1,720,982 research outputs found
Oral iron chelators
No full textDeferoxamine (DFO) was the standard of care for transfusional iron overload for > 40 years, requiring subcutaneous infusion for 8-12 h/day, 5-7 days/week. Oral iron chelators are an important development, offering the potential to improve compliance and patients' quality of life. The oral, three-times-daily agent deferiprone appeared to be a promising advance; however, its use has been limited owing to serious adverse events, such as neutropenia and agranulocytosis. Therapy combining deferiprone with DFO has proved effective in the management of severe cardiac siderosis. Deferasirox is a novel, orally active agent that provides 24-h chelation with a once-daily dose. An extensive clinical trial program has demonstrated that deferasirox at appropriate doses is effective in reducing or maintaining iron burden in adult and pediatric patients. The clinical program demonstrated that deferasirox has a safety profile that is clinically manageable with regular monitoring. Copyrigh
Echocardiography in internal medicine: usefulness ofDoppler tissue analysis
No full textAbstrtact
The Doppler echocardiographic examination is the most important and common method used for
cardiac imaging due to its safety, its relatively low cost and the great amount of morphofunctional information
it can provide. It also plays a relevant role in the assessment of cardiac involvement underlying
systemic diseases, in most of which the complication rate is often subclinical and represents
one of the main causes of mortality. Advances in technology have lead to an increase in diagnostic accuracy
and the range of applications of echocardiography. Among the new techniques, tissue Doppler
echocardiography has been shown to identify early ventricular changes in both diastolic and systolic
phases and in the preclinical stage of systemic diseases such as amyloidosis, systemic lupus erythematosus,
vasculitis, with possible cardiac involvement. The increased sensitivity derives from the possibility
of assessing longitudinal mechanics, which seems affected earlier than the circumferential one
by pathological processes. Tissue Doppler echocardiography analysis, associated with a complete
clinical history, can make an early diagnosis or arise a diagnostic suspicion to the physician, allowing
early detection and rapid implementation of diagnostic and therapeutic measures able to improve patient
prognosis.
Progress in the management of iron overload in erytrocyte disorders
No full textAlthough blood transfusions are essential for patients with hemoglobinopathies (ie Thalassemia, Sickle Cell Disease) or with rare anemias (ie Fanconi, Blackfan Diamond, PK deficiency), chronic transfusions inevitably lead to iron overload as humans cannot actively remove excess iron. The cumulative effects of iron overload lead to significant morbidity and mortality, if untreated. Desferrioxamine (DFO) is the reference-standard iron chelator whose safety and efficacy profile has been established through many years of clinical use. DFO side effects are accettable and manageable however the prolonged subcutaneous infusion regimen of 5-7 days per week is very demanding and results in poor adherence to therapy. Deferiprone (Ferriprox, L1) is a bidentate molecule, orally administrable three-times/day, licensed in Europe and in other regions but in the USA and Canada, for the treatment of iron overload in patients for whom DFO therapy is contraindicated or inadequate. Preliminary evidences suggest that deferiprone may be more effective than DFO in chelating cardiac iron. The side effects include gastrointestinal symptoms, liver dysfunction, joint pain, neutropenia and agranulocytosis. A weekly assessment of white blood cell counts is recommended because of the risk of agranulocytosis. Deferasirox is a new, convenient, once-daily oral iron chelator that has demonstrated in various clinical trials good efficacy and acceptable safety profile in adult and pediatric patients affected by transfusion-dependent thalassemia major and by different chronic anemias (SCD, BDA, MDS). The long half-life of deferasirox (16-18 hours) provides sustained 24 hr iron chelation coverage. The efficacy and safety profile have been evaluated in more than 3000 patients in clinical trials. Patient satisfaction with deferasirox was superior than with DFO therapy
Bi-ventricular myocardial performance : a new approach to evaluate interventricular delay
No full textPerformance ventricolare sinistra in giovani normotesi con familiarità per ipertensione arteriosa
No full text
- …
