National Consensus on Diagnosis and Management Guidelines for Primary Immunodeficiency

Document Type: Review

Authors

1 Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden

2 Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran

3 Pediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

4 Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran

5 Department of Allergy and Clinical Immunology, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran

6 Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran, and the University of Medical Science, Tehran, Iran

7 Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran

8 Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran

9 Hematology, Oncology and Stem Cell Transplantation Research Centre, Tehran University of Medical Sciences, Tehran, Iran

10 Department of Allergy and Clinical Immunology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran

11 Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

12 Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.

13 Department of Pediatrics, Bo-Ali children's Hospital of Ardabil University of Medical Sciences, Ardabil, Iran

14 Acquired Immunodeficiency Research Center, lsfahan University of Medical Sciences, Isfahan, lran

15 Allergy and Clinical Immunology Department, Bushehr University of Medical Science, School of Medicine, Bushehr, Iran

16 Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

17 Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

18 Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran

19 Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran

20 Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran

21 Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran

22 Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran

23 Department of Pediatrics, School of Medicine, Arak University of Medical Sciences, Arak, Iran

24 Department of Pediatrics, Hamadan University of Medical Sciences, Hamadan, Iran

25 Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran

26 Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

27 Cellular & Molecular Research Center, Kurdistan University of Medical Sciences, Sanandaj, Iran

28 Department of Pediatrics, Kermanshah University of Medical Sciences, Kermanshah, Iran

29 Department of Pediatrics, Qom University of Medical Sciences, Qom, Iran

30 Department of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran

31 Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran

32 Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD)

33 Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran

34 Mousavi Hospital, Zanjan University of Meical Sciences, Zanjan, Iran

35 Department of Immunology and Allergy, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

36 Department of Immunology and Allergy, Semnan University of Medical Sciences, Semnan, Iran

37 Department of Immunology and Allergy, Kashan University of Medical Sciences, Kashan, Iran

38 Department of Immunology and Allergy, Qazvin University of Medical Sciences, Qazvin, Iran

39 Department of Immunology and Allergy, Ilam University of Medical Sciences, Ilam, Iran

40 Department of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran

41 Department of Immunology and Allergy, Shahrekord University of Medical Sciences, Shahrekord , Iran

42 Department of Pediatrics, Lorestan University of Medical Sciences, Khorramabad, Iran

43 Department of Internal Medicine Shahid Sadoughi University of Medical Sciences, Yazd, Iran

44 Pediatric Infectious Diseases Research Center, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran

45 Iranian Primary Immunodeficiencies Network (IPIN), Tehran University of Medical Science, Tehran, Iran

Abstract

Primary immunodeficiency (PID) is a group of more than 400 distinct genetic disorders affecting both children and adults. As signs and symptoms of PID are usually heterogeneous and unspecific, based on the available human resources and laboratory facilities, diagnosis and follow-up of these patients can be challenging. In line with this notion, management and treatment of PID are practiced divergently in different PID centers. In order to reach a distinct national protocol and given the little evidence on appropriate or universal guidelines to improve the current status of management of the disease, the Iranian PID network designed a consensus suitable for regional resources. This review summarizes this PID guideline based on the importance of different clinical complications and the level of medical authority visiting those at the first line. Further, for each complication, appropriate interventions are mentioned to improve the approach.

Keywords


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