eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | thalassemia |
| Symptom | C0282193|iron overload |
| Sentences | 79 |
| PubMedID- 26466432 | Impaired endothelial function in pediatric hemoglobin e/beta-thalassemia patients with iron overload. |
| PubMedID- 22391807 | The objective of this review is to discuss the factors which should be considered when designing clinically useful iron chelators, to present the application of iron chelators in the treatment of iron overload associated with beta-thalassaemia major and sickle cell anaemia, and to highlight the potential applications in the treatment of neurodegenerative disorders and microbial infection. |
| PubMedID- 25671931 | Comparison of deferiprone and deferrioxamine for the treatment of transfusional iron overload in children with beta thalassemia major. |
| PubMedID- 20950401 | Abnormal iron regulation in patients with thalassaemia intermedia may lead to iron overload even in the absence of transfusions. |
| PubMedID- 25922644 | Hepatic iron overload and fibrosis in patients with beta thalassemia major after hematopoietic stem cell transplantation: a pilot study. |
| PubMedID- 20631898 | Despite iron overload in patients with β-thalassemia, hepcidin levels are not increased. |
| PubMedID- 24044606 | Cardiac complications represent the leading cause of mortality in thalassemia major with uncontrolled iron overload. |
| PubMedID- 24040004 | iron overload in patients with thalassemia results from both excessive iron absorption and transfusion. |
| PubMedID- 24575259 | Background: frequent blood transfusion in patients with beta thalassemia major can lead to iron overload especially in liver. |
| PubMedID- 23963793 | Authors' conclusions: desferrioxamine is the recommended first-line therapy for iron overload in people with thalassaemia major and deferiprone or deferasirox are indicated for treating iron overload when desferrioxamine is contraindicated or inadequate. |
| PubMedID- 22035015 | Hypogonadotrophic hypogonadism and diminished gonadal reserve accounts for dysfunctional gametogenesis in thalassaemia patients with iron overload presenting with infertility. |
| PubMedID- 23019522 | Moreover, a review article that addresses the mechanism of tissue damage arising from iron overload in patients with β-thalassemia major is presented. |
| PubMedID- 24934354 | The hepatic iron overload in beta-thalassemia patients is associated with haemosiderin storage both in kupffer cells and in the cytoplasm of hepatocytes. |
| PubMedID- 22900514 | The complete treatment of transfusional iron overload in thalassaemia using the deferiprone (l1) and deferoxamine combination is a paradigm to be followed in the treatment of many other metal toxicity conditions. |
| PubMedID- 23241074 | Conclusion: we conclude that the use of dfx instead of dfo represents a cost-effective use of resources for treatment of iron overload in patients with beta-thalassemia from iran's society perspective. |
| PubMedID- 25850001 | Furthermore, a pilot trial investigating the effect of amlodipine (a ccb) on iron overload in patients with thalassemia major reported that it can serve as a complementary treatment to standard chelation regimens and may improve the efficacy of iron removal in the heart without the burden of significant side effects . |
| PubMedID- 25402221 | The effects of deferasirox on renal haemodynamics, including glomerular filtration rate (gfr) and renal plasma flow (rpf), were investigated in a phase i, open-label study in beta thalassaemia major patients with iron overload. |
| PubMedID- 23600689 | Assessment and management of iron overload in beta-thalassaemia major patients during the 21st century: a real-life experience from the italian webthal project. |
| PubMedID- 23026844 | Lifetime cost-utility analyses of deferasirox in beta-thalassaemia patients with chronic iron overload: a uk perspective. |
| PubMedID- 20662901 | Conclusion: transfusional iron overload in patients with thalassaemia could be reduced to normal body iron range levels using effective deferiprone/deferoxamine combinations. |
| PubMedID- 23966805 | Although it was being used in other countries, deferiprone received fda approval only in october 2011 for treatment of patients with transfusional iron overload due to thalassemia syndromes who were not adequately chelated with other agents. |
| PubMedID- 25252297 | Assessment of the relationship between fragmented qrs and cardiac iron overload in patients with beta-thalassemia major. |
| PubMedID- 22645668 | A study done on status of thyroid function and iron overload in patients with beta thalassemia major on deferoxamine in jordan concluded that there is significantly high (p < 0.05) levels of serum ferritin, ft3, zinc, and copper in patients with beta thalassemia major as compared to controls . |
| PubMedID- 23868464 | While dfx has been cited as a factor in a number of treatment-related deaths across several disease areas , no quantitative data were published on thalassaemia patients with transfusional iron overload, and so it was conservatively assumed that dfx was not associated with excess mortality. |
| PubMedID- 24847266 | iron overload in patients with beta-thalassemia major lead to alterations in the arterial structures and in the thickness of the carotid arteries (cheung et al., 2002; tantawy et al., 2009). |
| PubMedID- 21752315 | Curative effects and safety of deferasirox in treatment of iron overload in children with beta-thalassemia major. |
| PubMedID- 21757620 | Reproductive capacity in iron overloaded women with thalassemia major. |
| PubMedID- 23028894 | Given the well documented impaired exercise capacity of thalassaemia patients with significant iron overload , , a submaximal exercise testing protocol was adopted. |
| PubMedID- 23443782 | Objective: to assess the value of magnetic resonance imaging t2* tests in the detection of myocardial and liver iron overload in patients with beta-thalassemia major (beta-tm). |
| PubMedID- 21531154 | Combined chelation treatment may be a better approach for transfusion-dependent thalassemia major patients with iron overload complications because of increased efficacy. |
| PubMedID- 22665732 | Most of the endocrine complications in thalassaemia are attributable to iron overload which may be the result of economic circumstances (expense of the chelation therapy), late onset of chelation therapy or poor compliance with the iron chelation therapy. |
| PubMedID- 20383704 | These results imply that combining the atrial ejection force index with the transmitral-derived echocardiographic assessment is a feasible way to detect early stages of myocardial iron overload in patients with beta thalassemia major. |
| PubMedID- 24460526 | Myocardial iron overload in thalassaemia major. |
| PubMedID- 24087894 | Objective: we aimed to investigate the effect of human hemochromatosis protein (hfe) polymorphisms on cardiac iron overload in patients with beta-thalassemia major. |
| PubMedID- 20001472 | Background: pituitary iron overload in patients with transfusion-dependent beta-thalassemia may lead to delayed puberty. |
| PubMedID- 23056768 | Objective: hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. |
| PubMedID- 23830536 | Conclusions: the use of amlodipine in conjunction with standard chelation therapy may suggest a new strategy in preventing and treating iron overload in patients with thalassemia major, especially in organs where iron absorption depends on active uptake by calcium channels like the heart. |
| PubMedID- 25745550 | This comes in harmony with the results of a previous study that was carried out by cheung et al,28 who found that iron overloading in patients with beta-thalassemia major results in alterations of arterial structures with disruption of elastic tissue and calcification. |
| PubMedID- 19744128 | Elevated serum parathormone levels are associated with myocardial iron overload in patients with beta-thalassaemia major. |
| PubMedID- 23687960 | iron overload in beta-thalassaemia major patients is secondary to multiple blood transfusions and increased iron absorption. |
| PubMedID- 20027547 | Dual-echo tfe mri for the assessment of myocardial iron overload in beta-thalassemia major patients. |
| PubMedID- 22052662 | Electrocardiographic consequences of cardiac iron overload in thalassemia major. |
| PubMedID- 23056717 | Objective: dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. |
| PubMedID- 25822525 | Tnf-α and il-10 were found to be elevated in iron overloaded patients with thalassemia major . |
| PubMedID- 25161967 | Background: diabetes mellitus is a major complication of iron overload in patients with beta thalassemia major. |
| PubMedID- 23460118 | However, in the case of patient with primary myelofibrosis the magnetic anisotropy energy barrier differed from that in normal case and, probably, the iron core size was supposed to be slightly larger than that in both normal spleen tissue and normal human liver ferritin in contrast to well-known data for iron overload in patients with thalassemia accompanied by the iron-core size increase. |
| PubMedID- 23228075 | Recently it has been used to detect myocardial dysfunction related to iron overload in patients with beta-thalassemia . |
| PubMedID- 21382180 | The value of nontransferrin-bound iron (ntbi) as an index of iron overload in patients with thalassemia has been evaluated; however, data in patients with sickle cell disease (scd) is limited. |
| PubMedID- 22287873 | Cardiac toxicity includes congestive heart failure and arrhythmias and is the leading cause of death related to iron overload in patients with thalassemia major.13 excess iron deposition in the liver leads to inflammation, fibrosis, and cirrhosis,14 which may be further exacerbated by concomitant viral hepatitis, alcohol use, and other hepatotoxins. |
| PubMedID- 22066516 | Relation between nt-probnp levels, iron overload, and early stage of myocardial dysfunction in beta-thalassemia major patients. |
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