XXXX uptake kinetics and exercise capacity in @entity1 with @entity1577 .

@entity1 @entity4962 @entity138 @entity26 @entity1386 @entity1577
UNASSIGNED: Exercise capacity, an objective measure of exercise intolerance, is known to predict quality of life and mortality in @entity1577 ( @entity1577 ). The mechanisms for exercise intolerance in @entity1 with @entity1577 ( @entity1577 ), however, have yet to be fully elucidated. Accordingly, this study sought to investigate @entity26 uptake kinetics and the impact of @entity1386 ( @entity1386 ) on exercise capacity in young @entity1 with @entity1577 . 16 young @entity1 with @entity1577 (age 13        4 years; 10 female) and 15 matched controls (age 14        3 years; nine female) participated. Pulmonary function and a maximal exercise test on a cycle ergometer using the Godfrey protocol were performed. Exercise capacity ( @entity4962 peak), @entity4962 response time ( @entity4962 @entity138 ), and functional @entity4962 gain (  @entity4962 /  WR) were all determined. Lung function was the only demographic parameter significantly lower (P   <   0.05) in @entity1577 compared to controls. Exercise capacity was lower in @entity1577 (P   <   0.014) only when @entity4962 peak was normalized for @entity1386 (43.5        7.7 vs. 50.6        7.4 @entity1386 ) or expressed as % predicted (70.1        14.3 vs. 85.4        16.0%). The @entity4962 @entity138 was slower (36.1        15.1 vs. 25.0        12.4   sec; P   =   0.03) and the   @entity4962 /  WR slope was lower (8.4        3 ml/min/watt vs. 10.1        1.4 ml/min/watt; P   =   0.02) in @entity1 compared to controls, respectively. In conclusion, a delayed @entity4962 response time coupled with the lower functional @entity4962 gain (  @entity4962 /  WR) suggest that young @entity1 with @entity1577 have impairment in @entity26 transport and @entity26 utilization by the muscles. These data in addition to differences in @entity4962 peak normalized for @entity1386 provide some insight that muscle mass and muscle metabolism contribute to exercise intolerance in @entity1577 . Pediatr Pulmonol. 2015; 50:647-654.    2015 Wiley Periodicals, Inc.
