Management of sickle cell disease: a review for physician education in Nigeria (sub‐saharan Africa)

AS Adewoyin - Anemia, 2015 - Wiley Online Library
Sickle cell disease (SCD) predominates in sub‐Saharan Africa, East Mediterranean areas,
Middle East, and India. Nigeria, being the most populous black nation in the world, bears its …

Thrombosis and sickle cell disease

L De Franceschi, MD Cappellini… - Seminars in thrombosis …, 2011 - thieme-connect.com
Sickle cell disease (SCD) is characterized by the presence of sickle hemoglobin, which has
the unique property of polymerizing when deoxygenated. The pathophysiology of acute and …

[HTML][HTML] Pharmacological induction of fetal hemoglobin in β-thalassemia and sickle cell disease: An updated perspective

R Bou-Fakhredin, L De Franceschi, I Motta… - Pharmaceuticals, 2022 - mdpi.com
A significant amount of attention has recently been devoted to the mechanisms involved in
hemoglobin (Hb) switching, as it has previously been established that the induction of fetal …

[HTML][HTML] New therapeutic options for the treatment of sickle cell disease

A Matte, F Zorzi, F Mazzi, E Federti… - … journal of hematology …, 2019 - ncbi.nlm.nih.gov
Sickle cell disease (SCD; ORPHA232; OMIM# 603903) is a chronic and invalidating
disorder distributed worldwide, with high morbidity and mortality. Given the disease …

[HTML][HTML] Evidence of protective effects of recombinant ADAMTS13 in a humanized model of sickle cell disease

P Rossato, E Federti, H Glantschnig, F Canneva… - …, 2022 - ncbi.nlm.nih.gov
Sickle cell disease (SCD) is an inherited red blood cell disorder that occurs worldwide.
Acute vaso-occlusive crisis is the main cause of hospitalization in patients with SCD. There …

[HTML][HTML] Factor H interferes with the adhesion of sickle red cells to vascular endothelium: a novel disease-modulating molecule

E Lombardi, A Matte, AM Risitano, D Ricklin… - …, 2019 - ncbi.nlm.nih.gov
Sickle cell disease is an autosomal recessive genetic red cell disorder with a worldwide
distribution. Growing evidence suggests a possible involvement of complement activation in …

[HTML][HTML] The genetics of blood disorders: hereditary hemoglobinopathies

MF Sonati, FF Costa - Jornal de Pediatria, 2008 - SciELO Brasil
Objective: To summarize recently published data on the pathophysiology, diagnosis and
treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary …

Hypoxia-reperfusion affects osteogenic lineage and promotes sickle cell bone disease

LD Carbonare, A Matte', MT Valenti… - Blood, The Journal …, 2015 - ashpublications.org
Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder,
characterized by severe organ complication. Sickle bone disease (SBD) affects a large part …

Erythroid adhesion molecules in sickle cell disease: effect of hydroxyurea

JP Cartron, J Elion - Transfusion clinique et biologique, 2008 - Elsevier
In sickle cell disease, the complex scenario of vaso-occlusive crisis (VOC) typical of this
disease is clearly multifactorial and not fully understood. Cell-cell and cell-cell matrix …

[HTML][HTML] Pathophisiology of sickle cell disease and new drugs for the treatment

L De Franceschi - … Journal of Hematology and Infectious Diseases, 2009 - ncbi.nlm.nih.gov
A homozygous mutation in the gene for β globin, a subunit of adult hemoglobin A (HbA), is
the proximate cause of sickle cell disease (SCD). Sickle hemoglobin (HbS) shows peculiar …