Intravascular hemolysis and the pathophysiology of sickle cell disease

GJ Kato, MH Steinberg… - The Journal of clinical …, 2017 - Am Soc Clin Investig
Hemolysis is a fundamental feature of sickle cell anemia that contributes to its
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …

Sickle cell disease: renal manifestations and mechanisms

KA Nath, RP Hebbel - Nature Reviews Nephrology, 2015 - nature.com
Sickle cell disease (SCD) substantially alters renal structure and function, and causes
various renal syndromes and diseases. Such diverse renal outcomes reflect the uniquely …

Therapeutic strategies for sickle cell disease: towards a multi-agent approach

MJ Telen, P Malik, GM Vercellotti - Nature reviews Drug discovery, 2019 - nature.com
For over 100 years, clinicians and scientists have been unravelling the consequences of the
A to T substitution in the β-globin gene that produces haemoglobin S, which leads to the …

Circulating cell membrane microparticles transfer heme to endothelial cells and trigger vasoocclusions in sickle cell disease

SM Camus, JA De Moraes, P Bonnin… - Blood, The Journal …, 2015 - ashpublications.org
Intravascular hemolysis describes the relocalization of heme and hemoglobin (Hb) from
erythrocytes to plasma. We investigated the concept that erythrocyte membrane …

Pulmonary complications of sickle cell disease

MT Gladwin, E Vichinsky - New England journal of medicine, 2008 - Mass Medical Soc
This review presents evidence for two overlapping yet distinctive clinical types of sickle cell
disease. The basis of one is the vaso-occlusive crisis; the other is the consequence of …

TGF-β activity protects against inflammatory aortic aneurysm progression and complications in angiotensin II–infused mice

Y Wang, H Ait-Oufella, O Herbin… - The Journal of …, 2010 - Am Soc Clin Investig
Complicated abdominal aortic aneurysm (AAA) is a major cause of mortality in elderly men.
Ang II–dependent TGF-β activity promotes aortic aneurysm progression in experimental …

[HTML][HTML] Sickle hemoglobin confers tolerance to Plasmodium infection

A Ferreira, I Marguti, I Bechmann, V Jeney, Â Chora… - Cell, 2011 - cell.com
Sickle human hemoglobin (Hb) confers a survival advantage to individuals living in endemic
areas of malaria, the disease caused by Plasmodium infection. As demonstrated hereby …

Acute chest syndrome in children with sickle cell disease

S Jain, N Bakshi, L Krishnamurti - Pediatric allergy, immunology …, 2017 - liebertpub.com
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle
cell disease (SCD). Patients may present with ACS or may develop this complication during …

Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management

SK Ballas, MR Kesen, MF Goldberg… - The Scientific World …, 2012 - Wiley Online Library
The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG→ GTG) in
exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position …

Endothelin: 30 years from discovery to therapy

M Barton, M Yanagisawa - Hypertension, 2019 - Am Heart Assoc
Discovered in 1987 as a potent endothelial cell–derived vasoconstrictor peptide, endothelin-
1 (ET-1), the predominant member of the endothelin peptide family, is now recognized as a …