Airway and alveolar nitric oxide production, lung function, and pulmonary blood flow in sickle cell disease

TY - JOUR

T1 - Airway and alveolar nitric oxide production, lung function, and pulmonary blood flow in sickle cell disease

AU - Lunt, Alan

AU - Ahmed, Na'eem

AU - Rafferty, Gerrard F.

AU - Dick, Moira

AU - Rees, David

AU - Height, Sue

AU - Thein, Swee Lay

AU - Greenough, Anne

PY - 2016/3

Y1 - 2016/3

N2 - Background: Children with sickle cell disease (SCD) often have obstructive lung function abnormalities which could be due to asthma or increased pulmonary blood volume; it is important to determine the underlying mechanism to direct appropriate treatment. In asthmatics, exhaled nitric oxide (F eNO) is elevated. F eNO, however, can also be raised due to increased alveolar production. Our aim, therefore, was to determine if airway or alveolar NO production differed between SCD children and ethnic and age-matched controls. Methods: Lung function, airway NO flux and alveolar NO production, and effective pulmonary blood flow were assessed in 18 SCD children and 18 ethnic and age-matched controls. Results: The SCD children compared to the controls had a higher respiratory system resistance (P = 0.0008), alveolar NO production (P = 0.0224), and pulmonary blood flow (P <0.0001), but not airway NO flux. There was no significant correlation between F eNO and respiratory system resistance in either group, but in the SCD children, there were correlations between alveolar NO production (P = 0.0006) and concentration (P <0.0001) and pulmonary blood flow. Conclusion: Airway NO flux was not elevated in the SCD children nor correlated with airways obstruction, suggesting that airways obstruction, at least in some SCD children, is not due to asthma.

AB - Background: Children with sickle cell disease (SCD) often have obstructive lung function abnormalities which could be due to asthma or increased pulmonary blood volume; it is important to determine the underlying mechanism to direct appropriate treatment. In asthmatics, exhaled nitric oxide (F eNO) is elevated. F eNO, however, can also be raised due to increased alveolar production. Our aim, therefore, was to determine if airway or alveolar NO production differed between SCD children and ethnic and age-matched controls. Methods: Lung function, airway NO flux and alveolar NO production, and effective pulmonary blood flow were assessed in 18 SCD children and 18 ethnic and age-matched controls. Results: The SCD children compared to the controls had a higher respiratory system resistance (P = 0.0008), alveolar NO production (P = 0.0224), and pulmonary blood flow (P <0.0001), but not airway NO flux. There was no significant correlation between F eNO and respiratory system resistance in either group, but in the SCD children, there were correlations between alveolar NO production (P = 0.0006) and concentration (P <0.0001) and pulmonary blood flow. Conclusion: Airway NO flux was not elevated in the SCD children nor correlated with airways obstruction, suggesting that airways obstruction, at least in some SCD children, is not due to asthma.

UR - http://www.scopus.com/inward/record.url?scp=84982313526&partnerID=8YFLogxK

U2 - 10.1038/pr.2015.217

DO - 10.1038/pr.2015.217

M3 - Article

AN - SCOPUS:84982313526

SN - 0031-3998

VL - 79

SP - 313

EP - 317

JO - Pediatric Research

JF - Pediatric Research

IS - 2

ER -