Clinical Findings and Lung Pathology in Children with Cystic Fibrosis

Abstract

Cystic fibrosis pulmonary disease is assessed by pulmonary function tests, arterial blood gases, and chest X-rays, but the correlation with lung pathology is unknown. We reviewed the clinical findings and lung pathology of 21 cystic fibrosis patients who had lung transplant. Pulmonary function tests, Brasfield scores, arterial blood gases, and age were correlated with lung pathology. All patients had severe Brasfield scores (9.0 +/- 3.2), airways obstruction (FEV1 25.6 +/- 5.6% predicted, FEF(25-75%) 11.0 +/- 4.5% predicted), and hyperinflation (residual volume [RV] 341.8 +/- 75.8% predicted). All patients were hypoxemic (PO2 64.2 +/- 8.2 mm Hg), and 5 of 21 (24%) were hypercapneic (PCO2 > 50 mm Hg). Pulmonary function tests and Brasfield scores were within a narrow range, and did not allow correlation with lung pathology. Small airway density (airways < 2 mm/cm2) decreased with increasing age. There were no differences in small airways inflammation and fibrous narrowing between the hypercapneic and nonhypercapneic patients, but the percent of smallest airways (airways < 0.35 mm) was significantly lower in the hypercapneic group. We conclude that there is significant correlation between airway pathology and increased age and CO2 retention. We speculate that decreased small airway density in older patients and the decreased proportion of smallest airways in hypercapneic patients is caused by increased dilatation of small airways.

Computed tomographic imaging of the airways: relationship to structure and function

“…Airway disease in CF is characterised by mucus plugging, chronic infection and an excessive inflammatory response, leading to peripheral airway changes in the first few months of life [101][102][103][104][105][106][107][108][109][110][111][112][113][114][115][116][117][118][119]. The characteristic airway abnormalities are bronchiectasis, thickening of the airway wall and mucus plugging [97,103,107,[120][121][122][123][124][125][126], as shown in figure 5.…”

Improved treatment response to dornase alfa in cystic fibrosis patients using controlled inhalation

“…Because most CF patients show lung function evidence of persistent small airway obstruction [13,15] and patients with advanced lung disease have extensive destruction of the small airways [16,17], more efficient treatment of the small airways is still needed [18]. This could be achieved through improved delivery of dornase alfa to the small airways.…”

Constrictive Bronchiolitis in Cystic Fibrosis Adolescents with Refractory Pulmonary Decline

“…Although CF pulmonary decline is attributed frequently to progressive mucus plugging and bronchiectasis (30)(31)(32)(33)(34)(35), peribronchiolar stenosis has been reported ORIGINAL RESEARCH frequently in "older" patients with CF (36)(37)(38). Lesions in our biopsied adolescent patients reflect similarities to findings in end-stage disease, suggesting the important contribution of distal airway limitation to respiratory decline.…”