Sickle cell disease (SCD) is characterized by chronic hemolysis, frequent infections, and recurrent occlusions of microcirculation, which cause painful crises and result in chronic organ damage and failure. Occlusions of the microcirculation and infections are important factors that stimulate the production of cytokines and acute-phase proteins. Cytokines seem to be involved with several possible mechanisms in the pathogenesis of vasoocclusive phenomena in SCD: vascular endothelial activation, induction of red-cell adhesiveness to vascular endothelium, induction of neutrophil adhesiveness to endothelium, development of vascular intimal hyperplasia, platelet activation, endothelin-1 production, and dysregulation of endothelial apoptosis. Cytokines are also thought to be involved in the regulation of hemopoiesis, the inhibition of immune functions, and the development of growth deficits. Investigation of cytokines in SCD patients will elucidate the pathogenesis of the disease and its complications and may help in assessing disease severity and prognosis.
This study demonstrated a high prevalence of anti-HEV in a separate hemodialysis unit, without an association with the known routes of transmission of blood-borne viruses. This observation suggests that a still-undefined intra-unit factor or other factors are associated with HEV transmission.
Population screening and prenatal diagnosis are significantly facilitated by these data. Furthermore, the detailed distribution tables of beta-thalassemia mutations are essential for counseling and extraction of genetic diversity estimates for population genetic studies in other inherited disorders.
Four patients (1 male, 3 female, age range 16–56 yr) with β‐thalassemia intermedia were given high doses of recombinant human erythropoietin (rHuEpo), iron sulfate and folic acid in an attempt to improve their anemia. The dose schedule was: rHuEpo, 500 U/kg 3 times weekly, iron sulfate, 300 mg/d and folic acid, 5 mg/d. All patients were red blood cell transfusion‐dependent. Hematological data and fetal hemoglobin (HbF) were assayed every 2 wk. XmnI polymorphism and β‐thalassemia mutations were identified by PCR. All patients showed a moderate to high increase in hemoglobin values (mean value: 2.5 g/dl) and in 1 patient HbF levels also increased; 3 patients became red blood cell transfusion‐independent and 1 patient was able to extend the intervals between transfusions significantly. No side effects were observed during rHuEpo therapy.
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