Non-cystic fibrosis bronchiectasis in children

Abstract

Introduction: Non-cystic fibrosis (CF) bronchiectasis is a chronic inflammatory lung disease that causes persistent productive cough, recurrent pneumonia and obstructive pulmonary disease.

Aims: To evaluate etiology and clinical features of children with non-CF bronchiectasis.

Methods: All children with non-CF bronchiectasis were included in the study. Patients’ gender, parental consanguinity, age at diagnosis, complaints, duration of complaints, etiology of bronchiectasis, radiologic findings, pulmonary function test (PFT) results and operations were evaluated.

Results: Of the 77 patients with non-CF bronchiectasis, 47.4% were female and mean age at diagnosis was 10.3±4.0 years. The median duration of complaints was 78 (IQR:12-120) months. Parental consanguinity was present in 59.4% of patients. Most frequent symptoms were chronic productive cough, sputum expectoration, shortness of breath, wheezing, hemoptysis and weight loss. Common causes were post-infectious complication in 50.6%, PCD  in 33.7%, immunodeficiency in 9.0%, history of tuberculosis in 3.8%, history of foreign body aspiration in 1.3% and other anomalies in 1.6% of patients. Pulmonary lobe was involved in 60.8% left lower lobe, 50.9% right lower lobe, 40% right middle lobe, 25.6% right upper lobe, and 14.2% left upper lobe. Fifty-one patients were able to perform PFT. Mean of FEV1, FVC, FEV1/FVC, FEF25-75 were 81.8±21.4%, 82.3±19.7%, 87.0±22.2, 83.5±27.8% respectively. Lobectomy was performed in 5 patients with localized bronchiectasis causing recurrent pneumonia.

Conclusions: Although infections are most common causes of non-CF bronchiectasis, other underlying diseases should be investigated.