疑似原发性免疫缺陷患者的诊断方法
Diagnostic Approach to the Patients with Suspected Primary Immunodeficiency.
作者信息
Tavakol Marzieh, Jamee Mahnaz, Azizi Gholamreza, Sadri Homa, Bagheri Yasser, Zaki-Dizaji Majid, Mahdavi Fatemeh Sadat, Jadidi-Niaragh Farhad, Tajfirooz Sanaz, Kamali Ali N, Aghamahdi Fatemeh, Noorian Shahab, Kojidi Habibeh Taghavi, Mosavian Mehdi, Matani Rahman, Dolatshahi Elahe, Porrostami Kumars, Elahimehr Nasrin, Fatemi-Abhari Marzie, Sharifi Laleh, Arjmand Reza, Haghi Sabahat, Zainaldain Hamed, Yazdani Reza, Shaghaghi Mohammadreza, Abolhassani Hassan, Aghamohammadi Asghar
机构信息
Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.
Department of Allergy and Clinical Immunology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran.
出版信息
Endocr Metab Immune Disord Drug Targets. 2020;20(2):157-171. doi: 10.2174/1871530319666190828125316.
BACKGROUND AND OBJECTIVE
Primary immunodeficiency diseases (PIDs) are a group of more than 350 disorders affecting distinct components of the innate and adaptive immune systems. In this review, the classic and advanced stepwise approach towards the diagnosis of PIDs are simplified and explained in detail.
RESULTS
Susceptibility to recurrent infections is the main hallmark of almost all PIDs. However, noninfectious complications attributable to immune dysregulation presenting with lymphoproliferative and/or autoimmune disorders are not uncommon. Moreover, PIDs could be associated with misleading presentations including allergic manifestations, enteropathies, and malignancies.
CONCLUSION
Timely diagnosis is the most essential element in improving outcome and reducing the morbidity and mortality in PIDs. This wouldn't be possible unless the physicians keep the diagnosis of PID in mind and be sufficiently aware of the approach to these patients.
背景与目的
原发性免疫缺陷病（PID）是一组超过350种的疾病，影响先天性和适应性免疫系统的不同组成部分。在本综述中，对PID诊断的经典和先进的逐步方法进行了简化并详细解释。
结果
反复感染的易感性是几乎所有PID的主要标志。然而，由免疫失调引起的非感染性并发症，表现为淋巴增殖性和/或自身免疫性疾病，并不罕见。此外，PID可能与包括过敏表现、肠道病和恶性肿瘤在内的误导性表现相关。
结论
及时诊断是改善PID预后以及降低其发病率和死亡率的最关键因素。除非医生牢记PID的诊断并充分了解对这些患者的诊断方法，否则这是不可能实现的。
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