نتایج جستجو برای: intrahepatic sickling
تعداد نتایج: 12228 فیلتر نتایج به سال:
Rapid transformatIon of discoid hemoglobin SS erythrocytes into grossly distorted sickled forms suggests alteration of surface/volume ratio. We, therefore, examined the effect of deoxygenation on red cell volume. ‘31llaholed albumin was used as a marker of plasma volume in normal (Hb AA), sickle (Hb SS), and sickle-C (Hb SC) blood. Heparinized blood was incubated for 30 mm at 37#{176}Cin a tpno...
Sphingosine-1-phosphate (S1P) is a bioactive lipid that regulates multicellular functions through interactions with its receptors on cell surfaces. S1P is enriched and stored in erythrocytes; however, it is not clear whether alterations in S1P are involved in the prevalent and debilitating hemolytic disorder sickle cell disease (SCD). Here, using metabolomic screening, we found that S1P is high...
Two human hemoglobins designed to inhibit the polymerization of sickle hemoglobin (Hb S; alpha 2 beta S2) have been produced. Mutations that disrupt the ability of Hb S to form polymers were introduced into the normal human beta-globin gene by site-specific mutagenesis. These mutations affect the axial and lateral contacts in the sickle fiber. The recombinant hemoglobin designated anti-sickling...
Deoxygenation-induced cation movements in sickle cells were inhibited 80% to 85% by the anion transport inhibitor, 4,4'-diisothiocyano-2,2'disulfostilbene (DIDS). Morphologic sickling was not altered by DIDS treatment, demonstrating that morphologic sickling was not sufficient to produce cation leaks in sickle cells. DIDS inhibition of deoxygenation-induced cation flux was not affected when l- ...
Autologous hematopoietic stem cell gene therapy is an approach to treating sickle cell disease (SCD) patients that may result in lower morbidity than allogeneic transplantation. We examined the potential of a lentiviral vector (LV) (CCL-βAS3-FB) encoding a human hemoglobin (HBB) gene engineered to impede sickle hemoglobin polymerization (HBBAS3) to transduce human BM CD34+ cells from SCD donors...
Sickle cell disease (SCD) is an inherited hemolytic disorder, defined by a point mutation in the β-globin gene. Stress conditions such as infection, inflammation, dehydration, and hypoxia trigger erythrocyte sickling. Sickled red blood cells (RBCs) hemolyze more rapidly, show impaired deformability, increased adhesive properties to endothelium. In proinflammatory, pro-coagulative environment wi...
background all conditions that interfere with blood flow at any level within the portal system can lead to portal hypertension. for better management of this disorder, it is important to determine the underlying cause. in previous studies, extra-hepatic disorders have been reported as the main cause of portal hypertension in children. in this study, we investigate the underlying causes of por...
this report presents an 11 year-old girl with benign recurrent cholestasis (bric) who developed episodes of severe jaundice and pruritus at the ages of 2.5 and 10 years. each episode lasted for 3-4 months. the peak level of serum bilirubin reached 33-37 mg/dl.liver function tests were all normal during the attack except for increased alkaline phosphatase and prolonged prothrombin time responsiv...
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