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Pathophysiology of sickle cell disease
Since the discovery of sickle cell disease (SCD) in 1910, enormous strides have been made
in the elucidation of the pathogenesis of its protean complications, which has inspired recent …
in the elucidation of the pathogenesis of its protean complications, which has inspired recent …
Intravascular hemolysis and the pathophysiology of sickle cell disease
Hemolysis is a fundamental feature of sickle cell anemia that contributes to its
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …
The red blood cell—inflammation vicious circle in sickle cell disease
Sickle cell disease (SCD) is a genetic disease caused by a single mutation in the β-globin
gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS) …
gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS) …
Hematopoietic stem cell transplantation in thalassemia major and sickle cell disease: indications and management recommendations from an international expert …
Thalassemia major and sickle cell disease are the two most widely disseminated hereditary
hemoglobinopathies in the world. The outlook for affected individuals has improved in recent …
hemoglobinopathies in the world. The outlook for affected individuals has improved in recent …
Microvesicles in vascular homeostasis and diseases
Microvesicles are members of the family of extracellular vesicles shed from the plasma
membrane of activated or apoptotic cells. Microvesicles were initially characterised by their …
membrane of activated or apoptotic cells. Microvesicles were initially characterised by their …
Circulating cell membrane microparticles transfer heme to endothelial cells and trigger vasoocclusions in sickle cell disease
Intravascular hemolysis describes the relocalization of heme and hemoglobin (Hb) from
erythrocytes to plasma. We investigated the concept that erythrocyte membrane …
erythrocytes to plasma. We investigated the concept that erythrocyte membrane …
Genetic variation and sickle cell disease severity: a systematic review and meta-analysis
Importance Sickle cell disease (SCD) is a monogenic disorder, yet clinical outcomes are
influenced by additional genetic factors. Despite decades of research, the genetics of SCD …
influenced by additional genetic factors. Despite decades of research, the genetics of SCD …
Cardiovascular complications and risk of death in sickle-cell disease
In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to
polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and …
polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and …
Pathophysiology and treatment of pulmonary hypertension in sickle cell disease
Pulmonary hypertension affects∼ 10% of adult patients with sickle cell disease (SCD),
particularly those with the homozygous genotype. An increase in pulmonary artery systolic …
particularly those with the homozygous genotype. An increase in pulmonary artery systolic …
Multiple-ancestry genome-wide association study identifies 27 loci associated with measures of hemolysis following blood storage
Background The evolutionary pressure of endemic malaria and other erythrocytic pathogens
has shaped variation in genes encoding erythrocyte structural and functional proteins …
has shaped variation in genes encoding erythrocyte structural and functional proteins …