title: Do New Zealand children with non-cystic fibrosis bronchiectasis show disease progression?
abstract: BACKGROUND
  There is minimal literature available on the long-term outcome of pediatric non-cystic fibrosis (CF) bronchiectasis.
  
  
  OBJECTIVE
  To document 5-year outcomes of children with chest computerized tomography (CT) scan diagnosed bronchiectasis from a tertiary New Zealand (NZ) respiratory clinic.
  
  
  METHODS
  Review of a clinical database identified 91 children. Demographics, clinical data, lung function, chest X-ray (CXR), sputum, presumed etiology, admission data, and the NZ deprivation index (NZDep) were collected. Univariate and multivariate regression were used to correlate clinical findings with lung function data and CXR scores using the Brasfield Scoring System.
  
  
  RESULTS
  Of the 91 children, 53 (59%) were Pacific Island, 22 (24%) Maori, 14 (15%) European, and 2 (2%) Other. The median follow-up period was 6.7 years (range 5.0-15.3 years) and median age at diagnosis was 7.3 years (range 11 months-16 years). Lung function data (n = 64) showed a mean decline of -1.6% predicted/year. In 30 children lung function declined (mean FEV(1) -4.4% predicted/year, range 1-17%), remained stable in 13 and improved in 21 children (mean FEV(1) of +3% predicted/year, range 1-15%). Reduced lung function was associated with male gender, chronic Haemophilus influenzae infection, longevity of disease, and Maori and Pacific Island ethnicity. There was a significant correlation with FEV(1) and CXR score at beginning (n = 47, r = 0.45, P = 0.001) and end (n = 26, r = 0.59, P = 0.002) of the follow-up period. The only variable consistently related to CXR score was chronic Haemophilus influenzae infection occurring in 27 (30%) (r(2)  = 0.52, P = <0.0001). Only four children were chronically infected with Pseudomonas species. Six children died.
  
  
  CONCLUSIONS
  In our experience despite management in a tertiary multidisciplinary bronchiectasis clinic, progression of lung disease continues in a group of children and young adults.
authors: Department of Paediatrics, University of Auckland & Starship Children's Hospital, Auckland, New Zealand.
authors: Karen A
authors: KA
authors: Munro
authors: Peter W
authors: PW
authors: Reed
authors: Helen
authors: H
authors: Joyce
authors: David
authors: D
authors: Perry
authors: Jacob
authors: J
authors: Twiss
authors: Catherine A
authors: CA
authors: Byrnes
authors: Elizabeth A
authors: EA
authors: Edwards
mesh_terms: D000293:Adolescent; D001835:Body Weight; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology; D001992:Bronchoalveolar Lavage Fluid / Q000382:microbiology; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D018450:Disease Progression; D005260:Female; D006192:Haemophilus Infections / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D006193:Haemophilus influenzae / Q000302:isolation & purification; D006801:Humans; D007223:Infant; D008168:Lung / Q000382:microbiology / Q000503:physiopathology; D008297:Male; D009520:New Zealand / Q000453:epidemiology; D011552:Pseudomonas Infections / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D013183:Sputum / Q000382:microbiology; D014057:Tomography, X-Ray Computed