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Ann Hematol. 2018 May;97(5):755-762. doi: 10.1007/s00277-017-3199-z. Epub 2017 Dec 6.

Sleep-disordered breathing in patients with sickle cell disease.

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Department of Medicine, Brown University Warren Alpert Medical School, Providence, RI, USA.
Department of Medicine, Howard University College of Medicine, Washington, DC, USA.
Department of Medicine, Brown University Warren Alpert Medical School, Providence, RI, USA.
Rhode Island Hospital, Room 140, APC Building593 Eddy Street, Providence, RI, 02903, USA.


Sickle cell disease is one of the most common hereditary hemoglobinopathies worldwide, and its vaso-occlusive and hemolytic crises cause considerable patient morbidity. A growing body of evidence has shown that sleep-disordered breathing, and in particular, obstructive sleep apnea, occurs at high frequency in the sickle cell population, and that there is significant overlap in the underlying pathophysiology of these two conditions. Through a variety of mechanisms including nocturnal hypoxemia and increased oxidative stress, production of pro-inflammatory cytokines, and endothelial dysfunction, sickle cell anemia and sleep-disordered breathing potentiate each other's clinical effects and end-organ complications. Here, we will review the shared pathophysiologic mechanisms of these conditions and discuss their clinical sequelae. We will also examine the results of studies that have been carried out with clinical intervention of nocturnal hypoxemia in patients with sickle cell disease in the attempts to overcome the complications of the disease. Finally, we will propose the areas of investigation that merit further investigations in future in patients with sickle cell disease and sleep-disordered breathing.


Clinical characteristics; Common pathophysiologic pathways; Sickle cell disease; Sleep-disordered breathing

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