Pathophysiology and recent therapeutic insights of sickle cell disease.

Annals of Hematology
Firdosh Shah, Mitesh Dwivedi

Abstract

Sickle cell disease (SCD) is an autosomal recessive blood disorder which occurs due to point mutation in the β-globin chain of hemoglobin. Since the past decades, various therapies have been put forth, which are based on obstructing pathophysiological mechanisms of SCD including inhibition of Gardos channel and cation fluxes which in turn prevents sickle erythrocyte destruction and dehydration. The pharmacological approaches are based on the mechanism of reactivating γ-globin expression by utilizing fetal hemoglobin (HbF)-inducing drugs such as hydroxyurea. In SCD, gene therapy could be considered as a promising tool which involves modifying mutation at the gene-specific target by either promoting insertion or deletion of globins. Although there are various therapies emerged so far in the treatment of SCD, many of them have faced a major setback in most of developing countries in terms of cost, unavailability of expertise, and suitable donor. Therefore, in addition to pathophysiological aspects, this review will discuss new advancements and approaches made in the therapeutic domain of SCD including a viewpoint of modulating hemoglobin in SCD by the intervention of probiotics.

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Citations

Jan 19, 2021·Human Molecular Genetics·Kevin Esoh, Ambroise Wonkam
Mar 5, 2021·Journal of Blood Medicine·Ugochi O OguPatricia Adams-Graves
Apr 15, 2021·Current Opinion in Anaesthesiology·Ada Ezihe-Ejiofor, Jaleesa Jackson
Jul 3, 2021·International Journal of Molecular Sciences·Dito AnurogoJeanne Adiwinata Pawitan
Aug 31, 2021·Biologics : Targets & Therapy·Misganaw Asmamaw, Belay Zawdie

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