Non-CF bronchiectasis: clinical and HRCT evaluation.

Abstract

Childhood bronchiectasis not related to underlying disease is still common in some populations in affluent countries. The aims of the study were to: 1) describe demographics, 2) evaluate the effectiveness of routine investigations, and 3) determine the relationship between spirometry and radiology scoring systems, in children with chronic suppurative lung disease (CSLD) living in Central Australia. Data of children living in Central Australia aged </=15 years with noncystic fibrosis CSLD were identified over 2 years. Chest high-resolution computed tomography (HRCT) scans were interpreted by a pediatric radiologist and scored according to three systems. Sixty-five children were identified, resulting in a prevalence of at least 14.7/1,000 aboriginal children aged </=15 years. Comorbidities (most common: suppurative otitis media in >70%) and early hospitalisation for pneumonia were common (median age, 0.5 years). Previous admissions for pneumonia were almost universally present and significantly more common than bronchiolitis (95% CI for proportional difference, 0.4-0.51). Although the majority did not have a treatable underlying cause, investigations had significant impact on management in 12.3% of children. None of the chest HRCT scores related to any spirometry data. In conclusion, CSLD is unacceptably common in indigenous children of this region, commences early in life, and is associated with significant comorbidities. Spirometry data do not reflect the severity of lung disease in HRCT scans. While improvement in the living standards is of utmost importance, the medical management that includes thorough investigations of these children should not be neglected.