TY - JOUR
T1 - Decreased exhaled nitric oxide in sickle cell disease
T2 - Relationship with chronic lung involvement
AU - Girgis, Reda E.
AU - Qureshi, Mohammed A.
AU - Abrams, Judith
AU - Swerdlow, Paul
PY - 2003/3/1
Y1 - 2003/3/1
N2 - A deficiency in airway nitric oxide (NO) could contribute to pulmonary vaso-occlusion in sickle cell disease (SCD). We measured the fractional expired concentration of NO (FENO) by chemiluminescence during a slow vital capacity maneuver against a positive pressure of 16 cm H2O at an expiratory flow rate of 50 mL/sec in 44 stable ambulatory adults with SCD and 30 healthy controls. A history of acute chest syndrome was present in 29 patients, and 22 complained of dyspnea. Mean ± SD FENO was significantly reduced in the SCD group compared with controls (14.8 ± 8.4 vs. 24.9 ± 13.5 ppb, P < 0.001). SCD patients with dyspnea had lower FENO than those without dyspnea (10.1 ± 5.7 vs. 19.6 ± 8 ppb, P < 0.001) and those with a history of ACS had lower values than those no episodes of ACS (13.0 ± 8.3 vs. 18.4 ± 7.6 ppb, P < 0.05). There was a weak correlation between FENO and percent-predicted DLCO (r = 0.4, P = 0.02) among the SCD patients. We conclude that exhaled NO is reduced in adults with SCD, and this may play a role in the pathogenesis of acute chest syndrome and chronic sickle cell lung disease.
AB - A deficiency in airway nitric oxide (NO) could contribute to pulmonary vaso-occlusion in sickle cell disease (SCD). We measured the fractional expired concentration of NO (FENO) by chemiluminescence during a slow vital capacity maneuver against a positive pressure of 16 cm H2O at an expiratory flow rate of 50 mL/sec in 44 stable ambulatory adults with SCD and 30 healthy controls. A history of acute chest syndrome was present in 29 patients, and 22 complained of dyspnea. Mean ± SD FENO was significantly reduced in the SCD group compared with controls (14.8 ± 8.4 vs. 24.9 ± 13.5 ppb, P < 0.001). SCD patients with dyspnea had lower FENO than those without dyspnea (10.1 ± 5.7 vs. 19.6 ± 8 ppb, P < 0.001) and those with a history of ACS had lower values than those no episodes of ACS (13.0 ± 8.3 vs. 18.4 ± 7.6 ppb, P < 0.05). There was a weak correlation between FENO and percent-predicted DLCO (r = 0.4, P = 0.02) among the SCD patients. We conclude that exhaled NO is reduced in adults with SCD, and this may play a role in the pathogenesis of acute chest syndrome and chronic sickle cell lung disease.
KW - Acute chest syndrome
KW - Airway nitric oxide
KW - Chronic sickle cell lung disease
KW - Dyspnea
KW - Pulmonary hypertension
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U2 - 10.1002/ajh.10284
DO - 10.1002/ajh.10284
M3 - Article
C2 - 12605389
AN - SCOPUS:0037371369
SN - 0361-8609
VL - 72
SP - 177
EP - 184
JO - American journal of hematology
JF - American journal of hematology
IS - 3
ER -