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 …

Sickle cell dehydration: Pathophysiology and therapeutic applications

C Brugnara - Clinical hemorheology and microcirculation, 2018 - content.iospress.com
Cell dehydration is a distinguishing characteristic of sickle cell disease and an important
contributor to disease pathophysiology. Due to the unique dependence of Hb S …

Early initiation of hydroxyurea (hydroxycarbamide) using individualised, pharmacokinetics‐guided dosing can produce sustained and nearly pancellular expression of …

CT Quinn, O Niss, M Dong, A Pfeiffer… - British journal of …, 2021 - Wiley Online Library
Hydroxyurea (hydroxycarbamide) is an effective treatment for sickle cell anaemia (SCA), but
clinical responses depend primarily upon the degree of fetal haemoglobin (HbF) induction …

Safety, Pharmacokinetics, and Pharmacodynamics of Etavopivat (FT‐4202), an Allosteric Activator of Pyruvate Kinase‐R, in Healthy Adults: A Randomized, Placebo …

S Forsyth, P Schroeder, J Geib… - Clinical …, 2022 - Wiley Online Library
Abstract Etavopivat (FT‐4202) is an orally administered, small‐molecule allosteric activator
of erythrocyte pyruvate kinase‐R (PKR) in clinical development for the treatment of sickle …

Sickle cell disease in the new era: advances in drug treatment

MT Lee, UO Ogu - Transfusion and Apheresis Science, 2022 - Elsevier
Sickle cell disease is an inherited blood disorder afflicting an estimated 100,000 individuals
in the United States and over 20 million people worldwide. The disease is heralded as the …

Caffeic acid: an antioxidant with novel antisickling properties

T Kassa, JG Whalin, MP Richards, AI Alayash - FEBS Open Bio, 2021 - Wiley Online Library
It is well documented that caffeic acid (3, 4‐dihydroxycinnamic acid)(CA) interacts with and
inhibits the oxidative reactions of myoglobin (Mb) and hemoglobin (Hb), and this interaction …

Individual red blood cell fetal hemoglobin quantification allows to determine protective thresholds in sickle cell disease

N Hebert, MG Rakotoson, G Bodivit… - American Journal of …, 2020 - Wiley Online Library
Polymerization of the sickle hemoglobin (HbS) is a key determinant of sickle cell disease
(SCD), an inherited blood disorder. Fetal hemoglobin (HbF) is a major modulator of the …

[HTML][HTML] Research in sickle cell disease: from bedside to bench to bedside

GS Cisneros, SL Thein - Hemasphere, 2021 - journals.lww.com
Sickle cell disease (SCD) is an exemplar of bidirectional translational research, starting with
a remarkable astute observation of the abnormally shaped red blood cells that motivated …

Allosteric control of hemoglobin S fiber formation by oxygen and its relation to the pathophysiology of sickle cell disease

ER Henry, T Cellmer… - Proceedings of the …, 2020 - National Acad Sciences
The pathology of sickle cell disease is caused by polymerization of the abnormal
hemoglobin S upon deoxygenation in the tissues to form fibers in red cells, causing them to …

Fetal hemoglobin in sickle cell anemia

MH Steinberg - Blood, The Journal of the American Society of …, 2020 - ashpublications.org
Fetal hemoglobin (HbF) can blunt the pathophysiology, temper the clinical course, and offer
prospects for curative therapy of sickle cell disease. This review focuses on (1) HbF …