Saba Fekrvand1, Reza Yazdani1, Peter Olbrich2, Gholamreza Azizi3, Rohola Shirzadi4, Mohammadreza Modaresi4, Mahsa Sohani1, Samaneh Delavari1, Arash Kalantari5, Mansoureh Shariat6, Alireza Shafiei7, Na Lu8, Gholamreza Hassanpour9, Maziar Rahimi Hajiabadi1, Parisa Ashournia1, Anahita Razaghian1, Marzieh Asgharyan1, Zahra Shahraki-Ghadimi1, Roja Rouhani1, Fatemeh Hoda Fallah1, Nima Rezaei1,10, Hassan Abolhassani11,12, Asghar Aghamohammadi1. 1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran. 2. Sección de Infectología e Inmunopatología, Unidad de Pediatría, Hospital Virgen del Rocío/Instituto de Biomedicina de Sevilla (IBiS), Seville, Spain. 3. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran. 4. Department of Pediatric Pulmonary and Sleep Medicine, Children Medical Center, Tehran University of Medical Sciences, Tehran, Iran. 5. Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran. 6. Department of Allergy and Clinical Immunology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. 7. The Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran. 8. State Key Lab of Bioelectronics, School of Biological Science and Medical Engineering, Southeast University, Nanjing, China. 9. Center for Research of Endemic Parasites of Iran, Tehran University of Medical Sciences, Tehran, Iran. 10. Network for Immunology in Infection, Malignancy, and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran. 11. Research Center for Primary Immunodeficiencies, Iran University of Medical Sciences, Tehran, Iran. 12. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge, Stockholm, Sweden.
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.
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.
Authors: Jose A Rodriguez; Tami J Bang; Carlos S Restrepo; Daniel B Green; Lorna P Browne; Daniel Vargas Journal: Radiol Cardiothorac Imaging Date: 2021-03-25
Authors: Di Sun; Jennifer R Heimall; Matthew J Greenhawt; Nancy J Bunin; Marcus S Shaker; Neil Romberg Journal: JAMA Pediatr Date: 2022-02-01 Impact factor: 16.193