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The need to redefine non-cystic fibrosis bronchiectasis in childhood

Lookup NU author(s): Dr Katherine Eastham, Dr Leslie Mitchell, Dr David Spencer

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Abstract

Background: Non-cystic fibrosis (CF) bronchiectasis has previously been reported to be rare and progressive in children living in western societies. Method: 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.


Publication metadata

Author(s): Eastham KM, Fall AJ, Mitchell L, Spencer DA

Publication type: Article

Publication status: Published

Journal: Thorax

Year: 2004

Volume: 59

Issue: 4

Pages: 324-327

ISSN (print): 0040-6376

ISSN (electronic): 1468-3296

Publisher: BMJ Publishing Group

URL: http://dx.doi.org/10.1136/thx.2003.011577

DOI: 10.1136/thx.2003.011577


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