title: Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies.
abstract: Recurrent and unpredictable episodes of vaso-occlusion are the hallmark of sickle cell disease. Symptomatic management and prevention of these events using the fetal hemoglobin-reactivating agent hydroxyurea are currently the mainstay of treatment. Discoveries over the past 2 decades have highlighted the important contributions of various cellular and soluble participants in the vaso-occlusive cascade. The role of these elements and the opportunities for therapeutic intervention are summarized in this review.
authors: Departments of 1Pediatrics.
authors: Deepa
authors: D
authors: Manwani
authors: Paul S
authors: PS
authors: Frenette
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D000986:Antisickling Agents / Q000627:therapeutic use; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D014652:Vascular Diseases / Q000188:drug therapy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea