Iron chelation therapy

A Bruzzese, EA Martino, F Mendicino… - European Journal of …, 2023 - Wiley Online Library
Iron overload is a pathological condition resulting from a congenital impairment of its
regulation, increased intestinal iron absorption secondary to bone marrow erythroid …

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 …

Systematic literature review of the burden of disease and treatment for transfusion-dependent β-thalassemia

M Betts, PA Flight, LC Paramore, L Tian… - Clinical Therapeutics, 2020 - Elsevier
Purpose β-Thalassemia is an inherited blood disorder characterized by reduced or no
production of adult hemoglobin. Systematic identification of the burden of β-thalassemia with …

The pathophysiology of transfusional iron overload

JB Porter, M Garbowski - Hematology/oncology clinics, 2014 - hemonc.theclinics.com
Iron homeostatic mechanisms are key to the pathophysiology of transfusional iron overload
(TIO). In humans, these mechanisms are best adapted to increasing iron acquisition in …

Consequences and management of iron overload in sickle cell disease

J Porter, M Garbowski - Hematology 2013, the American …, 2013 - ashpublications.org
The aims of this review are to highlight the mechanisms and consequences of iron
distribution that are most relevant to transfused sickle cell disease (SCD) patients and to …

Mass spectrometry imaging of metals in tissues and cells: Methods and biological applications

BL Gorman, SV Torti, FM Torti, CR Anderton - Biochimica et Biophysica …, 2024 - Elsevier
Background Metals are pervasive throughout biological processes, where they play
essential structural and catalytic roles. Metals can also exhibit deleterious effects on human …

Regulation of iron homeostasis and related diseases

Y Li, X Huang, J Wang, R Huang… - Mediators of …, 2020 - Wiley Online Library
The liver is the organ for iron storage and regulation; it senses circulating iron
concentrations in the body through the BMP‐SMAD pathway and regulates the iron intake …

The liver in sickle cell disease

E Theocharidou, AR Suddle - Clinics in liver disease, 2019 - liver.theclinics.com
Sickle cell disease (SCD) or homozygous sickle cell anemia is the most common inherited
disorder of erythrocytes, characterized by the presence of pathologic hemoglobin S (HbS). A …

Hepatic manifestations in hematological disorders

J Murakami, Y Shimizu - International journal of hepatology, 2013 - Wiley Online Library
Liver involvement is often observed in several hematological disorders, resulting in
abnormal liver function tests, abnormalities in liver imaging studies, or clinical symptoms …

Sickle hepatopathy

DL Praharaj, AC Anand - Journal of Clinical and Experimental Hepatology, 2021 - Elsevier
Sickle hepatopathy is an umbrella term describing various pattern of liver injury seen in
patients with sickle cell disease. The disease is not uncommon in India; in terms of …