PubMed Journals Articles About "Iron Chelation With Deferasirox Patients With Hemochromatosis Chronic" RSS

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Showing "Iron Chelation with Deferasirox Patients with Hemochromatosis Chronic" PubMed Articles 1–25 of 42,000+

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Longitudinal Study on Liver Functions in Patients with Thalassemia Major before and after Deferasirox (DFX) Therapy.

By performing regular blood transfusion and iron chelation therapy, most patients with beta thalassemia major (BTM) now survive beyond the third decade of life. Liver disease is becoming an important cause of morbidity and mortality in these patients. Chronic hepatitis and/or severe iron overload are both important causes of liver pathology. Iron chelation with desferrioxamine (DFO) reduces excessive body iron, but its efficacy is limited by poor compliance and dose related toxicity. The recent use of Defer...

Brazilian Thalassemia Association protocol for iron chelation therapy in patients under regular transfusion.

In the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. Without iron chelation therapy, heart disease is the major cause of death; however, hepatic and endocrine complications also occur. Currently there are three iron chelating agents available for continuous use in patients with thalassemia on regular transfusions (desferrioxamine, deferiprone, and deferasirox) providing good results in reducing cardiac, ...

Efficacy and safety of combined oral iron chelation therapy with deferasirox and deferiprone in a patient with beta-thalassemia major and persistent iron overload.

Safety of deferasirox: a retrospective cohort study on the risks of gastrointestinal, liver and renal events.

Deferasirox (DFX) is an effective and well-tolerated oral iron chelator elevating the adherence to iron chelating therapy among patients with iron overload. However, the US Food and Drug Administration issued a warning about the potential adverse events associated with DFX in 2010.

Deferasirox for managing transfusional iron overload in people with sickle cell disease.

Sickle cell disease (SCD) is a group of genetic haemoglobin disorders, that occurs in about 2.2 per 1000 births worldwide. Increasingly, some people with SCD develop secondary iron overload due to occasional red blood cell transfusions or are on long-term transfusion programmes for e.g. secondary stroke prevention. Iron chelation therapy can prevent long-term complications.Deferoxamine and deferiprone have been found to be efficacious. However, questions exist about the effectiveness and safety of the newer...

Iron chelation and multiple sclerosis.

Histochemical and MRI studies have demonstrated that multiple sclerosis (MS) patients have abnormal deposition of iron in both gray and white matter structures. Data is emerging indicating that this iron could partake in pathogenesis by various mechanisms, e.g., promoting the production of reactive oxygen species and enhancing the production of proinflammatory cytokines. Iron chelation therapy could be a viable strategy to block iron-related pathological events or it can confer cellular protection by stabil...

Iron and multiple sclerosis.

Iron is essential for normal cellular functioning of the central nervous system. Abnormalities in iron metabolism may lead to neuronal death and abnormal iron deposition in the brain. Several studies have suggested a link between brain iron deposition in normal aging and chronic neurologic diseases, including multiple sclerosis (MS). In MS, it is still not clear whether iron deposition is an epiphenomenon or a mediator of disease processes. In this review, the role of iron in the pathophysiology of MS will ...

Phase IV open-label study of the efficacy and safety of deferasirox after allogeneic stem cell transplantation.

This is the first prospective study of deferasirox in adult allogeneic hematopoietic stem cell transplant recipients with transfusional iron overload in hematologic malignancies. Patients at least 6 months post-transplant were treated with deferasirox at a starting dose of 10 mg/kg/day for 52 weeks or until serum ferritin was ≤400 ng/mL on two consecutive determinations. Thirty patients were enrolled and 22 patients completed the study. A significant reduction from baseline in median serum ferritin and in...

Cardiac iron overload in sickle-cell disease.

Chronically transfused sickle cell disease (SCD) patients have lower risk of myocardial iron overload (MIO) than comparably transfused thalassemia major patients. However, cardioprotection is incomplete. We present the clinical characteristics of 6 patients who have prospectively developed MIO, to identify potential risk factors for cardiac iron accumulation. From 2002 to 2011, cardiac, hepatic, and pancreatic iron overload were assessed by R2 and R2* Magnetic Resonance Imaging (MRI) techniques in 201 chron...

Diabetes and Hemochromatosis.

The common form of hereditary hemochromatosis is an autosomal recessive disorder most prevalent in Caucasians that results in excessive iron storage. The clinical manifestations of hemochromatosis are protean. HFE genotype, which determines the degree of iron overload and duration of disease have profound effects on disease expression. The prevalence of diabetes in this population has likely been underestimated because of studies that include a broad range of ethnicities and associating diabetes with allele...

Molecular basis of HFE-hemochromatosis.

Iron-overload disorders owing to genetic misregulation of iron acquisition are referred to as hereditary hemochromatosis (HH). The most prevalent genetic iron overload disorder in Caucasians is caused by mutations in the HFE gene, an atypical MHC class I molecule. Recent studies classified HFE/Hfe-HH as a liver disease with the primarily failure in the production of the liver iron hormone hepcidin in hepatocytes. Inadequate hepcidin expression signals for excessive iron absorption from the diet and iron dep...

Cardiac T2* MRI assessment in patients with thalassaemia major and its effect on the preference of chelation therapy.

The aim of the study is to assess the relationship between T2* magnetic resonance imaging (MRI) values and age, serum ferritin level, left ventricular ejection fraction (LVEF), splenectomy status, and to identify appropriate modifications to chelation therapy based on T2* MRI results of children with thalassaemia major. Sixty-four patients with thalassaemia major (37 girls/27 boys) older than 8 years of age were enrolled in the study. Based on the first T2* MRI, the patients' myocardial iron depositions we...

Hypoparathyroidism and subclinical hypothyroidism with secondary hemochromatosis.

Hemochromatosis is an inherited genetic disorder of iron metabolism which can also occur as a secondary result of iron-overload. It leads to organ damage such as cardiomyopathy, liver cirrhosis, hypogonadism, and diabetes. This paper discusses a case of secondary hemochromatosis associated with repeated transfusions, presenting as asymptomatic hypoparathyroidism and subclinical hypothyroidism with multiple organ involvement. The 29-year-old female, who had severe aplastic anemia, received multiple transfusi...

MRI Guided Iron Assessment and Oral Chelator Use Improve Iron Status in Thalassemia Major Patients.

Background: Oral iron chelators and MRI assessment of heart and liver iron burden have become widely available since the mid 2000s, allowing for improved patient compliance with chelation and noninvasive monitoring of iron levels for titration of therapy. We evaluated the impact of these changes in our center for patients with thalassemia major and transfusional iron overload. Methods: This single center, retrospective observational study covered the period from 2005 through 2012. Liver iron content (LIC) w...

Iron Overload in Beta Thalassaemia Major and Intermedia Patients.

Background: In beta thalassaemia major multiple blood transfusions, ineffective erythropoiesis and increased gastrointestinal iron absorption lead to iron overload in the body. Iron overload impairs the immune system, placing patients at greater risk of infection and illness. Iron overload can be determined by serum ferritin measurement.Objective: The aim of the present study is to assess the serum ferritin levels in multi-transfused Thalassaemia major and Thalassaemia intermedia patients. The study was als...

Update on intravenous iron choices.

Iron deficiency is a major factor in the prevalence and severity of anemia in patients with chronic kidney disease (CKD). We review the pathophysiology impairing normal intestinal iron absorption in CKD and compare the characteristics of newer intravenous (i.v.) iron agents to the longstanding i.v. iron products in the market.

Iron-Catalyzed, Chelation-Induced Remote C-H Allylation of Quinolines via 8-Amido Assistance.

An iron-catalyzed, 8-amido-enabled regiodivergent C-H allylation of quinolines is described. This reaction represents a rare example of chelation-induced geometrically inaccessible C-H functionalization, allowing for the highly regio- and stereoselective preparation of either the C5- or the C4-allylated quinoline scaffolds regiocontrolled by the catalytic systems.

Iron Chelation for Iron Overload Secondary to Transfusions of Packed Red Blood Cells.

Iron distribution and histopathological characterization of the liver and heart of β-thalassemic mice with parenteral iron overload: Effects of deferoxamine and deferiprone.

The liver and heart are the major target organs for iron accumulation and iron toxicity in β-thalassemia. To mimic the phenomenon of heavy iron overload resulting from repeated blood transfusions, a total of 180mg of iron dextran was intraperitoneally injected into C57BL/6J mice (WT) and heterozygous β-globin knockout mice ((mu)β(th-3/+), BKO). The effects of deferiprone and deferoxamine in this model were investigated. The iron was distributed homogenously throughout the 4 liver lobes (left, caudate, ri...

Predicting C282Y homozygote genotype for hemochromatosis using serum ferritin and transferrin saturation values from 44,809 participants of the HEIRS Study.

The simultaneous interpretation of serum ferritin level and transferrin saturation has been used as a clinical guide to diagnose genetic hemochromatosis. The Hemochromatosis and Iron Overload Screening (HEIRS) Study screened 101,168 North American participants for serum ferritin level and transferrin saturation, and C282Y genotyping for the HFE gene.

Effects of Iron Depletion on CALM-AF10 Leukemias.

Iron, an essential nutrient for cellular growth and proliferation, enters cells via clathrin-mediated endocytosis (CME). The clathrin assembly lymphoid myeloid (CALM) protein plays an essential role in the cellular import of iron by CME. CALM-AF10 leukemias harbor a single copy of the normal CALM gene, and may therefore be more sensitive to the growth inhibitory effect of iron restriction compared with normal hematopoietic cells. We found that Calm heterozygous (Calm(HET)) murine fibroblasts exhibit signs o...

Coinfection with HIV-1 Alleviates Iron Accumulation in Patients with Chronic Hepatitis C Virus Infection.

Most chronically-infected hepatitis C virus (HCV) patients have increased levels of iron in the liver. Iron overload reduces sustained responses to antiviral therapy, leading to more rapid progression to liver cirrhosis and the development of hepatocellular carcinoma. However, it is still unclear how HIV-1 infection affects iron status in patients chronically infected with HCV. The present study recruited 227 patients from a village in central China. These patients were either monoinfected with HCV (n =...

Laboratory Investigation of Platelet Function in Patients with Thalassaemia.

The aim of this study was to investigate platelet function in patients with thalassaemia and to detect any relation to chelation treatment (deferasirox or deferiprone/deferiprone plus desferioxamine). Thirty-three transfusion-dependent patients with thalassaemia were included. The investigation consisted of aggregation testing of platelet-rich plasma by light transmission aggregometry (LTA) with the use of 5 agonists as well as the global test of haemostasis by means of the PFA-100 platelet function analyse...

High iron accumulation in hair and nail of people living in iron affected areas of Assam, India.

Human populace of Assam, India repeatedly exposed to high concentration of iron in groundwater results in adverse health effects like hemochromatosis, liver cirrhosis and siderosis. In the present study, human hair and nail analysis were carried out to establish a possible relationship between iron toxicity and its deposition among the residents. Nail and hair iron concentrations ranged from 28.2 to 1046μgg(-1) (n=114) and 26.5-838 (n=108)μgg(-1) levels, respectively, among all the study participants. The...

Deletion of Hemojuvelin, an Iron-regulatory Protein, in Mice Results in Abnormal Angiogenesis and Vasculogenesis in Retina along with Reactive Gliosis.

Loss-of-function mutations in hemojuvelin (HJV) cause juvenile hemochromatosis, an iron-overload disease. Deletion of Hjv in mice results in excessive iron accumulation and morphological changes in the retina. Here we studied the retinal vasculature in Hjv-/- mice.


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