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Evidenced Based Management: A Journey for Physicians
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Historical Perspective, Epidemiology, and Methodology
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Overview of the SCD guidelines and chapters
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Process and methodology
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Consensus Statements
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Clinical Practice Guidelines and the institute of Medicine
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Prevention of invasive infection
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Screening for Renal Disease
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Electrocardiogram Screening
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Screening for hypertension
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Screening for Retinopathy
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Screening for risk of stroke using neuroimaging
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Screening for Pulmonary disease
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Reproductive counseling
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Contraception
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Clinical Preventive services
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Immunizations
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Vaso-Occlusive Crisis
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Fever
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Acute Renal Failure
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Priapism
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Hepatobiliary Complications
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Acute Anemia
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Splenic Sequestration
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Acute Chest Syndrome
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Acute Stroke
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Multisystem Organ Failure
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Acute Ocular Conditions
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Chronic pain
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Avascular Necrosis
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Leg Ulcers
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Pulmonary Hypertension
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Renal Complications
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Stuttering/Recurrent Priapism
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Ophthalmologic Complications
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Summary of the Evidence
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Hydroxurea Treatment Recommendations
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Consensus Treatment Protocol and Technical remarks for the implementation of Hydroxyurea Therapy
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Indications for transfusions
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Recommendations for Acute and Chronic Transfusion Therapy
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Appropriate Management/ Monitoring
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Consensus Protocol for Monitoring Individuals on Chronic Transfusion Therapy
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Complications of Transfusions
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Recommendations for the Management and Prevention of Transfusion Complications
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New Research is Needed
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Data Systems That Meet the Highest Standards of Scientific Rigor Can Be Invaluable
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Improved Phenotyping is needed
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Broad collaborations for Research and Care
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Beyond Efficacy
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Look, Listen, Empathize and Ask
Participants 164
Background
Young children with SCA have a very high risk for septicemia and meningitis in the absence of appropriate prophylaxis. These infections result from defective or absent splenic function that typically has its onset in people with SCA early in the first year of life. Case fatality is high, and the risk is greatest in young people who lack humoral immunity against the specific pneumococcal serotype causing infection. People with HbSC and HbSb -thalassemia have a much lower incidence of life-threatening infection because their spleen function is normal or only minimally impaired during infancy. However, older children and adults with all SCD genotypes are at increased risk for invasive bacterial infection.
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