Evaluation of respiratory complications in patients with X-linked and autosomal recessive agammaglobulinemia

Fekrvand, S. and Yazdani, R. and Olbrich, P. and Azizi, G. and Shirzadi, R. and Modaresi, M. and Sohani, M. and Delavari, S. and Kalantari, A. and Shariat, M. and Shafiei, A. and Lu, N. and Hassanpour, G. and Rahimi Hajiabadi, M. and Ashournia, P. and Razaghian, A. and Asgharyan, M. and Shahraki-Ghadimi, Z. and Rouhani, R. and Hoda Fallah, F. and Rezaei, N. and Abolhassani, H. and Aghamohammadi, A. (2020) Evaluation of respiratory complications in patients with X-linked and autosomal recessive agammaglobulinemia. Pediatric Allergy and Immunology, 31 (4). pp. 405-417.

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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.

Item Type: Article
Additional Information: cited By 0
Uncontrolled Keywords: genomic DNA, agammaglobulinemia; Article; atopy; autosomal recessive disorder; bronchiectasis; bronchitis; cause of death; child; chronic cough; clinical feature; computer assisted tomography; DNA extraction; genetic screening; human; lung function test; lung infection; lung injury; major clinical study; molecular diagnosis; otitis media; pneumonia; priority journal; recurrent infection; respiratory failure; respiratory tract disease; respiratory tract infection; Sanger sequencing; sinusitis; wheezing; whole exome sequencing; X linked agammaglobulinemia
Subjects: WF Respiratory System
QW Microbiology. Immunology
Depositing User: eprints admin
Date Deposited: 13 Sep 2020 09:44
Last Modified: 13 Sep 2020 09:44
URI: http://eprints.iums.ac.ir/id/eprint/23714

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