Infecțiile bronhopulmonare în imunodeficiențele primare la copii

Abstract

Summary. Primary immunodeficiencies (PIDs) represent a heterogeneous group of inherited disorders characterized by impaired immune responses, predisposing affected children to recurrent and severe infections. Among these, bronchopulmonary infections are particularly frequent and contribute significantly to morbidity and long-term pulmonary complications. This review aims to summarize the clinical spectrum, pathophysiology, and management of bronchopulmonary infections in pediatric PIDs patients. Recurrent bacterial pneumonias, bronchiectasis, chronic sinusitis, and atypical infections are commonly observed, with pathogens including Streptococcus pneumoniae, Haemophilus influenzae, Pseudomonas aeruginosa, and opportunistic organisms in severe immunodeficiencies. Early recognition of infection patterns, combined with immunological evaluation, is essential for timely diagnosis of underlying PIDs. Diagnostic approaches include chest imaging, microbiological cultures, and immunophenotyping, while management strategies encompass targeted antimicrobial therapy, prophylactic antibiotics, immunoglobulin replacement, and, in selected cases, hematopoietic stem cell transplantation. Preventive measures, including vaccination and airway clearance techniques, are crucial to reduce infection burden and preserve lung function. Despite advances in diagnosis and therapy, chronic pulmonary complications remain a leading cause of morbidity and mortality, underscoring the need for multidisciplinary care and long-term follow-up. Enhanced awareness of infection patterns in PIDs facilitates early intervention, improves clinical outcomes, and highlights the importance of individualized management in this vulnerable population.