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

20:34 EDT 25th May 2016 | BioPortfolio

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

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...

Deferasirox therapy is associated with reduced mortality risk in a medicare population with myelodysplastic syndromes.

Iron overload adversely affects patients with myelodysplastic syndromes (MDS), but benefits of iron chelation therapy have not been clearly demonstrated. We examined the association between deferasirox (DFX) therapy and mortality in transfusion-receiving Medicare patients.

Five Years of Deferasirox Therapy for Cardiac Iron in β-Thalassemia Major.

Myocardial siderosis in β-thalassemia major (β-TM) remains the leading cause of death. Deferasirox (DFX), a new iron chelation treatment, has proved to be effective in reducing or preventing cardiac iron burden in thalassemic patients according to clinical trials with maximum duration of up to 3 years except one that was recently published and lasted 5 years. The aim of this study was to evaluate the efficacy of DFX in reducing or preventing cardiac iron burden in 23 patients with β-TM after 5 years of t...

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.

Chelation efficacy and erythroid response during deferasirox treatment in patients with Myeloproliferative Neoplasms in fibrotic phase.

At present, very few data are available on deferasirox (DFX) in the treatment of patients with Philadelphia negative myeloproliferative neoplasms in fibrotic phase (FP-MPN) and transfusion dependence. To address this issue, a retrospective analysis of 28 patients (22 male, 6 female) with FP-MPN and iron overload secondary to transfusion dependence was performed, based on patients enrolled in the database of our regional cooperative group who received treatment with DFX. DFX was started after a median interv...

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...

Pathology of hepatic iron deposition in hemochromatosis.

To identify the type of iron deposition and describe its amount, distribution and associated lesions, in order to support an etiologic diagnosis for hemochromatosis.

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 ...

Iron Status and Inflammation in Early Stages of Chronic Kidney Disease.

One of the most common causes of anemia of chronic disease (ACD) is chronic kidney disease. The main pathomechanism responsible for ACD is subclinical inflammation. The key element involved in iron metabolism is hepcidin, however, studies on new indices of iron status are in progress.The aim of the study was to assess the iron status in patients in early stages of chronic kidney disease, iron correlation with inflammation parameters and novel biomarkers of iron metabolism.


chronic inflammation induced by obesity alters iron homeostasis leading to mild /moderate iron deficiency and anaemia. Between 14% and 43% of patients may suffer from iron deficiency without anaemia before surgery. The management of peri-operative iron deficiency improves patient outcome and quality of life. Under certain circumstances intravenous (IV) iron must be considered. IV iron (which may avoid iron blockage in enterocytes and macrophages) has turned out to be a safe and efficient alternative. Objeti...

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...

The role of genetic factors in patients with hepatocellular carcinoma and iron overload - a prospective series of 234 patients.

Iron overload (IO) in HFE-related hereditary hemochromatosis is associated with increased risk of liver cancer. This study aimed to investigate the role of other genes involved in hereditary IO among patients with hepatocellular carcinoma (HCC).

The role of magnetic resonance imaging-T2* in the evaluation of iron overload early in hereditary hemochromatosis. A cross-sectional study with 159 patients.

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 ...

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.

The influence of oxidative stress induced by iron on telomere length.

Oxidative stress can be induced by increased concentrations of iron in the body and consequently can cause shortening of telomeres. Telomeres, called mitotic clocks, are non-coding fragments at the end of chromosomes. During the replication of genetic material they are shortened, playing the role of ageing biomarkers in eukaryotes. In human endothelial cells, oxidative stress causes a decrease in telomerase activity. Shortening of chromosomes in telomeric parts was found in patients with primary hemochromat...

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...

Exome sequencing for molecular characterization of non-HFE hereditary hemochromatosis.

Diagnostic genetic testing for hereditary hemochromatosis is readily available for clinically relevant HFE variants (i.e., those that generate the C282Y, H63D and S65C HFE polymorphisms); however, genetic testing for other known causes of iron overload, including mutations affecting genes encoding hemojuvelin, transferrin receptor 2, HAMP, and ferroportin is not. As an alternative to conventional genetic testing we propose diagnostic use of whole exome sequencing for characterization of non-HFE hemochromato...

EDTA Chelation Therapy to Reduce Cardiovascular Events in Persons with Diabetes.

The Trial to Assess Chelation Therapy (TACT) was a randomized double-blind placebo-controlled trial enrolling patients age ≥50 years with prior myocardial infarction. TACT used a 2 × 2 factorial design to study ethylene diamine tetraacetic acid (EDTA) chelation and high-dose vitamin supplementation. Chelation provided a modest but significant reduction in cardiovascular endpoints. The benefit was stronger and significant among participants with diabetes but absent in those without diabetes. Mechanis...

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