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

Abstract:

: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.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Montgomery GS,Sagel SD,Taylor AL,Abman SH

doi

10.1002/ppul.20393

subject

Has Abstract

pub_date

2006-04-01 00:00:00

pages

383-5

issue

4

eissn

8755-6863

issn

1099-0496

journal_volume

41

pub_type

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