Results 1 to 10 of about 398,905 (201)

A Case of Hemoglobin Sickle-D Punjab [PDF]

Indian J Hematol Blood Transfus, 2019
W. Ali, Mili Jain, S. Agarwal, Ashutosh Kumar   +3 more
semanticscholar   +2 more sources

Pathophysiology of Sickle Cell Disease

Annual Review of Pathology: Mechanisms of Disease, 2019
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 advances in targeted molecular therapies. In SCD, a single amino acid
Prithu Sundd, Mark T Gladwin, Enrico M Novelli   +2 more
exaly   +2 more sources

Genetic variants and cell-free hemoglobin processing in sickle cell nephropathy

Haematologica, 2015
Intravascular hemolysis and hemoglobinuria are associated with sickle cell nephropathy. ApoL1 is involved in cell-free hemoglobin scavenging through association with haptoglobin-related protein.
Santosh L. Saraf, Xu Zhang, Binal Shah, Tamir Kanias, Krishnamurthy P. Gudehithlu, Rick Kittles, Roberto F. Machado, Jose A.L. Arruda, Mark T. Gladwin, Ashok K. Singh, Victor R. Gordeuk   +10 more
doaj   +2 more sources

Radiologic Evaluation of Paranasal Sinuses in Sickle Cell Anemia and Thalassemia: Case–Control Study [PDF]

Laryngoscope Investigative Otolaryngology
Background Sickle cell disease and thalassemia are inherited hematological disorders that are common worldwide. These patients suffer from chronic hemolytic anemia, which can result in bone marrow dysfunction and, in rare cases, extramedullary ...
Maha A. Alharbi, Ayat AlDarwish, Reem M. Alamier, Nawal E. Omer, Mahmoud A. Alabbad, Abdulmohsin M. Aljassem, Qasem M. ALalwan, Danah H. Althomaly, Hussain Abdullali Albaharna   +8 more
doaj   +2 more sources

Treatment of sickle cell disease by increasing oxygen affinity of hemoglobin.

Blood, 2021
The issue of treating sickle cell disease with drugs that increase hemoglobin oxygen affinity has come to the fore with the FDA approval in 2019 of voxelotor, the only anti-sickling drug approved since hydroxyurea in 1998.
E. Henry, B. Metaferia, Quan Li, Julia Harper, R. Best, Kristen E. Glass, Troy Cellmer, Emily B. Dunkelberger, Anna K. Conrey, S. Thein, H. Bunn, W. Eaton   +11 more
semanticscholar   +1 more source

Elastic property of sickle and normal hemoglobin protein: Molecular dynamics

AIP Advances, 2022
This work focuses on identifying the conformational stability and binding components in sickle and normal hemoglobin to explore the elastic properties and realize the stiffness by using molecular dynamics simulation. Our investigation shows that a larger
Jhulan Powrel, Narayan Prasad Adhikari
doaj   +1 more source

Atomic force microscopy reveals involvement of the cell envelope in biomechanical properties of sickle erythrocytes

BMC Biology, 2023
Background Intracellular hemoglobin polymerization has been supposed to be the major determinant for the elevated rigidity/stiffness of sickle erythrocytes from sickle cell anemia (SCA) patients.
Kun Wang, Zhiqiang Li, Ogechukwu Egini, Raj Wadgaonkar, Xian-Cheng Jiang, Yong Chen   +5 more
doaj   +1 more source

Fetal Hemoglobin in Sickle Cell Anemia.

Blood, 2020
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.
M. Steinberg
semanticscholar   +1 more source

Frequency of sickle cell hemoglobin in high-performance liquid chromatography received in a centralized laboratory

Journal of Applied Hematology, 2022
BACKGROUND: Sickle cell disease and its variants result from an abnormal hemoglobin, hemoglobin S (HbS), caused by a single point mutation in the Beta-Globin gene.
Hareem Alam, Natasha Ali
doaj   +1 more source

Editing a γ-globin repressor binding site restores fetal hemoglobin synthesis and corrects the sickle cell disease phenotype

Science Advances, 2020
Editing the fetal γ-globin promoters in hematopoietic stem cells from sickle cell disease patients induces therapeutic γ-globin levels. Sickle cell disease (SCD) is caused by a single amino acid change in the adult hemoglobin (Hb) β chain that causes Hb ...
Leslie Weber, G. Frati, T. Felix, G. Hardouin, Antonio Casini, Clara Wollenschlaeger, Vasco Meneghini, C. Masson, A. de Cian, Anne Chalumeau, F. Mavilio, Mario Amendola, I. André-Schmutz, A. Cereseto, W. El Nemer, J. Concordet, C. Giovannangeli, M. Cavazzana, A. Miccio   +18 more
semanticscholar   +1 more source