Assessment of clinical and radiological correlation in non-cystic fibrosis bronchiectasis

The aim of study was to review HRCT scores, demographic data, physical findings, clinical characteristics, laboratory findings, PFT results, growth in sputum culture, VAS scores, annual frequency of exacerbations, cough severity score, therapies employed in patients with non-cystic fibrosis bronchiectasis retrospectively and to compare with literature data; in addition, to compare HRCT scores with demographic data, clinical characteristics, laboratory findings, annual frequency of exacerbations, PFT, VAS and cough severity score. The study included 59 patients with age ranging from 6 months to 23 years who had been followed with a diagnosis of non-cystic fibrosis bronchiectasis between 2003 and 2015 at Pediatric Allergy & Immunology Department and Chest Department of Akdeniz University, Medicine School. Data regarding age, gender, age at diagnosis, age at onset of symptoms, etiology, family history, clinical characteristics, physical findings, history of recurrent wheezing, history of recurrent upper tract infection, second-hand smoke exposure, complete blood count, PFT, immune parameter, GER scintigraphy, sweat test, CFTR gene mutation, sputum culture and annual frequency of exacerbations were recorded from patient files. In addition, CSS, VAS and HRCT scores were also assessed. HRCT scores were assessed by a radiologist blinded to patient's clinical. Assessment was performed by using modified Bhalla scoring and another scoring system taking anatomic extent into consideration. Of the patients included, 31 (52.5%) were male and 28 (47.5%) were female. Mean age was 12.69±4.67 ranging from 5 to 23 years. Mean age at onset was 3.78±4.06 ranging from 0.5 to 16 years. Mean age at diagnosis was 9.61±4.84 ranging from 1 to 20 years. When etiology of bronchiectasis was considered, the most common cause was immune deficiency detected in 13 patients (22.0%); followed by PSD and previous infection in 9 (15.3%) each, asthma detected in 7 (11.9%), bronchiolitis obliterans in 2 (3.4%), rheumatoid disease in one (1.7%) and foreign body aspiration in one patient (1.7%). B total, D total, EXBRNC and SBRDIL values were found to be significantly higher in patients with hemoptysis when compared to those without. B total, D total, EXBRNC, SBRDIL and SBWTHICK values were found to be significantly higher in patients with sputum when compared to those without. In our study, EXBRNC, SBRDIL, SBWTHICK, PMPLA, EXDECAT were negatively correlated with FEV1, FVC and MEF25-75. In the second scoring employed in our study, total score of anatomic extent (D total) was significantly negatively correlated with FEV1, FVC and MEF25-75. The annual frequency f exacerbations was found to be positively correlated with EXBRNC, SBRDIL, SWITHICK, B total, D total and VAS. B total and D total were positively correlated with CSS while there was no correlation with VAS. Despite earlier age of onset, children with non-cystic fibrosis bronchiectasis are diagnosed late. In the patients, symptom severity and increased frequency negatively affect pulmonary functions, HRCT scores and quality of life. Early diagnosis, regular follow-up and management are of importance in order to improve quality of life in children with non-cystic fibrosis bronchiectasis