Impact of underlying cause of bronchiectasis on clinical outcome: A comparative study on CF and Non-CF bronchiectasis in Egyptian children

Background: Bronchiectasis represents an important cause of chronic lung disease in children in developing countries and continues to be one of the leading causes of morbidity and mortality with worsening quality of life in these children. Aim: To compare the clinical course and outcome in Cystic Fibrosis (CF) and non CF bronchiectasis in children. Patients and method: This cross sectional observational study included 50 children with bronchiectasis; all were followed up at pulmonology Unit, Cairo University Children Hospital, Egypt. Diagnosis of bronchiectasis was confirmed by high-resolution computed tomography (HRCT). Forced expiratory volume in the first second (FEV1) was recorded for all patients, severity classification of the FEV1 was according to the interpretive strategy set out by the ATS/ERS task force. All enrolled cases were followed up for 2 years to record clinical outcome (hospitalization, exacerbation, and mortality). Results: There was a significant association between CF bronchiectasis and very severe FEV1 impairment (p value 0.002). A significant proportion of children had hospitalization with severe exacerbation in 72%, and 16% of children died during the period of follow up. There was a strong relationship between the duration of bronchiectasis and hospitalization with severe exacerbation (p value 0.01). Also, there was a strong positive correlation between the severity of impairment of FEV1 and both hospitalization with severe exacerbation and mortality (p value < 0.001). Conclusion: Although, the majority of bronchiectasis cases in this study were due to non-CF bronchiectasis causes. CF bronchiectasis was associated with significant very severe impairment of FEV1, also with the greatest proportion of deaths compared to non-CF bronchiectasis. Keywords: Bronchiectasis, Children, Cystic fibrosis, Non cystic fibrosis, Outcom