The role of iron chelation therapy in myelodysplastic syndrome (MDS) remains controversial. Averting cardiac dysfunction in low-grade MDS patients who have sufficient longevity to experience deleterious cardiac effects of iron overload has been the major argument in favor of iron chelation. Although there is significant evidence showing the adverse impact of transfusion dependency on survival i...

SYNOPSIs The differential ferrioxamine test is a simple method for the measurement of chelation of body iron by desferrioxamine. A single six-hour specimen of urine is obtained after intravenous Desferal, accompanied by 59Fe-ferrioxamine. Two values are measured: Fd, the excretion of ferrioxamine derived from body iron by chelation, and Fex, the proportion of ferrioxamine excreted from a known ...

The term hemochromatosis encompasses at least four types of genetic iron overload conditions, most of them recently distinguished from one another as a result of the identification of a series of genes related to iron metabolism. At least three of these entities (HFE hemochromatosis, juvenile hemochromatosis and transferrin receptor 2 hemochromatosis) involve systemic hepcidin deficiency as a k...

BACKGROUND Thalassemia is a hereditary disorder requiring regular blood transfusion. PURPOSE To determine hearing sensitivity in transfusion-dependent thalassemics who were receiving desferioximine and on oral iron chelation (desferioximine) therapy. METHODS 26 patients with B-thelassemia in the age range of 5-22 years were enrolled in the thalassemia transfusion unit in the Advanced Pediat...

Significant mechanical stability is an essential feature shared by many elastomeric proteins, which function as molecular springs in a wide variety of biological machinery and biomaterials of superb mechanical properties. Despite the progress in understanding molecular determinants of mechanical stability, it remains challenging to rationally enhance the mechanical stability of proteins. Using ...

The phenomena of displacement and immobilization of calcium ions by chelation is being studied in an increasing number of clinical states. Chelation therapy in hypercalcmmia (Holland et al., 1953; Spencer et al., 1956), calcium deposition in coronary arteries (Meltzer et al., 1960) and peripheral vessels (Clarke et al., 1960) and in skin (Muller et al., 1959), and in the treatment of digitalis ...

Chronic red blood cell transfusion can prevent many of the manifestations of sickle cell disease. The medical costs of chronic transfusion and management of associated side effects, especially iron overload, are considerable. This study was undertaken to evaluate the financial impact of chronic transfusion for stroke prevention in patients with sickle cell anemia. Outpatient charges pertaining ...