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Expert Review of Clinical Immunology Volume 16, 2020 - Issue 7
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Review

Global systematic review of primary immunodeficiency registries

Hassan Abolhassania Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;b Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, SwedenCorrespondence[email protected]
ORCID Iconhttps://orcid.org/0000-0002-4838-0407View further author information
ORCID Icon,
Gholamreza Azizic Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, IranView further author information
,
Laleh Sharifia Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;ab Uro-Oncology Research Center, Tehran University of Medical Sciences, Tehran, IranView further author information
,
Reza Yazdania Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, IranORCID Iconhttps://orcid.org/0000-0002-5578-6595View further author information
ORCID Icon,
Monireh Mohsenzadegana Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, IranView further author information
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Samaneh Delavaria Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;ac Department of Medical Laboratory Sciences, Faculty of Allied Medical Sciences Iran University of Medical Sciences, Tehran, IranView further author information
,
Mahsa Sohania Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, IranView further author information
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Paniz Shirmasta Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, IranView further author information
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Zahra Chavoshzadehd Pediatric Infections Research Center, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, IranView further author information
,
Seyed Alireza Mahdavianie Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases(NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, IranView further author information
,
Arash Kalantarif Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, IranView further author information
,
Marzieh Tavakolc Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, IranView further author information
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Farahzad Jabbari-Azadg Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, IranView further author information
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Hamid Ahanchiang Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, IranView further author information
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Tooba Momenh 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, IranView further author information
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Roya Sherkati Acquired Immunodeficiency Research Center, Lsfahan University of Medical Sciences, Isfahan, LranView further author information
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Mahnaz Sadeghi-Shabestarij Immunology research center of Tabriz, TB and lung research center of Tabriz, Children Hospital, Tabriz University of Medical Science, Tabriz, IranView further author information
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Soheila Aleyasink Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, IranView further author information
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Hossein Esmaeilzadehk Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, IranView further author information
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Waleed Al-Herzl Department of Pediatrics, Kuwait University, Kuwait City, Kuwait;m Allergy and Clinical Immunology Unit, Department of Pediatrics, Al-Sabah Hospital, Kuwait City, KuwaitView further author information
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Ahmed Aziz Bousfihan Laboratoire d’Immunologie Clinique, d’Inflammation Et d’Allergie LICIA, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco;o Clinical Immunology Unit, Casablanca Children’s Hospital, Ibn Rochd Medical School, Hassan II University, Casablanca, Morocco;p The African Society for Immunodeficiencies (ASID) RegistryView further author information
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Antonio Condino-Netoq Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, São Paulo, Brazil;r The Latin American Society for Immunodeficiencies (LASID) RegistryView further author information
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Mikko Seppänens Adult Immunodeficiency Unit, Infectious Diseases, Inflammation Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland;t Rare Disease Center and Pediatric Research Center, Children’s Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland;u European Society for Immunodeficiencies (ESID) RegistryView further author information
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Kathleen E. Sullivanv Division of Allergy Immunology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA;w The United States Immunodeficiency Network (USIDNET) RegistryORCID Iconhttps://orcid.org/0000-0003-4018-1646View further author information
ORCID Icon,
Lennart Hammarströmb Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, SwedenView further author information
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Vicki Modellx Jeffrey Modell Foundation (JMF), New York City, NY, USAView further author information
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Fred Modellx Jeffrey Modell Foundation (JMF), New York City, NY, USAView further author information
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Jessica Quinnx Jeffrey Modell Foundation (JMF), New York City, NY, USAView further author information
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Jordan S. Orangex Jeffrey Modell Foundation (JMF), New York City, NY, USA;y Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NY, USAView further author information
&
Asghar Aghamohammadia Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;z Iranian Primary Immunodeficiencies Network (IPIN), Tehran University of Medical Science, Tehran, Iran;aa Asia Pacific Society for Immunodeficiencies (APSID) RegistryCorrespondence[email protected]
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Pages 717-732 | Received 02 Jun 2020, Accepted 08 Jul 2020, Published online: 17 Sep 2020

Figures & data

Figure 1. Distribution of primary immunodeficiency prevalence in the world based on the number of reported patients per 100000 individuals (Gray color represents countries without registry or without published report).

Figure 1. Distribution of primary immunodeficiency prevalence in the world based on the number of reported patients per 100000 individuals (Gray color represents countries without registry or without published report).

Figure 2. Distribution of parental consanguinity among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 2. Distribution of parental consanguinity among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 3. Proportion of predominant antibody deficiencies among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 3. Proportion of predominant antibody deficiencies among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 4. Proportion of male to female ratio among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 4. Proportion of male to female ratio among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 5. Proportion of molecular diagnosis among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Figure 5. Proportion of molecular diagnosis among different registries of patients with primary immunodeficiency in the world. (Gray color represents countries without registry or without appropriate report).

Table 1. The frequency of diagnosed primary immunodeficiency (PID) cases in the world and percentage of Predominantly antibody deficiencies (PAD), immunodeficiencies affecting cellular and humoral immunity (CID), congenital defects of phagocyte number or function, complement deficiencies and other PIDs (including syndromic CID, diseases of immune dysregulation, defects in intrinsic and innate immunity, autoinflammatory disorders and PID phenocopies).

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