Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent.
OBJECTIVE
To report a case of acute chest syndrome (ACS) of sickle cell disease treated successfully with nitric oxide and to review the physiologic effects of nitric oxide and its potential ability to improve outcome in ACS.


METHODS
Descriptive case report.


METHODS
Eighteen-bed pediatric intensive care unit in a university children's hospital.


METHODS
A 15-yr-old black male with sickle cell disease, bilateral pulmonary infiltrates, refractory hypoxemia, and unstable hemodynamics.


METHODS
In addition to exchange transfusion, invasive hemodynamic monitoring, and aggressive ventilatory support, inhaled nitric oxide was administered in the gas mixture in a concentration of 20 ppm for 72 hrs.


RESULTS
Cardiac output, pulmonary arterial pressure, pulmonary artery occlusion pressure, systemic vascular resistance, pulmonary vascular resistance, shunt fraction, and alveolar-arterial oxygen gradient were compared with and without inhaled nitric oxide. Marked reductions in pulmonary arterial pressure and pulmonary vascular resistance were noted. Cardiac output improved, and shunt fraction and alveolar-arterial oxygen gradient were markedly reduced. The patient required decreased ventilator and hemodynamic support and rapidly made a complete recovery.


CONCLUSIONS
Nitric oxide may be beneficial for patients with ACS because of its ability to ameliorate pulmonary hypertension and ventilation/perfusion mismatch. Nitric oxide may confer some protection against polymerization of sickle hemoglobin and exert a reversible antiplatelet effect that may be beneficial in ACS. Further study is necessary to determine the safety and efficacy of inhaled nitric oxide as a treatment for ACS.
Sullivan
K J
KJ
Department of Anesthesiology, University of Florida College of Medicine, Gainesville, USA.
Goodwin
S R
SR
Evangelist
J
J
Moore
R D
RD
Mehta
P
P
D000208:Acute Disease; D000280:Administration, Inhalation; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D002637:Chest Pain / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D004417:Dyspnea / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D016166:Free Radical Scavengers / Q000008:administration & dosage*; D006439:Hemodynamics / Q000187:drug effects; D006451:Hemoglobin, Sickle / Q000187:drug effects / Q000378:metabolism; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D000860:Hypoxia / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D007958:Leukocyte Count; D008297:Male; D009569:Nitric Oxide / Q000008:administration & dosage*; D012121:Respiration, Artificial; D013577:Syndrome
D016166:Free Radical Scavengers; D006451:Hemoglobin, Sickle; D009569:Nitric Oxide