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PURPOSE To determine normative R2* values in the liver and heart at 3T, and establish the relationship between R2* at 3T and 1.5T over a range of tissue iron concentrations. MATERIALS AND METHODS A total of 20 healthy control subjects and 14 transfusion-dependent patients were scanned at 1.5T and 3T. At each field strength R2* imaging was performed in the(More)
BACKGROUND Effective ways to prevent arthropathy in severe hemophilia are unknown. METHODS We randomly assigned young boys with severe hemophilia A to regular infusions of recombinant factor VIII (prophylaxis) or to an enhanced episodic infusion schedule of at least three doses totaling a minimum of 80 IU of factor VIII per kilogram of body weight at the(More)
Carriers of a single sickle cell gene mutation generally enjoy normal lifespans without serious health consequences related to their sickle cell status, but under extreme conditions such as severe dehydration and high-intensity physical activity, complications such as exertional rhabdomyolysis, splenic infarction, and papillary necrosis can occur. Recently,(More)
BACKGROUND Silent cerebral infarcts are the most common neurologic injury in children with sickle cell anemia and are associated with the recurrence of an infarct (stroke or silent cerebral infarct). We tested the hypothesis that the incidence of the recurrence of an infarct would be lower among children who underwent regular blood-transfusion therapy than(More)
Mutations in genes encoding proteins that are involved in mitochondrial heme synthesis, iron-sulfur cluster biogenesis, and mitochondrial protein synthesis have previously been implicated in the pathogenesis of the congenital sideroblastic anemias (CSAs). We recently described a syndromic form of CSA associated with B-cell immunodeficiency, periodic fevers,(More)
Thalassemia is an inherited blood disorder that requires lifelong adherence to a complicated and burdensome medical regimen which could potentially impact emotional functioning of patients. The importance of understanding and promoting healthy emotional functioning is crucial not only to psychological well-being, but also to physical health as it has been(More)
Transfusional iron overload leads to gonadal failure and low bone mass in patients with thalassemia (Thal). However, gonadal failure is rarely reported in transfused patients with sickle cell disease (SCD) and the literature regarding fracture prevalence in SCD is limited. The objective of this study was to assess self-reported fracture prevalence and its(More)
Massive blood transfusion is defined as the loss of one or more circulating blood volumes. It is, therefore, important to calculate the patient’s estimated blood volume (EBV) and to relate this to the volume of blood products and other fluids administered (Table 1). In addition the anesthesiologist should estimate how much blood will be allowed to be lost(More)
Little is known about the prevalence of metabolic bone disease among adults with mental retardation (now known as learning disability), although they may be at increased risk of fractures. Broadband-ultrasound attenuation (BUA) and velocity-of-sound (VOS) measurements were performed on the left heel of 170 patients in a large hospital for adults with mental(More)