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Fifteen false-negative serologic results were associated with alleles encoding weak antigens or single-dose Fyb expression.

Many studies report estimated pulmonary artery systolic pressure (ePASP) in patients with sickle cell disease (SCD) screened by echocardiography.

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We conclude that these cytogenetic abnormalities are not linked to the natural history of the disease, but rather that they might be induced by the cytoreductive treatment.

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Although hydroxyurea is beneficial to some patients with sickle cell disease, several issues about use of the drug are unresolved.

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In a phase II study from the same group (), fetal hemoglobin was directly associated with the most recent plasma level of hydroxyurea and baseline fetal hemoglobin percentage and leukocyte count.

Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation.

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Among its functions are the disintegration of erythrocytes and the setting free of hemoglobin, which the liver converts into bilirubin; the genesis of new erythrocytes during fetal life and in the newborn; serving as a blood reservoir; and production of lymphocytes and plasma cells.

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Before early screening for sickle cell disease and the use of preventive antibiotics in children, 35% of infants with sickle cell died from infections.

The second section discusses the epidemiology of the disease (prevalence, morbidity, and mortality), at global level and within Africa.

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The role of this approach in the context of concomitant chemoradiotherapy is currently being investigated in several large, multicenter, randomized trials.

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National Heart, Lung and Blood Institute to establish a basic and translational research center for sickle cell disease that will consolidate research, treatment and care of adult and pediatric patients under one roof and speed up the translation of scientific discovery from bench to bedside.

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Deletions of variable extent of the beta gene or of the HBB cluster that result in beta-thalassemia or in the complex beta-thalassemias, called gammadeltabeta-thalassemia and deltabeta-thalassemia, are rare causes of beta-thalassemia and testing that deletions is available clinically by using MPLA.

The drug inhibits DNA repair processes, which is why the drug is also used as a chemotherapy agent in various cancers.

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The NHS Sickle Cell and Thalassaemia Screening Programme has invested in a where laboratory personnel can participate in online training which tests them on the basic heterozygous conditions which must be detected by the antenatal screening programme.

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Follow-up with the trial participants, including patients who were originally given placebo and were later prescribed hydroxyurea after the drug was determined to be beneficial, has shown that hydroxyurea reduces the damaging effects of sickle cell disease and improves some aspects of quality of life.

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Since hydroxyurea may raise the serum uric acid level, dosage adjustment of uricosuric medication may be necessary.

Using a murine model of sickle cell disease in which fetal hemoglobin induction does not occur, we evaluated whether hydroxyurea administration would still yield improvements in hematologic parameters and reduce end-organ damage.

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If both parents have the sickle cell trait, the chance that a child will have sickle cell disease is 25%.

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Montalembert M, Brousse V, Elie C, Bernaudin F, Shi J, Landais P; French Study Group on Sickle Cell Disease.

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At age three years, the dose is increased to 250 mg orally, twice a day, and then continued until at least age five years.

Other studies of nurses who handle CD's and of treated patients have yielded variable results, which could be due to confounding by smoking, PPE, and glutathione-S-transferase activity.

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This occurs because the sickle shaped cells prevent blood flow out of these extremities.

Seventy-four children with SCA, their parent, and members of their health care team completed the GCQ.

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Controlled trial of hydroxyurea vs placebo in 145 patients with HIV infection on didanosine and stavudine found no excess toxicity with addition of hydroxyurea, no mention of ALT elevations, hepatitis, liver failure or drug fever).

Deformities of the hip in adults who have sickle-cell disease and had avascular necrosis in childhood: a natural history of fifty-two patients.

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The National Institutes of Health funds sickle cell research centers, and several states currently support sickle cell specialty clinics.

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In sickle cell, the drug boosts fetal hemoglobin production, which prevents red blood cells from acquiring the sickle shape that causes organ damage and frequent bouts of pain.

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In children with SCD, measure blood pressure, and evaluate and treat HTN following recommendations from the NHLBI's "Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.

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