Mahdaviani Seyed Alireza, Darougar Sepideh, Mansouri Davood, Tashayoie-Nejad Sabereh, Movahedi Mahshid, Aghdam Karim Rahimi, Ghaffaripour Hosseinali, Baghaie Nooshin, Hassanzad Maryam, Eslaminejad Alireza, Fakharian Atefeh, Pourdowlat Guitti, Heshmatnia Jalal, Bakhshayeshkaram Mehrdad, Boloursaz Mohammadreza, Tabarsi Payam, Hashemitari Seyed Karen, Velayati Ali Akbar
Pediatric Respiratory Diseases Research Centre, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Clinical Tuberculosis and Epidemiology Research Centre, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Interv Med Appl Sci. 2019 Mar;11(1):1-7. doi: 10.1556/1646.10.2018.49.
Respiratory infections are expressed very soon in the life in humoral immunodeficiencies and often lead to chronic irreversible complications such as bronchiectasis and chronic airflow limitation. This study was conducted to evaluate the pulmonary complications of predominantly antibody immunodeficiencies to show the benefits of timely diagnosis and appropriate therapy.
The information of 48 patients involved with a type of predominantly antibody immunodeficiencies, including sex, type of primary immunodeficiency, age at the onset of symptoms, age at diagnosis, recurrent infections, respiratory symptoms, and pulmonary radiological and functional abnormalities were recorded and analyzed.
In 48 patients evaluated, the mean age at diagnosis was 25.63 years. The mean diagnostic delay was estimated to be 13.62 years. The most recurring clinical manifestations, sinusitis (69.6%), otitis (43.5%), and recurrent pneumonia were the cause of frequent admissions in 68.8% of these patients. Bronchiectasis was frequently found (58.3%) in these patients mostly involving the middle and lower lobes (48.8% and 41.5%, respectively).
Respiratory complications, infectious or non-infectious, determine the prognosis of the disease in patients with predominantly antibody immunodeficiencies. Timely diagnosis and appropriate management may improve life expectancy and the quality of life in these patients.
呼吸道感染在体液免疫缺陷患者的生命早期就会出现,并且常常导致慢性不可逆并发症,如支气管扩张和慢性气流受限。本研究旨在评估以抗体缺陷为主的免疫缺陷患者的肺部并发症,以显示及时诊断和适当治疗的益处。
记录并分析了48例以抗体缺陷为主的免疫缺陷患者的信息,包括性别、原发性免疫缺陷类型、症状出现年龄、诊断年龄、反复感染、呼吸道症状以及肺部影像学和功能异常情况。
在评估的48例患者中,诊断时的平均年龄为25.63岁。估计平均诊断延迟为13.62年。最常见的临床表现为鼻窦炎(69.6%)、中耳炎(43.5%),68.8%的患者因反复肺炎频繁入院。这些患者中经常发现支气管扩张(58.3%),主要累及中叶和下叶(分别为48.8%和41.5%)。
呼吸道并发症,无论是感染性还是非感染性的,都决定了以抗体缺陷为主的免疫缺陷患者的疾病预后。及时诊断和适当管理可能会改善这些患者的预期寿命和生活质量。