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Bronchiectasis in patients without cystic fibrosis

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In prosperous countries most children with bronchiectasis have cystic fibrosis and the condition is otherwise uncommon. In economically disadvantaged countries it remains an important feature of chronic suppurative lung disease. In the UK the number of children admitted to hospital with bronchiectasis has declined in the last 50 years, probably because of improved overall health and nutrition and vaccination against whooping cough and measles. For diagnosis high resolution computed tomography (HRCT) has replaced bronchography. Despite the decline almost 10% of the children referred to the paediatric respiratory unit in Newcastle upon Tyne between November 1996 and May 2002 had non-cystic-fibrosis bronchiectasis confirmed by HRCT (

). Workers there have reviewed their findings in these 93 patients.

The HRCT diagnosis was based on one or more of the following four findings: cross sectional diameter of at least one bronchus greater than that of the accompanying pulmonary artery, mucoid impaction within a dilated bronchus, bronchi non-tapering in longitudinal cuts, and bronchi visible adjacent to non-mediastinal pleura. The 93 children were aged up to 16 years (median 1.1 years) at the onset of symptoms and two thirds were boys. The age at HRCT diagnosis was 1.6–18.8 years (median 7.2 years) and the time from symptom onset to diagnosis was 0.2–14.8 years (median 3.0 years). Most bronchiectasis was postpneumonic (28 cases, 30%); 19 cases (21%) were associated with immunodeficiency or immunosuppression, and 17 (18%) were idiopathic. Among the immunoincompetent group four children had chronic grandomatous disease and one agammaglobulinaemia; five had had heart transplants and one had been treated for leukaemia. Deficiencies of IgA, IgG2, IgG3, or specific antibodies were demonstrated in a number of children. Other conditions associated with bronchiectasis included bronchiolitis obliterans (8 cases), congenital lung abnormality (4), and chronic aspiration (3). Almost half of the children (45) had a referral diagnosis of asthma, erroneous in 39 cases. The organisms most commonly isolated from the 93 children were Haemophilus influenzae, Streptococcus pneumoniae, and Moraxella catharalis. Eighty-one children had had a chest X-ray in the 12 months before the HRCT diagnosis but only five reports accurately predicted the HRCT findings. Repeat HRCT scanning was done for 18 children 18 months to 5 years after the diagnostic scan; in six cases there had been complete resolution of the bronchiectasis, six had remained the same, five had progressed, and in one case there had been improvement but not complete resolution.

Some children with a diagnosis of “cough variant asthma” may have bronchiectasis. Chest X-ray is unreliable in the diagnosis. Treatment with antibiotics and physiotherapy may be curative for disease with a non-progressive or idiopathic aetiology. The prevalence of bronchiectasis in this population is estimated at around one in 5800 children under 17 years. To take account of the possibility of resolution the authors of this report propose the terms prebronchiectasis, HRCT bronchiectasis, and established bronchiectasis.