Sujit S Sheth

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Diamond Blackfan anaemia (DBA) is a rare, genetically and clinically heterogeneous, inherited red cell aplasia. Classical DBA affects about seven per million live births and presents during the first year of life. However, as mutated genes have been discovered in DBA, non-classical cases with less distinct phenotypes are being described in adults as well as(More)
Although red blood cell (RBC) transfusions can be lifesaving, they are not without risk. In critically ill patients, RBC transfusions are associated with increased morbidity and mortality, which may increase with prolonged RBC storage before transfusion. The mechanisms responsible remain unknown. We hypothesized that acute clearance of a subset of damaged,(More)
Transfusions of RBCs stored for longer durations are associated with adverse effects in hospitalized patients. We prospectively studied 14 healthy human volunteers who donated standard leuko-reduced, double RBC units. One unit was autologously transfused "fresh" (3-7 days of storage), and the other "older" unit was transfused after 40 to 42 days of storage.(More)
A new MRI method is proposed for separately quantifying the two principal forms of tissue storage (nonheme) iron: ferritin iron, a dispersed, soluble fraction that can be rapidly mobilized, and hemosiderin iron, an aggregated, insoluble fraction that serves as a long-term reserve. The method utilizes multiple spin echo sequences, exploiting the fact that(More)
The degree of fetal hemoglobin (HbF) expression is a major determinant of phenotypic severity of sickle cell disease (SCD). Genetic regulation of HbF production is complex and can vary among ethnic groups. The pediatric sickle cell population at our institution is approximately half Hispanic, nearly all from the Dominican Republic. Hydroxyurea (HU) is the(More)
In healthy adults, transfusion of older stored red blood cells (RBCs) produces extravascular hemolysis and circulating non-transferrin-bound iron. In a prospective, observational study of critically ill children, we examined the effect of RBC storage duration on the extent of hemolysis by comparing laboratory measurements obtained before, and 4 hr after,(More)
Deferoxamine chelation therapy (widely used to reduce iron overload in hypertransfused thalassemic patients) has been implicated in causing skeletal growth abnormalities (rachitic-like changes in the long bones and vertebral body flattening), particularly when used in early infancy and at high dose levels. Radiographs of seven hypertransfused and(More)
Pulmonary disease, including thromboembolic problems, accounts for a large portion of the morbidity of sickle cell disease. Chronic transfusion therapy is now a part of long-term treatment of sickle cell patients with stroke and chest syndrome. The resultant iron overload must be treated with chelation therapy using deferoxamine. Poor compliance with(More)
Measurement of proton transverse relaxation rates (R(2)) is a generally useful means for quantitative characterization of pathological changes in tissue with a variety of clinical applications. The most widely used R(2) measurement method is the Carr-Purcell-Meiboom-Gill (CPMG) pulse sequence but its relatively long scan time requires respiratory gating for(More)
OBJECTIVE Pediatric oncology patients who undergo intensive chemotherapy develop anemia caused by myeloid suppression that necessitates transfusions that, in turn, cause iron deposition in the reticuloendothelial system. We describe MR imaging of iron overload in pediatric patients who underwent such chemotherapy and who have solid and hematologic tumors.(More)