Demographic characteristisc:
Recent cohort included 104 patients diagnosed as bronchiectasis after 2002 (45% male) with a median age of 8 years (range, 0.1-16.5) at presentation.
In the recent cohort, median age of diagnosis was 7 years. The most common presenting symptom was cough (95.2%) followed by sputum (77.9%), wheezing (42.3%) and dyspnea (51%). Table-1 shows the general characteristics of two cohorts. Rate of clubbing was decreased, baseline FEV1 and FVC values were higher in the recent cohort when compared to the historical one (Table-1).
Table-1: Demographic data for non-CF patients
Historical Cohort Recent Cohort
1987- 2001 (n:111) 2002-2019 (n:104) p-value
Male gender n(%) 56 (50.5) 47 (45) 0.52
Age of onset of symptoms (yr), median (min-max) 1.5 (0-11.9) 1 (0-16) 0.11
Duration of symptoms before diagnosis (yr),
median (min-max) 4 (0.1-14.9) 4 (0-15.5) 0.89
Age at presentation (yr), median (min-max) 7 (1-17.5) 8 (0.1-16.5) 0.36
Hemoptysis n(%) 11 (10) 7 (6.7) 0.40
Clubbing n(%) 45 (40.9) 22 (21.2) 0.02
Chest deformity,n(%) 16 (14.5) 12 (11.5) 0.53
Annual lower respiratory tract infection rate (mean±SD) 6.6±4.0 6.1±2.9 0.91
Spirometry n:93 ( mean±SD)
FEV1 (% predicted) 63.3±22.1 76.6±17.1 < 0.001
FVC (% predicted) 67.3±23.1 76.7±15.1 < 0.001
In terms of pulmonary function, there was an increase in the mean FEV1 and FVC (p<0.001) in the recent cohort. Similar to the historical cohort, annual lower respiratory tract infection rate decreased from 6.1±2.9 at presentation to 3.2±2.1 during follow-up (p<0.0001). There was no change between the baseline and last FEV1 % in follow-up in the recent cohort (76.6±17.1 vs. 76.96±18.1; p=0.91) which increased in the historical one (63.3 ± 22.1 vs. 75.2± 25.2; p<0.001).
Etiology
Underlying etiology was identified in 80.8 % (n:84) of the patients in the resent cohort (Table-2). Most common cause of non-CF bronchiectasis was PCD (32.7%), followed by post-infectious (26%) and immunodeficiencies (17.3%). All of the PCD patients (n:34) had typical findings of PCD and PICADAR score>5. In 26 of patients nasal nasal nitric oxide measurement, in 4 transmission electron microscopy, in 19 immunofluorescence staining (7 with outer dynein arm defect, 4 with inner dynein arm defect, 4 with nexin-dynein regulatory complex defect and 2 with radial spoke defects) and in 4 genetic analysis (2 patients had CCDC40 homozygous, 1 CCNO homozygous and RSPH4A homozygous mutations) were performed [26]. There was a significant increase in the frequency of PCD (6.3% vs. 32.7%, p=0.001), and decrease in idiopathic cases (37.8% vs. 19.2%, p=0.03) compared with the historical cohort. In the recent cohort, there was no patient with a history of foreign body aspiration. One patient with esophageal atresia and tracheoesophageal fistula and two patients operated for complex cardiac disease were classified in other group.
Table-2: Underlying etiologies for non-CF patients
Historical Cohort Recent Cohort
1987-2001 2002-2019 p value
Idiopathic 42(37.8) 20(19.2) 0.03
Postinfectious 33(29.7) 27(26) 0.43
Immunodeficiencies 17(15.3) 18(17.3) 0.69
PCD 7(6.3) 34(32.7) < 0.001
Asthma 5(4.5) 3(2.9) 0.72
Foreign body aspirations 4(3.6) 0 NA
Others 3(2.7) 3(2.9) 0.64
Esophageal atresia-tracheoesophageal fistula 3(2.7) 1(0.9)
Cardiac diseases 0 2(2)
Values in parentheses are percentages
NA: not applicable
Localization of Bronchiectasis
Localization of bronchiectasis was similar to the historical cohort, and left lower lobe was the most affected lobe in both groups. In 38.5% of the patients there was one lobe involvement, mostly the left lower lobe (21.2%) similar to the historical cohort (p= 0.75). Although statistically insignificant, multilobar involvement tended to decrease (31.9% vs. 21.4%, p= 0.26) and bilobar involvement trended to increase (28.1% vs 41.3% (p=0.09) in the recent cohort compared with the historical one.
Sputum culture
Rate of positive sputum culture increased in the recent cohort (46.9% vs. 77.9%, p=0.001). Most frequently isolated organisms were H.influenza (71.8%), S.pneumonia (47.1%), M.catarrhalis (14.4%), P.aeruginosa (11.5%) and S.aureus (11.5%), with the first three being significantly more common compared to the historical cohort (p=0.001, p=0.001 and p=0.03, respectively) (Table-3).