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Diagnostic Approach to the Patients with Suspected Primary Immunodeficiency

[ Vol. 20 , Issue. 2 ]

Author(s):

Marzieh Tavakol, Mahnaz Jamee, Gholamreza Azizi*, Homa Sadri, Yasser Bagheri, Majid Zaki-Dizaji, Fatemeh Sadat Mahdavi, Farhad Jadidi-Niaragh, Sanaz Tajfirooz, Ali N. Kamali, Fatemeh Aghamahdi, Shahab Noorian, Habibeh Taghavi Kojidi, Mehdi Mosavian, Rahman Matani, Elahe Dolatshahi, Kumars Porrostami, Nasrin Elahimehr, Marzie Fatemi-Abhari, Laleh Sharifi, Reza Arjmand, Sabahat Haghi, Hamed Zainaldain, Reza Yazdani, Mohammadreza Shaghaghi, Hassan Abolhassani and Asghar Aghamohammadi   Pages 157 - 171 ( 15 )

Abstract:


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.

Keywords:

Primary immunodeficiency disorders, infection, antibody deficiency, combined immunodeficiency, autoimmunity, enteropathy.

Affiliation:

Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Student Research Committee, Alborz University of Medical Sciences, Karaj, Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Clinical Research Development Unit (CRDU), 5 azar Hospital, Golestan University of Medical Sciences, Gorgan, Legal Medicine Research Center, Legal Medicine Organization, Tehran, Student Research Committee, Alborz University of Medical Sciences, Karaj, Immunology Research Center, Tabriz University of Medical Sciences, Tabriz, Department of Pediatrics, School of Medicine, Alborz University of Medical Sciences, Karaj, CinnaGen Medical Biotechnology Research Center, Alborz University of Medical Sciences, Karaj, Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Department of Pediatric Endocrinology, School of Medicine, Alborz University of Medical Sciences, Karaj, Department of Pediatric Endocrinology, School of Medicine, Alborz University of Medical Sciences, Karaj, Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Department of Gastroenterology and Hepatology, School of Medicine, Alborz University of Medical Sciences, Karaj, Department of Rheumatology, School of Medicine, Alborz University of Medical Sciences, Karaj, Department of Pediatrics, School of Medicine, Alborz University of Medical Sciences, Karaj, Department of Pediatrics, School of Medicine, Alborz University of Medical Sciences, Karaj, Department of Pediatrics, School of Medicine, Alborz University of Medical Sciences, Karaj, Uro- Oncology Research Center, Tehran University of Medical Sciences, Tehran, Department of Infectious Disease, School of Medicine, Alborz University of Medical Sciences, Karaj, Department of Hematology & Oncology, School of Medicine, Alborz university of medical sciences, Karaj, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins Hospital, Baltimore, MD, Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran

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