Am. J. Respir. Crit. Care Med., Volume 165, Number 8, April 2002, 1172-1175

Clinical Findings and Lung Pathology in Children with Cystic Fibrosis

Refika Hamutcu, Jon M. Rowland, Monica V. Horn, Cornelia Kaminsky, Eithne F. MacLaughlin, Vaughn A. Starnes, and Marlyn S. Woo

Divisions of Pediatric Pulmonology, Anatomic Pathology, Cardiothoracic Surgery and Pediatric Radiology, Childrens Hospital Los Angeles and Keck School of Medicine, University of Southern California, Los Angeles, California

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.

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