Objective:To assess the effects of sickle cell anemia (SCA)-on the right ventricle (RV). Study design:Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included d...Objective:To assess the effects of sickle cell anemia (SCA)-on the right ventricle (RV). Study design:Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included diastolic area index,fractional area change,free-wall mass index,ejection time corrected for heart rate (ETc),and tricuspid regurgitation(TR) gradient. Results:SCA subjects had elevated RV ETc(mean ±standard deviation,0.369 ±0.030 sec vs 0.351 ±0.022 sec; P < 0.01),diastolic area index (19.9 ±2.4 cm2/m2 vs13.2 ±2.1 cm2/m2; P < 0.01) and free-wall mass index (33.2 ±4.4 g/m2 vs 23.9 ±4.3 g/m2; P < 0.01),whereas RV fractional area change (37 ±8%vs 36 ±4%) was not different from controls. Although RV diastolic area index in SCA paralleled the normal range over time,RV free-wall mass index continued to gradually rise throughout childhood (r = 0.42; P < 0.05). TR gradients>2.5m/sec,consistent with pulmonary hypertension,were found in 5 (16%) of SCA subjects,all older than 9 years.Conclusions:RV preload and systolic function do not worsen during childhood in SCA; however,RV mass index and the prevalence of pulmonary hypertension increase consistent with rising pulmonary vascular resistance.展开更多
Objective:To test the hypothesis that children with sicklecell disease (SCD) who experienced an acute chest syndrome(ACS) hospitalization episode would have worse lung function than children with SCD without ACS episo...Objective:To test the hypothesis that children with sicklecell disease (SCD) who experienced an acute chest syndrome(ACS) hospitalization episode would have worse lung function than children with SCD without ACS episodes. Study design:Forced expiratory volume in 1 second (FEV1); forced vitalcapacity (FVC); FEV1/FVC ratio; peak expiratory flow (PEF);forced expiratory flow at 25%(FEF25),50%(FEF50),and 75%(FEF75) of FVC; airway resistance (Raw); and lung volumes were compared in 20 children with ACS and 20 aged-matched children without ACS (median age,11 years; range,6 to 16 years). Fourteen age-matched pairs were assessed before and after bronchodilator use. Results:The mean Raw (P = 0.03),TLC (P = 0.01),and RV (P = 0.003) were significantly higher in the group with ACS than in the group without ACS. There were no significant differences in the changes in lung function test results in response to bronchodilator administration between the 2 groups,but the children with ACS had a lower FEF25 (P = 0.04) and FEF75 (P = 0.03) pre-bronchodilator use and a lower mean FEV1/FVC ratio (P = 0.03) and FEF75 (P =0.03) post-bronchodilator use. Conclusions:Children with SCD who experienced an ACS hospitalization episode had significant differences in lung function compared with those who did not experience ACS episodes. Our results are compatible with the hypothesis that ACS episodes predispose children to increased airway obstruction.展开更多
文摘Objective:To assess the effects of sickle cell anemia (SCA)-on the right ventricle (RV). Study design:Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included diastolic area index,fractional area change,free-wall mass index,ejection time corrected for heart rate (ETc),and tricuspid regurgitation(TR) gradient. Results:SCA subjects had elevated RV ETc(mean ±standard deviation,0.369 ±0.030 sec vs 0.351 ±0.022 sec; P < 0.01),diastolic area index (19.9 ±2.4 cm2/m2 vs13.2 ±2.1 cm2/m2; P < 0.01) and free-wall mass index (33.2 ±4.4 g/m2 vs 23.9 ±4.3 g/m2; P < 0.01),whereas RV fractional area change (37 ±8%vs 36 ±4%) was not different from controls. Although RV diastolic area index in SCA paralleled the normal range over time,RV free-wall mass index continued to gradually rise throughout childhood (r = 0.42; P < 0.05). TR gradients>2.5m/sec,consistent with pulmonary hypertension,were found in 5 (16%) of SCA subjects,all older than 9 years.Conclusions:RV preload and systolic function do not worsen during childhood in SCA; however,RV mass index and the prevalence of pulmonary hypertension increase consistent with rising pulmonary vascular resistance.
文摘Objective:To test the hypothesis that children with sicklecell disease (SCD) who experienced an acute chest syndrome(ACS) hospitalization episode would have worse lung function than children with SCD without ACS episodes. Study design:Forced expiratory volume in 1 second (FEV1); forced vitalcapacity (FVC); FEV1/FVC ratio; peak expiratory flow (PEF);forced expiratory flow at 25%(FEF25),50%(FEF50),and 75%(FEF75) of FVC; airway resistance (Raw); and lung volumes were compared in 20 children with ACS and 20 aged-matched children without ACS (median age,11 years; range,6 to 16 years). Fourteen age-matched pairs were assessed before and after bronchodilator use. Results:The mean Raw (P = 0.03),TLC (P = 0.01),and RV (P = 0.003) were significantly higher in the group with ACS than in the group without ACS. There were no significant differences in the changes in lung function test results in response to bronchodilator administration between the 2 groups,but the children with ACS had a lower FEF25 (P = 0.04) and FEF75 (P = 0.03) pre-bronchodilator use and a lower mean FEV1/FVC ratio (P = 0.03) and FEF75 (P =0.03) post-bronchodilator use. Conclusions:Children with SCD who experienced an ACS hospitalization episode had significant differences in lung function compared with those who did not experience ACS episodes. Our results are compatible with the hypothesis that ACS episodes predispose children to increased airway obstruction.
