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伴有肺部并发症的原发性抗体缺陷:单中心经验

Primary Antibody Deficiencies With Pulmonary Complications: A Single-Center Experience.

作者信息

Özden Güzin, Pınar Deniz Pelin, Duru Cetinkaya Pelin

机构信息

Allergy and Immunology, Adana City Training and Research Hospital, Adana, TUR.

Department of Pulmonology, Çukurova University Faculty of Medicine, Adana, TUR.

出版信息

Cureus. 2022 Jan 12;14(1):e21140. doi: 10.7759/cureus.21140. eCollection 2022 Jan.

Abstract

Background Primary immunodeficiencies are a heterogeneous group of genetic diseases caused by one or more abnormalities in the immune system. Although pulmonary complications are common in patients with primary immunodeficiency diseases, these complications contribute significantly to morbidity and mortality. Aim The aim of our study was to evaluate the distribution of the features of pulmonary radiological involvement and demographic findings in this patient group. Materials and methods The files of patients who were treated and followed up with the diagnosis of primary immunodeficiency between 2014 and 2021 were analyzed retrospectively. Demographic data, symptoms, additional diseases, and computed tomography findings of the patients were recorded. Results The mean age of 32 cases was 37.34±13.54 (20-69) and the age of diagnosis was 28.90±15.75 (1-62). Twenty of the cases were male and 10 were female. The most common symptom was diarrhea with 53.1% and cough with 34.4%. The most common radiological finding is bronchiectasis in 75% of cases. Twenty-one (65.6%) of the cases had recurrent pneumonia before diagnosis and no pneumonia was observed after intravenous ımmunoglobulin replacement therapy. Three of the cases (9.4%) died during the follow-up. Conclusions Primary immunodeficiency should be considered in patients with bronchiectasis and a history of recurrent pneumonia, and further investigations should be performed. Early diagnosis of patients with primary immunodeficiency is very important for the early detection and treatment of malignancy and the interstitial lung disease that may develop.

摘要

背景 原发性免疫缺陷是一组由免疫系统中一个或多个异常引起的遗传性疾病。虽然肺部并发症在原发性免疫缺陷疾病患者中很常见,但这些并发症对发病率和死亡率有显著影响。目的 我们研究的目的是评估该患者群体中肺部影像学受累特征和人口统计学结果的分布情况。材料和方法 回顾性分析2014年至2021年间诊断为原发性免疫缺陷并接受治疗和随访的患者档案。记录患者的人口统计学数据、症状、其他疾病和计算机断层扫描结果。结果 32例患者的平均年龄为37.34±13.54岁(20 - 69岁),诊断年龄为28.90±15.75岁(1 - 62岁)。其中20例为男性,10例为女性。最常见的症状是腹泻(53.1%)和咳嗽(34.4%)。最常见的影像学表现是75%的病例出现支气管扩张。21例(65.6%)患者在诊断前有反复肺炎,静脉注射免疫球蛋白替代治疗后未观察到肺炎。3例(9.4%)患者在随访期间死亡。结论 对于有支气管扩张和反复肺炎病史的患者应考虑原发性免疫缺陷,并应进行进一步检查。原发性免疫缺陷患者的早期诊断对于早期发现和治疗可能发生的恶性肿瘤和间质性肺病非常重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b20c/8831950/878323c9b543/cureus-0014-00000021140-i01.jpg

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