Clinical “masks” of congenital pathology of the bronchopulmonary system

Congenital disorders of bronchopulmonary system are a group of rare diseases characterized by alterations in the morphological structure of the lungs, bronchi, and pulmonary blood vessels with manifestation in childhood. Congenital bronchopulmonary disorders are the third most common type of chronic lung and pulmonary conditions in children. The increase in the number of registered cases is associated with the use of modern imaging techniques, particularly computed tomography (CT). An urgent challenge in modern respiratory medicine is the development of methods for early diagnosis of congenital bronchopulmonary disorders, based on the integration and assessment of the diagnostic significance of key anamnestic, clinical and, potentially, radiological markers.

The aim was to present the most common congenital bronchopulmonary disorders using clinical cases of patients with primary ciliary dyskinesia and bilateral congenital lobar emphysema.

Methods. A retrospective analysis of the primary medical records of patients A and B (outpatient consultation reports and hospital discharge summaries) was performed, including an evaluation of their medical history, clinical presentation and diagnostic findings. Long-term personal follow-up data were also considered.

Conclusion. Raising awareness among primary care paediatricians about congenital bronchopulmonary disorders, including primary ciliary dyskinesia and congenital lobar emphysema, is essential for achieving early diagnosis.

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