Sherwani Poonam, Bhalla Ashu Seith, Jana Manisha, Naranje Priyanka, Kabra Sushil K, Gupta Arun Kumar, Kandasamy Devasenathipathy
Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, 110029, India.
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Indian J Pediatr. 2020 Oct;87(10):846-849. doi: 10.1007/s12098-020-03289-w. Epub 2020 May 8.
Primary immunodeficiency disorders (PIDD) are a group of disorders presenting with recurrent infections. The authors retrospectively reviewed the imaging records of 24 proven cases of PIDD and correlated the imaging findings with the type of defect. Final diagnoses were categorized in four groups; Group I (humoral immunodeficiency), Group 2 (cell mediated immunodeficiency), Group 3 (phagocytic disorders) and Group 4 (others). Group 3 was the commonest, followed by 1 and 2. Three most common disorders encountered were chronic granulomatous disease (CGD) (7/24), hyper IgE syndrome (5/24) and common variable immunodeficiency (CVID) (4/24). Consolidation (12/24), nodules (10/24), bronchiectasis (9/24) and lymphadenopathy (8/24) were the commonest imaging findings. Although not statistically significant, some definite imaging trends could be established. Presence of consolidation and nodules; with absence of bronchiectasis was the striking finding in Group 3. Group 2 disorders predominantly showed bronchiectasis, whereas presence of consolidation, nodules, bronchiectasis all were very common in Group 1.
原发性免疫缺陷病（PIDD）是一组以反复感染为特征的疾病。作者回顾性分析了24例确诊的PIDD患者的影像学记录，并将影像学表现与缺陷类型进行关联。最终诊断分为四组：第一组（体液免疫缺陷）、第二组（细胞介导免疫缺陷）、第三组（吞噬细胞疾病）和第四组（其他）。第三组最为常见，其次是第一组和第二组。最常见的三种疾病是慢性肉芽肿病（CGD）（7/24）、高IgE综合征（5/24）和普通变异型免疫缺陷病（CVID）（4/24）。实变（12/24）、结节（10/24）、支气管扩张（9/24）和淋巴结病（8/24）是最常见的影像学表现。尽管无统计学意义，但仍可确立一些明确的影像学趋势。实变和结节的存在；且无支气管扩张是第三组的显著表现。第二组疾病主要表现为支气管扩张，而实变、结节、支气管扩张在第一组中均很常见。
