The Middle East and North Africa Diagnosis and Management Guidelines for Inborn Errors of Immunity
Safa Baris 1, Hassan Abolhassani 2, Michel J Massaad 3, Maryam Al-Nesf 4, Zahra Chavoshzadeh 5, Sevgi Keles 6, Ismail Reisli 6, Azzeddine Tahiat 7, Hiba Mohammad Shendi 8, Dalia Abd Elaziz 9, Brahim Belaid 10, Fatima Al Dhaheri 11, Sule Haskologlu 12, Figen Dogu 12, Imen Ben-Mustapha 13, Ali Sobh 14, Nermeen Galal 9, Safa Meshaal 15, Rabab Elhawary 15, Aisha El-Marsafy 9, Fayhan J Alroqi 16, Bandar Al-Saud 17, Mona Al-Ahmad 18, Tariq Al Farsi 19, Nashat Al Sukaiti 19, Salem Al-Tamemi 20, Cybel Mehawej 21, Ghassan Dbaibo 22, Gehad ElGhazali 23, Sara Sebnem Kilic 24, Ferah Genel 25, Ayca Kiykim 26, Ugur Musabak 27, Hasibe Artac 28, Sukru Nail Guner 6, Rachida Boukari 29, Reda Djidjik 10, Nadia Kechout 30, Deniz Cagdas 31, Zeinab Awad El-Sayed 32, Elif Karakoc-Aydiner 33, Raed Alzyoud 34, Mohamed Ridha Barbouche 13, Mehdi Adeli 35, Rima Hanna Wakim 22, Shereen M Reda 32, Aydan Ikinciogullari 12, Ahmet Ozen 33, Aziz Bousfiha 36, Hamoud Al-Mousa 17, Nima Rezaei 37, Waleed Al-Herz 38, Raif S Geha 39
Affiliations
Affiliations
- Faculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Istanbul, Turkey; The Isil Berat Barlan Center for Translational Medicine, Istanbul Jeffrey Modell Foundation Diagnostic Center for Primary Immune Deficiencies, Istanbul, Turkey. Electronic address: safabaris@hotmail.com.
- Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Division of Clinical Immunology, Department of Biosciences and Nutrition, Karolinska Institute, Karolinska University Hospital, Huddinge, Stockholm, Sweden.
- Department of Experimental Pathology, Immunology, and Microbiology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon; Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, American University of Beirut Medical Center, Beirut, Lebanon; Center for Infectious Diseases Research (CIDR) and WHO Collaborating Center for Reference and Research on Bacterial Pathogens, American University of Beirut, Beirut, Lebanon.
- Allergy and Immunology Division, Department of Medicine, Hamad Medical Corporation, Doha, Qatar.
- Allergy and Clinical Immunology Department, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
- Division of Pediatric Allergy and Immunology, Meram Faculty of Medicine, Necmettin Erbakan University, Konya, Turkey.
- Laboratory of Immunology, Department of Medical Biology, University of Algiers, Rouiba Hospital, Algiers, Algeria.
- Division of Pediatric Allergy and Immunology, Tawam Hospital, Abu Dhabi, United Arab Emirates.
- Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
- Department of Medical Immunology, Beni Messous University Hospital Center, Faculty of Pharmacy, University of Algiers, Algiers, Algeria.
- Department of Pediatrics, Pediatric Infectious Diseases, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates.
- Department of Pediatric Immunology and Allergy, Ankara University School of Medicine, Ankara, Turkey.
- Department of Immunology, Institut Pasteur de Tunis and University Tunis El Manar, Tunis, Tunisia.
- Department of Pediatrics, Mansoura University Children's Hospital, Faculty of Medicine, Mansoura University, Mansoura, Egypt.
- Clinical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
- 16King Abdullah International Medical Research Center, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
- Department of Pediatrics, Division of Allergy and Immunology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia; College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
- Department of Microbiology, College of Medicine, Kuwait University, Kuwait.
- Department of Pediatric Allergy and Clinical Immunology, The Royal Hospital, Muscat, Oman.
- Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
- Department of Human Genetics, Gilbert and Rose-Marie Chagoury School of Medicine, Lebanese American University, Byblos, Lebanon.
- Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, American University of Beirut Medical Center, Beirut, Lebanon; Center for Infectious Diseases Research (CIDR) and WHO Collaborating Center for Reference and Research on Bacterial Pathogens, American University of Beirut, Beirut, Lebanon.
- Department of Immunology, Sheikh Khalifa Medical City-Union 71-Purehealth, Abu Dhabi, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates.
- Department of Pediatric Immunology and Rheumatology, Faculty of Medicine, Bursa Uludag University, Bursa, Turkey.
- Department of Pediatric Immunology and Allergy, University of Health Sciences Dr. Behcet Uz Children's Hospital, İzmir, Turkey.
- Division of Pediatric Allergy and Immunology, Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey.
- Department of Internal Medicine, Division of Immunology and Allergy, Faculty of Medicine, Baskent University, Ankara, Turkey.
- Division of Immunology and Allergy, Department of Pediatrics, Faculty of Medicine, Selcuk University, Konya, Turkey.
- Department of Pediatrics, Mustapha Pacha Faculty of Medicine, Algiers University, Algeria.
- Department of Immunology, Pasteur Institute of Algeria, Faculty of Medicine, Algiers, Algeria.
- Department of Pediatrics, Section of Pediatric Immunology, Ihsan Dogramaci Children's Hospital, Institute of Child Health, Hacettepe University Medical School, Ankara, Turkey.
- Pediatric Allergy, Immunology and Rheumatology Unit, Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
- Faculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Istanbul, Turkey; The Isil Berat Barlan Center for Translational Medicine, Istanbul Jeffrey Modell Foundation Diagnostic Center for Primary Immune Deficiencies, Istanbul, Turkey.
- Section of Immunology, Allergy and Rheumatology, Queen Rania Children Hospital, Amman, Jordan.
- Department of Immunology, Sidra Medicine, Ar-Rayyan, Qatar.
- Laboratory of Clinical Immunology, Inflammation and Allergy, Faculty of Medicine and Pharmacy of Casablanca, Department of pediatric infectious and immunological diseases, Ibn Rushd Children Hospital, King Hassan II University, Casablanca, Morocco.
- Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
- Department of Pediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait City, Kuwait; Allergy and Clinical Immunology Unit, Pediatric Department, Al-Sabah Hospital, Kuwait City, Kuwait.
- Division of Immunology, Boston Children's Hospital and Harvard Medical School, Boston, Mass.
Abstract
Human inborn errors of immunity (IEI) are a group of 485 distinct genetic disorders affecting children and adults. Signs and symptoms of IEI are heterogeneous, and accurate diagnosis can be challenging and depends on the available human expertise and laboratory resources. The Middle East and North Africa (MENA) region has an increased prevalence of IEI because of the high rate of consanguinity with a predominance of autosomal recessive disorders. This area also exhibits more severe disease phenotypes compared with other regions, probably due to the delay in diagnosis. The MENA-IEI registry network has designed protocols and guidelines for the diagnosis and treatment of IEI, taking into consideration the variable regional expertise and resources. These guidelines are primarily meant to improve the care of patients within the region, but can also be followed in other regions with similar patient populations.
Keywords: Diagnosis; Inborn errors of immunity; MENA region; Primary immunodeficiency; Treatment.
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