Sickle cell vasculopathy: vascular phenotype on fire!
نویسندگان
چکیده
منابع مشابه
Vasculopathy and pulmonary hypertension in sickle cell disease.
Sickle cell disease (SCD) is an autosomal recessive disorder in the gene encoding the β-chain of hemoglobin. Deoxygenation causes the mutant hemoglobin S to polymerize, resulting in rigid, adherent red blood cells that are entrapped in the microcirculation and hemolyze. Cardinal features include severe painful crises and episodic acute lung injury, called acute chest syndrome. This population, ...
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Sickle cell anemia (SCA)-associated cerebral vasculopathy and moyamoya is a unique entity reflecting the abnormal interactions between sickled red blood cells (RBCs) and the cerebral arterial endothelium. Endothelial injury, coagulation activation, and the inflammatory response generated by sickled RBCs are implicated in the development of cerebral vasculopathy, but the pathophysiology remains ...
متن کاملMechanisms of vasculopathy in sickle cell disease and thalassemia.
Many mechanisms contribute to the complex pathophysiology of sickle cell disease (SCD), with dysfunction of the vascular endothelium as a unifying theme. Specifically, hemolysis-associated low arginine and nitric oxide (NO) bioavailability, amplified by NO synthase uncoupling, elevated arginase activity, superoxide production, oxidative stress, accumulation of arginine analogs such as asymmetri...
متن کاملVascular Obstruction in Sickle Cell Disease
Initially, the pathophysiology of sickle cell anemia was attributed to deoxygenation-induced polymerization of a mutant form of hemoglobin, hemoglobin S, as well as sickling of red blood cells that plug blood vessels causing the onset of a painful vasoocclusive crisis. Although hemoglobin S polymerization is central to the pathophysiology of this disease, the initiation of a vasoocclusive episo...
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ژورنال
عنوان ژورنال: The Journal of Physiology
سال: 2018
ISSN: 0022-3751,1469-7793
DOI: 10.1113/jp276705