The need to redefine non-cystic fibrosis bronchiectasis in childhood.
BACKGROUND
Non-cystic fibrosis (CF) bronchiectasis has previously been reported to be rare and progressive in children living in western societies.


METHODS
A clinical and radiological review was undertaken of 93 children with non-CF bronchiectasis defined by high resolution computed tomographic (HRCT) scanning presenting to a tertiary paediatric respiratory centre since 1996.


RESULTS
Cases constituted 9.6% of all new referrals. Male to female ratio was 2:1. Median age at symptom onset was 1.1 years (range 0-16) and of HRCT diagnosis was 7.2 years (1.6-18.8). The most common referral diagnosis of asthma was refuted in 39 of 45 cases. Associations were previous pneumonic illness (30%), immunocompromise (21%), obliterative bronchiolitis (9%), congenital lung abnormality (5%), chronic aspiration (3%), eosinophilic oesophagitis (2%), familial syndrome (2%), primary ciliary dyskinesia (1%), and right middle lobe syndrome (1%). 8% had two associated diagnoses and 18% were idiopathic. There was agreement between the chest radiograph and HRCT scan for diagnosis and lobe affected in only five cases (5%). A repeat HRCT scan in 18 cases at a minimum interval of 18 months showed total resolution of the changes in six, improvement in one, progression in five, and was unchanged in six.


CONCLUSIONS
Radiologically defined non-CF bronchiectasis in children is not uncommon. Diagnostic delay is a problem. The most common association is a previous pneumonia. Chest radiography is of little diagnostic value, but resolution is possible on HRCT scanning. Bronchiectasis is currently defined as a condition which is both permanent and progressive. This term is not necessarily appropriate for all paediatric patients for whom we suggest an alternative nomenclature.
Eastham
K M
KM
Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK.
Fall
A J
AJ
Mitchell
L
L
Spencer
D A
DA
D000293:Adolescent; D001249:Asthma / Q000150:complications; D001987:Bronchiectasis / Q000150:complications / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003550:Cystic Fibrosis; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications; D007223:Infant; D008171:Lung Diseases / Q000150:complications; D008297:Male; D012017:Referral and Consultation; D012141:Respiratory Tract Infections / Q000150:complications; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods