M R DeBaun1, L C Jordan2, A A King3, J Schatz4, E Vichinsky5, C K Fox6,7, R C McKinstry8,9, P Telfer10, M A Kraut11, L Daraz12, F J Kirkham13,14,15, M H Murad12. 1. Department of Pediatrics, Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease, and. 2. Division of Pediatric Neurology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN. 3. Program in Occupational Therapy, Division of Hematology, Department of Pediatrics/Division of Hematology, Department of Medicine, Washington University School of Medicine, St. Louis, MO. 4. Department of Psychology, University of South Carolina, Columbia, SC. 5. Children's Hospital Oakland Research Institute, Oakland, CA. 6. Department of Neurology and. 7. Department of Pediatrics, University of California San Francisco, San Francisco, CA. 8. Department of Radiology and. 9. Department of Pediatrics, Washington University School of Medicine, St. Louis, MO. 10. Centre for Genomics and Child Health, Blizard Institute, Queen Mary University of London, London, United Kingdom. 11. Department of Radiology, School of Medicine, Johns Hopkins University, Baltimore, MD. 12. Evidence-Based Practice Center, Mayo Clinic, Rochester, MN. 13. Developmental Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London, United Kingdom. 14. Clinical and Experimental Sciences, University of Southampton, Southampton, United Kingdom; and. 15. Department of Child Health, University Hospital Southampton, Southampton, United Kingdom.
Abstract
BACKGROUND: Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan. OBJECTIVE: These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, diagnosis, and treatment of the most common neurological morbidities in SCD. METHODS: The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic evidence reviews. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations. RESULTS: The panel placed a higher value on maintaining cognitive function than on being alive with significantly less than baseline cognitive function. The panel developed 19 recommendations with evidence-based strategies to prevent, diagnose, and treat CNS complications of SCD in low-middle- and high-income settings. CONCLUSIONS: Three of 19 recommendations immediately impact clinical care. These recommendations include: use of transcranial Doppler ultrasound screening and hydroxyurea for primary stroke prevention in children with hemoglobin SS (HbSS) and hemoglobin Sβ0 (HbSβ0) thalassemia living in low-middle-income settings; surveillance for developmental delay, cognitive impairments, and neurodevelopmental disorders in children; and use of magnetic resonance imaging of the brain without sedation to detect silent cerebral infarcts at least once in early-school-age children and once in adults with HbSS or HbSβ0 thalassemia. Individuals with SCD, their family members, and clinicians should become aware of and implement these recommendations to reduce the burden of CNS complications in children and adults with SCD.
BACKGROUND: Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan. OBJECTIVE: These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, diagnosis, and treatment of the most common neurological morbidities in SCD. METHODS: The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic evidence reviews. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations. RESULTS: The panel placed a higher value on maintaining cognitive function than on being alive with significantly less than baseline cognitive function. The panel developed 19 recommendations with evidence-based strategies to prevent, diagnose, and treat CNS complications of SCD in low-middle- and high-income settings. CONCLUSIONS: Three of 19 recommendations immediately impact clinical care. These recommendations include: use of transcranial Doppler ultrasound screening and hydroxyurea for primary stroke prevention in children with hemoglobin SS (HbSS) and hemoglobin Sβ0 (HbSβ0) thalassemia living in low-middle-income settings; surveillance for developmental delay, cognitive impairments, and neurodevelopmental disorders in children; and use of magnetic resonance imaging of the brain without sedation to detect silent cerebral infarcts at least once in early-school-age children and once in adults with HbSS or HbSβ0 thalassemia. Individuals with SCD, their family members, and clinicians should become aware of and implement these recommendations to reduce the burden of CNS complications in children and adults with SCD.
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