Age at diagnosis and the symptom history of children with primary ciliary dyskinesia (PCD) are described by reviewing the case notes in the paediatric PCD clinic. Mean age at diagnosis was 4.4 y despite a history of neonatal respiratory distress in 37/55 cases, situs inversus in 38/55 cases and early onset troublesome rhinitis in 42/55. Conclusion: Diagnosis of PCD is often delayed despite the presence of typical symptoms early in life. The key clinical features of unexplained neonatal respiratory distress, early onset rhinitis, situs inversus and a productive cough are highlighted, which, especially when occurring in combination, makes early referral for specific testing for PCD mandatory.

A higher proportion of pediatric interstitial lung diseases can be diagnosed on thin-section CT than on chest radiographs. In our study, confident and correct diagnoses were made more frequently with CT than with chest radiographs.

The aim of the study was to investigate the contribution that open lung biopsy makes to the management of children with diffuse interstitial lung disease and to review the procedure-related morbidity in comparison with published literature on other biopsy techniques.The authors reviewed the case notes and histology of patients under 18 yrs who had had an open lung biopsy in 1991±1998 for investigation of diffuse interstitial lung disease.The majority of patients returned from theatre breathing spontaneously and without an intercostal drain. Three out of 27 suffered a complication related to the biopsy that required intervention. A clear histological diagnosis was reached in 25/27 patients resulting in a change of management in 15/27. The most common histological patterns were nonspecific interstitial pneumonitis which generally had a favourable prognosis and follicular bronchiolitis/lymphocytic interstitial pneumonitis where prognosis was largely dependent on that of an underlying systemic disorder.It is concluded that open lung biopsy makes a substantial contribution to the management of diffuse interstitial lung disease in children and considering both diagnostic yield and safety, remains the biopsy technique of choice. Eur Respir J 1999; 14: 817±821.

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Five children with severe asthma requiring at least 10 mg of prednisolone daily were treated with cyclosporin. Three were weaned from prednisolone, but one quickly relapsed. One girl had her steroid dose lowered but suVered unacceptable hirsutism and one child failed to respond. Cyclosporin may be useful in refractory childhood asthma. A prospective study is required to confirm this.