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机构地区:[1]暨南大学第一临床医学院,广东 广州 [2]暨南大学附属第一医院产科,广东 广州
出 处:《临床医学进展》2023年第6期9648-9654,共7页Advances in Clinical Medicine
摘 要:普通变异性免疫缺陷症(CVID)是一种以低免疫球蛋白血症、反复细菌感染为主要特征的异质性免疫缺陷综合征,临床罕见,易于漏诊、误诊。大量研究表明,不同的免疫和遗传缺陷参与了CVID的发病机制。然而,在大多数情况下,这种疾病的遗传背景仍未查明。本文对普通变异性免疫缺陷症的临床表现、发病机制、诊断和治疗进行归纳、总结,为该病的早期诊断、早期治疗提供依据,以减低误诊、漏诊率。Common variable immunodeficiency (CVID) is a rarely encountered heterogeneous immunodefi-ciency syndrome characterized mainly by low immuneglobulinemia and repeated bacterial infec-tions, easily to be missed and misdiagnosed. Numerous studies demonstrated that different immu-nological and genetic defects are involved in the pathogenesis of CVID. However, in most cases, the genetic background of the disease remains unidentified. This review aims to discuss different as-pects of CVID including clinical manifestations, pathogenesis, diagnosis and management of the disease and provide basis for early diagnosis and treatment of the disease, so as to reduce the rate of misdiagnosis and missed diagnosis.
关 键 词:普通变异性免疫缺陷症 临床表现 治疗
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