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Evaluation of Respiratory Complications in Patients With X-Linked and Autosomal Recessive Agammaglobulinemia Publisher Pubmed



Fekrvand S1 ; Yazdani R1 ; Olbrich P2 ; Azizi G3 ; Shirzadi R4 ; Modaresi M4 ; Sohani M1 ; Delavari S1 ; Kalantari A5 ; Shariat M6 ; Shafiei A7 ; Lu N8 ; Hassanpour G9 ; Rahimi Hajiabadi M1 Show All Authors
Authors
  1. Fekrvand S1
  2. Yazdani R1
  3. Olbrich P2
  4. Azizi G3
  5. Shirzadi R4
  6. Modaresi M4
  7. Sohani M1
  8. Delavari S1
  9. Kalantari A5
  10. Shariat M6
  11. Shafiei A7
  12. Lu N8
  13. Hassanpour G9
  14. Rahimi Hajiabadi M1
  15. Ashournia P1
  16. Razaghian A1
  17. Asgharyan M1
  18. Shahrakighadimi Z1
  19. Rouhani R1
  20. Hoda Fallah F1
  21. Rezaei N1, 10
  22. Abolhassani H11, 12
  23. Aghamohammadi A1
Show Affiliations
Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
  2. 2. Seccion de Infectologia e Inmunopatologia, Unidad de Pediatria, Hospital Virgen del Rocio/Instituto de Biomedicina de Sevilla (IBiS), Seville, Spain
  3. 3. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  4. 4. Department of Pediatric Pulmonary and Sleep Medicine, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran
  6. 6. Department of Allergy and Clinical Immunology, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. The Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
  8. 8. State Key Lab of Bioelectronics, School of Biological Science and Medical Engineering, Southeast University, Nanjing, China
  9. 9. Center for Research of Endemic Parasites of Iran, Tehran University of Medical Sciences, Tehran, Iran
  10. 10. Network for Immunology in Infection, Malignancy, and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  11. 11. Research Center for Primary Immunodeficiencies, Iran University of Medical Sciences, Tehran, Iran
  12. 12. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge, Stockholm, Sweden

Source: Pediatric Allergy and Immunology Published:2020


Abstract

Background: Congenital agammaglobulinemia is the first primary immunodeficiency disorder characterized by a defect in B lymphocyte development and subsequently decreased immunoglobulin levels. These patients are prone to suffer from recurrent infections mostly involving the respiratory tract. In this study, we aimed to describe in detail respiratory tract complications as the most prominent clinical feature among agammaglobulinemic patients. Methods: A total number of 115 patients were included. Demographic, clinical, and genetic data were collected from the patients’ medical records. Among the available patients, pulmonary function tests (PFTs) and/or high-resolution computed tomography (HRCT) were performed. Results: Respiratory tract complications (85.2%) especially pneumonia (62.6%) were the most prominent clinical features in our cohort. Among patients with abnormal PFT results (N = 19), a mixed respiratory pattern was observed in 36.8%. HRCT was carried out in 29 patients; Bhalla scoring-based evaluation of these patients indicated excellent (44.8%), followed by good (34.5%) and mild (20.7%) results. Bronchiectasis was found in 13 patients undergoing HRCT (44.8%). We found significant inverse correlations between the Bhalla score and incidence rate of pneumonia, as well as the presence of bronchiectasis. Patients with abnormal PFT results had statistically significant higher bronchiectasis frequency and lower Bhalla scores compared to those with normal results. Forty-one patients were deceased, and here, respiratory failure was the most common cause of death (45.5%). Conclusion: High prevalence of respiratory tract infections among agammaglobulinemic patients and subsequent progression to permanent lung damage highlights the importance of implementing respiratory evaluation as part of routine follow-up program of agammaglobulinemic patients. Physicians should be aware of this and regularly monitor the respiratory function of these patients to allow for timely diagnosis and treatment initiation aiming to improve patients’ prognosis and quality of life. © 2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.
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