Pulmonary Manifestations in Children with Predominantly Antibody Deficiencies: A Four-Year Cohort from a Referral Center in Bogotá Autores

Introduction Predominantly antibody deficiencies (PADs) are the most frequent group of inborn errors of immunity and commonly present with recurrent respiratory infections. Early recognition of pulmonary complications is essential to reduce long-term morbidity. Methods This descriptive retrospective cohort study aimed to characterize features of pediatric patients diagnosed with PADs, with a focus on pulmonary complications. Results We present a series of 72 pediatric patients diagnosed with PADs at a referral center in Bogota (June 2021 to June 2025). The most frequent diagnoses were specific antibody deficiency (SAD) (32 patients, 44.4%), selective IgA deficiency (12, 16.7%), common variable immunodeficiency (12, 16.7%), and X-linked agammaglobulinemia (XLA) (7, 9.7%). The median age at first infection was 7 months (interquartile range: 31.5), and the mean age at diagnosis was 70 months (SD: 44.7). Comorbid allergic diseases were highly prevalent (73.6%), particularly asthma (62.5%), allergic rhinitis (62.5%), and atopic dermatitis (34.7%). Pneumonia was observed in 60.9% of patients, otitis media in 37.7%, and sinusitis in 14.5%. Orotracheal intubation was required in 29.2% of patients, some on multiple occasions. Chest CT scans were performed in 32 patients; the most common findings were atelectasis (25%) and bronchiectasis (18.8%). Bronchiectasis was more frequent in those with XLA (3/7; 42.9%) and SAD (4/32; 12.5%). Lung function was abnormal in 80% of oscillometries (n = 20) and 42.1% of spirometries (n = 19). Bronchoalveolar lavage in 11 patients yielded three positive bacterial cultures. Immunoglobulin replacement was given to 65.3%; 25% received prophylactic azithromycin. One patient with an undiagnosed syndromic phenotype died. Conclusion This cohort highlights the high respiratory disease burden in children with PADs, including obstructive functional disorders, allergic comorbidities such as asthma and allergic rhinitis, and increased bronchiectasis in XLA.

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Journal
Journal of Human Immunity
Published
2025-12-22
DOI
https://doi.org/10.70962/lasid2025abstract.23
Primary Topic
Immunodeficiency and Autoimmune Disorders
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article
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article

Pulmonary Manifestations in Children with Predominantly Antibody Deficiencies: A Four-Year Cohort from a Referral Center in Bogotá Autores

José Ignacio Palencia-Palencia, Óscar Ramírez, Natalia Female Vélez-Tirado, Liliana Artunduaga-Mendez et al.
Journal of Human Immunity
Immunodeficiency and Autoimmune Disorders
article

Pulmonary Manifestations in Children with Predominantly Antibody Deficiencies: A Four-Year Cohort from a Referral Center in Bogotá Autores

José Ignacio Palencia-Palencia, Óscar Ramírez, Natalia Female Vélez-Tirado, Liliana Artunduaga-Mendez, Camilo Orbes-Guerrero, Santiago Moreno-Terreros, Tatiana Rodriguez-Noguera, Lina Castano-Jaramillo, Milena Villamil-Osorio
article en

Abstract

Introduction Predominantly antibody deficiencies (PADs) are the most frequent group of inborn errors of immunity and commonly present with recurrent respiratory infections. Early recognition of pulmonary complications is essential to reduce long-term morbidity. Methods This descriptive retrospective cohort study aimed to characterize features of pediatric patients diagnosed with PADs, with a focus on pulmonary complications. Results We present a series of 72 pediatric patients diagnosed with PADs at a referral center in Bogota (June 2021 to June 2025). The most frequent diagnoses were specific antibody deficiency (SAD) (32 patients, 44.4%), selective IgA deficiency (12, 16.7%), common variable immunodeficiency (12, 16.7%), and X-linked agammaglobulinemia (XLA) (7, 9.7%). The median age at first infection was 7 months (interquartile range: 31.5), and the mean age at diagnosis was 70 months (SD: 44.7). Comorbid allergic diseases were highly prevalent (73.6%), particularly asthma (62.5%), allergic rhinitis (62.5%), and atopic dermatitis (34.7%). Pneumonia was observed in 60.9% of patients, otitis media in 37.7%, and sinusitis in 14.5%. Orotracheal intubation was required in 29.2% of patients, some on multiple occasions. Chest CT scans were performed in 32 patients; the most common findings were atelectasis (25%) and bronchiectasis (18.8%). Bronchiectasis was more frequent in those with XLA (3/7; 42.9%) and SAD (4/32; 12.5%). Lung function was abnormal in 80% of oscillometries (n = 20) and 42.1% of spirometries (n = 19). Bronchoalveolar lavage in 11 patients yielded three positive bacterial cultures. Immunoglobulin replacement was given to 65.3%; 25% received prophylactic azithromycin. One patient with an undiagnosed syndromic phenotype died. Conclusion This cohort highlights the high respiratory disease burden in children with PADs, including obstructive functional disorders, allergic comorbidities such as asthma and allergic rhinitis, and increased bronchiectasis in XLA.

Journal of Human ImmunityVol. 1(LASID2025)
Universidad El Bosque (CO), Fundación Universitaria de Ciencias de la Salud (CO), Hospital Universitario Nacional de Colombia (CO)
Good health and well-being
Openalex Percentile: Top 37%
Immunodeficiency and Autoimmune Disorders
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