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.
