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

02:18 EDT 25th August 2016 | BioPortfolio

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Showing "Iron Chelation with Deferasirox Patients with Hemochromatosis Chronic" PubMed Articles 1–25 of 39,000+

Cardiac iron load and function in transfused patients treated with deferasirox (the MILE study).

To assess the effect of iron chelation therapy with deferasirox on cardiac iron and function in patients with transfusion-dependent thalassemia major, sickle-cell disease (SCD) and myelodysplastic syndromes (MDS).

Clinical monitoring and management of complications related to chelation therapy in patients with β-thalassemia.

Iron chelating agents - deferoxamine, deferiprone, and deferasirox - are used to treat chronic iron overload in patients with β-thalassemia in an attempt to reduce morbidity and mortality related to siderosis. Each of the approved iron chelating agents has its own advantages over the others and also has its own risks, whether related to over-chelation or not. In this review, we briefly discuss the methods to monitor the efficacy of iron chelation therapy and the evidence behind the use of each iron chelati...

Iron chelation monotherapy in transfusion-dependent beta-thalassemia major patients: a comparative study of deferasirox and deferoxamine.

Iron overload is the primary cause of mortality and morbidity in thalassemia major (TM) despite advances in chelation therapy. The aim of this study was to compare the effectiveness and safety of deferasirox (DFX) and deferoxamine (DFO) as iron-chelating agents in patients with transfusion-dependent β-thalassemia major.

Iron Chelation in Thalassemia Major.

Iron chelation has improved survival and quality of life of patients with thalassemia major. there are currently 3 commercially available iron-chelating drugs with different pharmacokinetic and pharmacodynamic activity. The choice of adequate chelation treatment should be tailored to patient needs and based on up-to-date scientific evidence.

Iron overload-related heart failure in a patient with transfusion-dependent myelodysplastic syndrome reversed by intensive combined chelation therapy.

Patients with transfusion-dependent myelodysplastic syndromes (MDS) have an increased risk of cardiac events, due to both chronic anemia and iron overload. Here, we report the recovery of cardiac function after an intensive iron chelation therapy in a MDS patient who had developed heart failure due to iron overload.

Severe Myocardial Dysfunction Reversed by Iron-Chelation Therapy in an Asian Patient with Hereditary Hemochromatosis.

A theoretical study on the electronic structures and equilibrium constants evaluation of Deferasirox iron complexes.

Elemental iron is essential for cellular growth and homeostasis but it is potentially toxic to the cells and tissues. Excess iron can contribute in tumor initiation and tumor growth. Obviously, in iron overload issues using an iron chelator in order to reduce iron concentration seems to be vital. This study presents the density functional theory calculations of the electronic structure and equilibrium constant for iron-deferasirox (Fe-DFX) complexes in the gas phase, water and DMSO. A comprehensive study wa...

CONIFER - Non-Interventional Study to Evaluate Therapy Monitoring During Deferasirox Treatment of Iron Toxicity in Myelodysplastic Syndrome Patients with Transfusional Iron Overload.

The non-interventional study CONIFER was designed to assess the safety and clinical practicability of deferasirox for the treatment of transfusional iron overload in myelodysplastic syndrome (MDS) patients.

Analysis of polymorphism and hepatic expression of duodenal cytochrome b in chronic hepatitis C.

Pathological iron overload is commonly found in chronic hepatitis C (CHC) patients and considered as a negative prognostic factor of the disease. A single nucleotide polymorphism (SNP) rs884409 in duodenal cytochrome b gene (CYBRD1) is implicated in pathogenesis of hemochromatosis. In our study we investigated the impact of CYBRD1 genotype and expression on iron overload in CHC patients.

Deferasirox at therapeutic doses is associated with dose-dependent hypercalciuria.

Deferasirox is an oral iron chelator used widely in the treatment of thalassemia major and other transfusion-dependent hemoglobinopathies. Whilst initial long-term studies established the renal safety of deferasirox, there are now increasing reports of hypercalciuria and renal tubular dysfunction. In addition, urolithiasis with rapid loss of bone density in patients with β thalassemia major has been reported. We conducted a cross-sectional cohort study enrolling 152 adult patients comprising of β thalasse...

Clinical Management of Patients With Thalassemia Syndromes.

Thalassemia is a chronic inherited blood disorder that reduces hemoglobin production, causing chronic hemolytic anemia. Patients often are diagnosed via newborn screening programs. Patients diagnosed with the most severe form of thalassemia often require chronic red blood cell transfusions to control their anemia. The side effect of chronic transfusions is cumulative iron overload for which chelation therapy is required. The incidence of thalassemia is low; therefore, care is best delivered at specialized t...

Adherence to iron chelation therapy and associated healthcare resource utilization and costs in Medicaid patients with sickle cell disease and thalassemia.

Sub-optimal patient adherence to iron chelation therapy (ICT) may impact patient outcomes and increase cost of care. This study evaluated the economic burden of ICT non-adherence in patients with sickle cell disease (SCD) or thalassemia.

Use of deferasirox, an iron chelator, to overcome imatinib resistance of chronic myeloid leukemia cells.

The treatment of chronic myeloid leukemia (CML) has achieved impressive success since the development of the Bcr-Abl tyrosine kinase inhibitor, imatinib mesylate. Nevertheless, resistance to imatinib has been observed, and a substantial number of patients need alternative treatment strategies.

Organ iron accumulation in chronically transfused children with sickle cell anaemia: baseline results from the TWiTCH trial.

Transcranial Doppler (TCD) With Transfusions Changing to Hydroxyurea (TWiTCH) trial is a randomized, open-label comparison of hydroxycarbamide (also termed hydroxyurea) versus continued chronic transfusion therapy for primary stroke prevention in patients with sickle cell anaemia (SCA) and abnormal TCD. Severity and location of iron overload is an important secondary outcome measure. We report the baseline findings of abdominal organ iron burden in 121 participants. At enrollment, patients were young (9·8...

Morbidity and mortality of adult patients with congenital dyserythropoietic anemia type I.

Congenital dyserythropoietic anemia type I (CDAI) is a rare autosomal recessive disease characterized by macrocytic anemia, ineffective erythropoiesis and secondary hemochromatosis. To better define the natural history of the disease among adult patients we studied 32 Bedouin patients (median age 34 years; range 21-60) all carrying the same CDAN1 founder mutation. Follow up studies included complete blood count, blood chemistry, abdominal ultrasound, echocardiography and T2*MRI. Main complications were due ...

Evaluation of Proteinuria in β-Thalassemia Major Patients With and Without Diabetes Mellitus Taking Deferasirox.

β-thalassemia is the most common heredity disease in Iran. Regular blood transfusion is critical to sustain life and normal growth. Deferasirox is an oral chelator. One of the side effects of the deferasirox is proteinuria.

Iron-induced epigenetic abnormalities of mouse bone marrow through aberrant activation of aconitase and isocitrate dehydrogenase.

Iron overload remains a concern in myelodysplastic syndrome (MDS) patients. Iron chelation therapy (ICT) thus plays an integral role in the management of these patients. Moreover, ICT has been shown to prolong leukemia-free survival in MDS patients; however, the mechanisms responsible for this effect are unclear. Iron is a key molecule for regulating cytosolic aconitase 1 (ACO1). Additionally, the mutation of isocitrate dehydrogenase (IDH), the enzyme downstream of ACO1 in the TCA cycle, is associated with ...

Iron Chelation Inhibits Osteoclastic Differentiation In Vitro and in Tg2576 Mouse Model of Alzheimer's Disease.

Patients of Alzheimer's disease (AD) frequently have lower bone mineral density and higher rate of hip fracture. Tg2576, a well characterized AD animal model that ubiquitously express Swedish mutant amyloid precursor protein (APPswe), displays not only AD-relevant neuropathology, but also age-dependent bone deficits. However, the underlying mechanisms remain poorly understood. As APP is implicated as a regulator of iron export, and the metal chelation is considered as a potential therapeutic strategy for AD...

Diagnosis of Iron-Deficiency Anemia in Chronic Kidney Disease.

Anemia is a common and clinically important consequence of chronic kidney disease (CKD). It is most commonly a result of decreased erythropoietin production by the kidneys and/or iron deficiency. Deciding on the appropriate treatment for anemia associated with CKD with iron replacement and erythropoietic-stimulating agents requires an ability to accurately diagnose iron-deficiency anemia. However, the diagnosis of iron-deficiency anemia in CKD patients is complicated by the relatively poor predictive abilit...

Characterisation of hepatic and cardiac iron deposition during standard treatment of anaemia in haemodialysis.

Parenteral iron is integral in the treatment of anaemia of chronic kidney disease (CKD) patients on haemodialysis (HD). However, increased liver iron concentration (LIC) can result from such treatment and this correlates poorly with serum ferritin or transferrin saturation values. It is unclear whether increased cardiac iron concentration also occurs in this setting. We aimed to evaluate the relationship of intravenous iron supplementation to hepatic and cardiac iron deposition in chronic HD subjects.

Dual T-type and L-type calcium channel blocker exerts beneficial effects in attenuating cardiovascular dysfunction in iron-overload thalassemic mice.

Iron chelation therapy is a standard treatment in thalassemia patients. However, its poor cardioprotective efficacy and serious side effects are a cause for concern. Previous studies have shown that treatment with L-type calcium channel (LTCC) or dual T-type calcium channel (TTCC) and LTCC blockers decreased cardiac iron and improved cardiac dysfunction in iron overloaded rodent model. Currently, the head to head comparison of therapeutic efficacy among commercial iron chelators, dual TTCC<CC blocker and ...

Longitudinal MRI and Ferritin Monitoring of Iron Overload in Chronically Transfused and Chelated Children With Sickle Cell Anemia and Thalassemia Major.

Iron overload is an ineluctable complication in chronically transfused children warranting accurate assessment to avoid related morbidity. We investigated longitudinally the relationships between ferritin levels and hepatic and cardiac T2* magnetic resonance imaging (MRI) in a cohort of chronically transfused children receiving chelation therapy. Thirty children with sickle cell anemia (SCA) and 7 with thalassemia major (TM) chelated similarly by deferasirox were analyzed. Sex ratio, age, median duration of...

Iron Balance and the Role of Hepcidin in Chronic Kidney Disease.

The hepatic iron-regulatory hormone hepcidin and its receptor, the cellular iron exporter ferroportin, constitute a feedback-regulated mechanism that maintains adequate plasma concentrations of iron-transferrin for erythropoiesis and other functions, ensures sufficient iron stores, and avoids iron toxicity and iron-dependent microbial pathogenesis. In chronic kidney disease, inflammation and impaired renal clearance increase plasma hepcidin, inhibiting duodenal iron absorption and sequestering iron in macro...

Iron Treatment Strategies in Nondialysis CKD.

In contrast to managing patients on hemodialysis in whom iron strategies are more focused on intravenous iron, nondialysis chronic kidney disease (CKD) patients may receive either oral or intravenous iron. There are advantages and disadvantages for both strategies. Oral iron is simple and cheap to administer and does not require hospital visits, but is poorly absorbed in advanced CKD and is associated with unpleasant gastrointestinal side effects. Intravenous iron, on the other hand, guarantees iron bioavai...

Is iron important in heart failure?

Iron deficiency is a frequent comorbidity in a patient with chronic heart failure, and it associates with a worse pro-gnosis of that patient. Mainly worse quality of life and more rehospitalizations are in these iron deficient patients. Iron metabolism is rather complex and there is some new information concerning this complexity in heart failure. We distinquish an absolute and a functional iron deficiency in heart failure. It is this deficit which is important and not as much is anemia important here. Prev...

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