diffuse lung parenchymal diseases, and neoplasms. Chest high-resolution computed tomography (HRCT) is a key imaging tool to characterise and quantify the extent of underlying thoracic involvement, as well as to direct and monitor treatment. The aims of this review are to provide a brief clinical overview of hPIDs and describe the related chest HRCT imaging features in the adult population, with a special focus on CVID and its complications.

摘要

体液原发性免疫缺陷病(hPIDs)是一组遗传性疾病,具有异质性,可导致对鼻窦和肺部感染的易感性异常。其中一些病症(如常见变异型免疫缺陷病 [CVID])会引发一些非感染性胸部并发症,如非感染性气道疾病、弥漫性肺实质疾病和肿瘤。胸部高分辨率计算机断层扫描(HRCT)是一种关键的成像工具,可用于表征和量化潜在胸部受累的程度,以及指导和监测治疗。本综述的目的是简要介绍hPIDs的临床概况,并描述成年人群中相关的胸部HRCT成像特征,特别关注CVID及其并发症。

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