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

被引：14

作者：

Montgomery, GS

Sagel, SD

Taylor, AL

Abman, SH

机构：

[1] Indiana Univ, Med Ctr, Div Pediat Pulmonol, Indianapolis, IN USA

[2] Univ Colorado, Denver Hlth Sci Ctr, Dept Pediat, Sect Pulm Med, Denver, CO 80202 USA

[3] Univ Colorado, Denver Hlth Sci Ctr, Dept Pediat, Cardiol Sect, Denver, CO 80202 USA

[4] Univ Colorado, Denver Hlth Sci Ctr, Dept Pediat, Pediat Heart Lung Ctr, Denver, CO 80202 USA

来源：

PEDIATRIC PULMONOLOGY | 2006年 / 41卷 / 04期

关键词：

cystic fibrosis; exercise; pulmonary hypertension; sildenafil; 6-min walk test; phosphodiesterase inhibitor; echocardiogram;

D O I：

10.1002/ppul.20393

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：383 / 385

页数：3

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