Vasculopathy in sickle cell disease: from red blood cell sickling to vascular dysfunction

E Nader, N Conran, M Romana… - Comprehensive …, 2011 - Wiley Online Library
Sickle cell disease (SCD) is a hereditary disorder that leads to the production of an
abnormal hemoglobin, hemoglobin S (HbS). HbS polymerizes in deoxygenated conditions …

Vascular pathophysiology of sickle cell disease

P Connes, C Renoux, P Joly, E Nader - La Presse Médicale, 2023 - Elsevier
Sickle cell disease (SCD) is an hereditary disorder characterized by the production of an
abnormal hemoglobin called hemoglobin S (HbS). HbS may polymerize in deoxygenated …

Vascular complications of sickle cell disease

A Usmani, RF Machado - Clinical hemorheology and …, 2018 - content.iospress.com
Sickle cell disease (SCD) is a monogenetic disorder caused by a mutation in the β-globin
gene HBB leading to polymerization of red blood cells causing damage to cell membranes …

Sickle cell disease as a vascular disorder

SF Ofori-Acquah - Expert Review of Hematology, 2020 - Taylor & Francis
Introduction In sickle cell disease (SCD), hemoglobin S (HbS) red blood cells (RBCs) are
characteristically deformed and inflexible. Often breaking down in the circulation, they …

[HTML][HTML] Vascular occlusion in sickle cell disease: current concepts and unanswered questions

RB Francis Jr, CS Johnson - Blood, 1991 - Elsevier
THE SICKLE CELL diseases (SCD) are inherited disorders of hemoglobin (Hb) structure
and synthesis, and include homozygous sickle cell anemia (Hb SS), sickle-C disease (Hb …

Sickle cell vasculopathy: vascular phenotype on fire!

GJ Kato - The Journal of Physiology, 2018 - pmc.ncbi.nlm.nih.gov
Clinicians, scientists and lay people alike grasp the concept that sickle-shaped cells flow
poorly in the circulation. Red cell stiffness is the basis of sickle cell vaso-occlusion, but …

Vaso-occlusion in sickle cell disease: is autonomic dysregulation of the microvasculature the trigger?

S Veluswamy, P Shah, CC Denton… - Journal of clinical …, 2019 - mdpi.com
Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by polymerization
of hemoglobin S upon deoxygenation that results in the formation of rigid sickled-shaped red …

[HTML][HTML] Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine and new research directions

GJ Kato, RP Hebbel, MH Steinberg… - American journal of …, 2009 - ncbi.nlm.nih.gov
Sickle cell disease has been very well characterized as a single amino acid molecular
disorder of hemoglobin leading to its pathological polymerization, with resulting red cell …

The red blood cell—inflammation vicious circle in sickle cell disease

E Nader, M Romana, P Connes - Frontiers in immunology, 2020 - frontiersin.org
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) …

Newer aspects of the pathophysiology of sickle cell disease vaso-occlusion

N Conran, CF Franco-Penteado, FF Costa - Hemoglobin, 2009 - Taylor & Francis
Sickle cell disease is an inherited disorder of hemoglobin (Hb) synthesis, caused by a single
nucleotide substitution (GTG> GAG) at the sixth codon of the β-globin gene, leading to the …