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Evaluation of the Frequency and Diagnostic Delay of Primary Immunodeficiency Disorders Among Suspected Patients Based on the 10 Warning Sign Criteria: A Cross-Sectional Study in Iran Publisher Pubmed



Bahrami A1 ; Sayyahfar S2 ; Soltani Z1 ; Khodadost M3 ; Moazzami B4, 5 ; Rezaei N4, 5
Authors
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Authors Affiliations
  1. 1. Department of Allergy and Immunology, Ali Asghar children's Hospital, Iran University of Medical Sciences, Tehran, Iran
  2. 2. Research Center of Pediatric Infectious Diseases, Institute of Immunology and Infectious Diseases, Iran University of Medical Sciences, Tehran, Iran
  3. 3. Department of Epidemiology, Faculty of Health, Iran University of Medical Sciences, Tehran, Iran
  4. 4. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
  5. 5. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Educationan and Research Netwrork (USERN), Tehran, Iran

Source: Allergologia et Immunopathologia Published:2020


Abstract

Introduction: The prevalence of undiagnosed primary immunodeficiency diseases is remarkably high and contributes to increasing the rate of morbidity and mortality among this group of patients. Objective: To examine the 10 warning sign scoring system in patients suspected of primary immune deficiency and also estimate the diagnostic delay in patients with proven disease. Methods: This descriptive cross-sectional study was carried out during the years 2015–2016 in Ali Asghar (AS) Clinic and Hospital. Two hundred patients with suspected primary immune deficiency disease were eligible for inclusion in the study. Multivariable logistic regression analysis was used to determine the relation between findings. Results: In this study, the majority of suspected cases of immunodeficiency were males (57%) with a mean age of 3.33 ± 2.89 years. Twenty-one (10.5%) patients were diagnosed with immunodeficiency disease. The mean diagnostic delay among primary immune deficient patients was 2.05 ± 1.7 years. There was a significant relationship between having parental consanguinity (OR = 2.68, 95% CI: 1.07–6.70), allergies (OR = 5.03, 95% CI: 1.13–22.31), vaccine adverse effects (OR = 9.31, 95% CI: 1.24–69.96) and primary immune deficiency diagnosis. No association was observed between age (OR = 0.98, 95% CI: 0.84–1.14), gender (OR = 0.99, 95% CI: 0.39–2.47), immune deficiency scoring (OR = 0.68, 95% CI: 0.31–1.45) and primary immune deficiency diagnosis. Conclusion: Ten warning sign scoring system is of less value to consider a patient suspected of having primary immune deficiency. There is a meaningful delay in diagnosis of primary immune deficiencies especially in antibody deficiency defects which seeks further upgrading of knowledge in physicians. © 2020 SEICAP
1. Global Systematic Review of Primary Immunodeficiency Registries, Expert Review of Clinical Immunology (2020)
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6. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders, Journal of Investigational Allergology and Clinical Immunology (2017)
8. Diagnostic Approach to the Patients With Suspected Primary Immunodeficiency, Endocrine# Metabolic and Immune Disorders - Drug Targets (2020)
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