PATTERN-URI CLINICE PULMONARE ÎN IMUNODEFICIENŢELE PRIMARE: DE LA INFECŢIE LA REMODELARE BRONHOPULMONARĂ

Abstract

Introducere. Imunodeficienţele primare (IDP) au fost definite ca afecţiuni genetice caracterizate prin infecţii respiratorii recurente şi evoluţie spre complicaţii pulmonare. A fost evidenţiată în timp importanţa depistării precoce a afectării respiratorii pentru prevenirea deteriorării structurale pulmonare. Scop. A fost realizată analiza manifestărilor clinice respiratorii şi a complicaţiilor bron-hopulmonare la pacienţii pediatrici cu IDP, evaluaţi în Clinica de Pneumologie pediatrică. Material şi metode. A fost efectuat un studiu observaţional retrospectiv pe un lot de 26 de pacienţi pediatrici diagnosticaţi cu IDP. Au fost colectate şi analizate date clinice, paraclinice şi evolutive. S-au documentat episoade de bronşită, pneumonie, pleurezie, precum şi semne de remodelare pulmonară. A fost aplicată analiza statistică descriptivă. Rezultate. Vârsta medie a pa-cienţilor-8,6ani, iar vârsta medie la diagnostic-5,2ani. Cele mai frecvente forme de IDP: sindromul DiGeorge (23,1%), SCID (11,5%), agamaglobulinemia Bruton (11,5%). 84,6% dintre pacienţi au prezentat cel puţin o infecţie respiratorie joasă. Bronşitele au fost cele mai frecvente(84,6%), urmate de pneumonii(73,1%), pleurezii(26,9%). Remod-elarea bronhopulmonară a fost documentată la 42,3% pacienţi: fibroatelectazii (26,9%), fibroză pulmonară(19,2%), bronşectazii (11,5%), pneumatocele (7,7%). Un pattern de progresie de la infecţii recurente la leziuni structurale a fost observat, în special, la pacienţii cu forme combinate de IDP. Concluzii. Infecţiile respiratorii recurente sunt un semnal precoce al IDP. Evaluarea funcţională, imagistică respiratorie periodică este esenţială în monitorizarea pacienţilor cu IDP. Identificarea precoce a patternurilor pulmonare permite intervenţia terapeutică timpurie şi prevenirea deteriorării pulmonare.

Introduction. Primary immunodeficiencies (PIDs) were defined as genetic disorders characterized by recurrent respiratory infections and progressive pulmonary complications. Over time, the importance of early detection of respiratory involvement for preventing structural lung damage has been emphasized. Objective. The analysis of respiratory clinical manifestations and bronchopulmonary complications was performed in pediatric patients with PID evaluated at the Pediatric Pulmonology Clinic. Material and methods. A retrospective observational study was conducted on a cohort of 26 pediatric patients diagnosed with PID. Clinical, paraclinical, and evolutionary data were collected and analyzed. Episodes of bronchitis, pneumonia, pleurisy, and signs of pulmonary remodeling were documented. Descriptive statistical analysis was applied. Results. The mean age of patients was 8.6 years, and the mean age at diagnosis was 5.2 years. The most frequent PID types were DiGeorge syndrome (23.1%), SCID (11.5%), and Bruton agammaglobulinemia (11.5%). Overall, 84.6% experienced at least one lower respiratory tract infection. Bronchitis was the most common (84.6%), followed by pneumonia (73.1%), and pleurisy (26.9%). Bronchopulmonary remodeling was documented in 42.3% of patients, including fibroatelecta-sis (26.9%), pulmonary fibrosis (19.2%), bronchiectasis (11.5%), and pneumatocele (7.7%). A progression pattern to structural lung damage was observed particularly in combined PID. Conclusion. Recurrent respiratory infections represent an early indicator of PID. Periodic functional and imaging assessment of the respiratory system proved essential in monitoring patients with PID. Early identification of pulmonary patterns enables timely intervention and prevention of lung damage.