Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent

Objectives: To report a case of acute chest syndrome (ACS) of sickle cell d isease treated successfully with nitric oxide and to review the physiologic effects of nitric oxide and its potential ability to improve outcome in AC S. Design: Descriptive case report. Setting: Eighteen-bed pediatric intensive care unit in a university childre n's hospital. Patient. A 15-yr-old black male with sickle cell disease, bilateral pulmona ry infiltrates, refractory hypoxemia, and unstable hemodynamics. Intervention: In addition to exchange transfusion, invasive hemodynamic mon itoring, and aggressive ventilatory support, inhaled nitric oxide was admin istered in the gas mixture in a concentration of 20 ppm for 72 hrs. Measurements and Main Results: cardiac output, pulmonary arterial pressure, pulmonary artery occlusion pressure, systemic vascular resistance, pulmona ry vascular resistance, shunt fraction, and alveolar-arterial oxygen gradie nt were compared with and without inhaled nitric oxide, Marked reductions i n pulmonary arterial pressure and pulmonary vascular resistance were noted, cardiac output improved, and shunt fraction and alveolar-arterial oxygen g radient were markedly reduced, The patient required decreased ventilator an d hemodynamic support and rapidly made a complete recovery. Conclusions: Nitric oxide may be beneficial for patients with ACS because o f its ability to ameliorate pulmonary hypertension and ventilation/perfusio n 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.