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Registry of Clinical Data and Laboratory Findings in 80 Patients With Primary Immunodeficiencies in Mofid Children Hospital



Mirzaee AZ1 ; Darougar S2 ; Chavoshzadeh Z1, 7 ; Mesdaghi M3 ; Mansouri M1 ; Babaie D1 ; Karimi A4 ; Armin S4 ; Fahimzad A4 ; Tabatabaei SR4 ; Ghanaie RM4 ; Shiari R4 ; Tabatabaii SA5 ; Babaee GK5 Show All Authors
Authors
  1. Mirzaee AZ1
  2. Darougar S2
  3. Chavoshzadeh Z1, 7
  4. Mesdaghi M3
  5. Mansouri M1
  6. Babaie D1
  7. Karimi A4
  8. Armin S4
  9. Fahimzad A4
  10. Tabatabaei SR4
  11. Ghanaie RM4
  12. Shiari R4
  13. Tabatabaii SA5
  14. Babaee GK5
  15. Sadr S5
  16. Hashemitari SK6
Show Affiliations
Authors Affiliations
  1. 1. Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  2. 2. Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
  3. 3. Department of Immunology, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  4. 4. Pediatric Infections Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  5. 5. Department of Pediatrics Pulmonary Diseases, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  6. 6. Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Shariati Avenue, Next to Mirdamad, Tehran, Zip Code: 15468-155514, Iran

Source: Journal of Comprehensive Pediatrics Published:2018

Abstract

Background: Primary immunodeficiency disorders are a diverse group of rare genetic diseases that are often under-recognized. Therefore, significant morbidities and mortalities ensue due to diagnosis delay. This is an evaluation of clinical presentations and laboratory data of children referred to Mofid University Hospital during a 10-year period. The aim of this study is to provide epidemiologic data for early diagnosis and proper management of these patients. Methods: A total of 80 children referred to Mofid's Children Hospital suspected to primary immunodeficiencies (PIDs) were evaluated clinically. The demographic, clinical, and laboratory data were collected in detail and were analyzed as a cross-sectional study. Missing data were completed in follow-up visits. Results: The prevalence of antibody deficiencies, phagocytic defects, and combined immunodeficiencies in 80 children with primary immunodeficiency syndromes were respectively 36.25%, 32.5%, and 31.25%. Common variable immunodeficiency (13.75%) and severe combined immunodeficiency (12.5%) had the highest prevalence among these disorders and the lowest prevalence were reported for cyclic neutropenia (5%), ataxia-telangiectasia (5%), and transient hypogammaglobulinemia of infancy (3.75%). The most common mode of inheritance detected was an autosomal recessive pattern. Pneumonia, otitis media, and diarrhea were the most common complications in the course of these disorders, which were seen respectively in 30%, 18.8%, and 15% of the patients. Conclusions: Issues regarding clinical presentation and management of primary immunodeficiencies continue to puzzle the clinical practitioner. Epidemiological data are needed to increase the awareness of the physicians regarding primary immunodeficiency disorders and to support the benefits of early diagnosis and treatment. Diagnosis delay is associated with increased morbidities and even mortalities. © 2018, Journal of Comprehensive Pediatrics.
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