抗体免疫缺陷病成年患者的呼吸系统表现  

Primary antibody deficiencies and respiratory manifestations in adults

作　　者：钟佳成 王凌伟 Zhong Jiacheng;Wang Lingwei(Department of Respiratory and Critical Care Medicine,Shenzhen People′s Hospital(The Second Clinical Medical College,Jinan University the First Affiliated Hospital,Southern University of Science and Technology),Shenzhen Institute of Respiratory Diseases in Shenzhen People′s Hospital,Shenzhen 518020,China)

机构地区：[1]深圳市人民医院呼吸与危重症医学科,暨南大学第二临床医学院,南方科技大学附属第一医院,深圳市呼吸疾病研究所,深圳518020

出　　处：《中华结核和呼吸杂志》2024年第4期388-392,共5页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：国家科技部重点研发计划(2022YFF0710800,2022YFF0710802);深圳市科技创新委员会可持续发展专项(KCXFZ202002011008256)。

摘　　要：抗体免疫缺陷病是一组由遗传缺陷引起的先天性疾病,影响机体免疫防御机制的发育和功能。患者可表现为反复感染、淋巴细胞增殖、自身免疫性疾病、自身炎症或恶性肿瘤。呼吸系统可表现为支气管扩张症、支气管哮喘、间质性肺疾病等。深入了解抗体免疫缺陷病将有助于将其从呼吸系统常见疾病中甄别出来。Primary antibody deficiencies(PAD)are a group of congenital disorders caused by genetic defects that affect the development and function of the body′s immune defence mechanisms.Patients with PAD may present with recurrent infections,lymphoproliferation,autoimmune diseases,autoinflammation,or malignancies.Respiratory system manifestations may include bronchiectasis,bronchial asthma,and interstitial lung disease,among others.A comprehensive understanding of PADs will help to distinguish these covert cases from more common respiratory diseases.

关 键 词：支气管扩张症 机体免疫防御 间质性肺疾病 自身免疫性疾病 先天性疾病 支气管哮喘 淋巴细胞增殖 遗传缺陷 

分 类 号：R593[医药卫生—内科学]