It is important to measure the rate of haemolysis in patients with sickle cell disease (SCD) to identify aplastic crises and indirectly assess the rate of vaso-occlusion and sequestration. The aim of this study was to...It is important to measure the rate of haemolysis in patients with sickle cell disease (SCD) to identify aplastic crises and indirectly assess the rate of vaso-occlusion and sequestration. The aim of this study was to assess whether end-tidal carbon monoxide (ETCOc) levels in children with sickle cell disease (SCD) could be measured reproducibly, reflected haemolysis and whether they were elevated compared to those of similarly aged, ethnic matched children without SCD (controls). ETCOc levels were measured non-invasively in 87 SCD children (age range 2.3-17.6 years) and 26 age and ethnic origin matched healthy controls using an electro-chemical sensor. The within-and between-occasion reproducibilities were assessed in ten and 15 SCD children respectively. ETCOc levels of 15 SCD children undergoing regular transfusions were related to carboxyhaemoglobin, haemoglobin and bilirubin levels. The within and between occasions’ mean intrasubject coefficients of reproducibility were 5% and 18% respectively. Positive correlations were found between the ETCOc and carboxyhaemoglobin ( P =0.007) and bilirubin ( P =0.02) levels, and a significant negative correlation between the ETCOc and haemoglobin ( P =0.0002) levels. The mean and SD ETCOc levels of the SCD children (4.9 ppm; SD 1.7 ppm) were significantly higher than that of the controls (mean 1.3 ppm; SD 0.4 ppm) (difference between means 3.60; 95% C.I. 2.93-4.28; P < 0.0001). Conclusion: These results suggest that measurement of endtidal carbon monoxide levels is a reliable and useful method to monitor haemolysis in children with sickle cell disease.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It is important to measure the rate of haemolysis in patients with sickle cell disease (SCD) to identify aplastic crises and indirectly assess the rate of vaso-occlusion and sequestration. The aim of this study was to assess whether end-tidal carbon monoxide (ETCOc) levels in children with sickle cell disease (SCD) could be measured reproducibly, reflected haemolysis and whether they were elevated compared to those of similarly aged, ethnic matched children without SCD (controls). ETCOc levels were measured non-invasively in 87 SCD children (age range 2.3-17.6 years) and 26 age and ethnic origin matched healthy controls using an electro-chemical sensor. The within-and between-occasion reproducibilities were assessed in ten and 15 SCD children respectively. ETCOc levels of 15 SCD children undergoing regular transfusions were related to carboxyhaemoglobin, haemoglobin and bilirubin levels. The within and between occasions’ mean intrasubject coefficients of reproducibility were 5% and 18% respectively. Positive correlations were found between the ETCOc and carboxyhaemoglobin ( P =0.007) and bilirubin ( P =0.02) levels, and a significant negative correlation between the ETCOc and haemoglobin ( P =0.0002) levels. The mean and SD ETCOc levels of the SCD children (4.9 ppm; SD 1.7 ppm) were significantly higher than that of the controls (mean 1.3 ppm; SD 0.4 ppm) (difference between means 3.60; 95% C.I. 2.93-4.28; P < 0.0001). Conclusion: These results suggest that measurement of endtidal carbon monoxide levels is a reliable and useful method to monitor haemolysis in children with sickle cell disease.
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.
