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Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease.

Montgomery GS, Sagel SD, Taylor AL, Abman SH

Division of Pediatric Pulmonology, Department of Pediatrics, Indiana University Medical Center, Indianapolis, Indiana, USA. gsmontgo@iupui.edu

Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy.

Published 6 March 2006 in Pediatr Pulmonol, 41(4): 383-5.
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