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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Sickle cell disease (SCD)
Definitions
- Abnormality in hemoglobin (Hgb) → change in shape (sickling) → increased "stickiness" of erythrocytes (RBCs) → capillary occlusions, ischemia, infarctions, premature RBC destruction (hemolytic anemia)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Issar P et al: Conventional and advanced brain MR imaging in patients with sickle cell anemia. Indian J Radiol Imaging. 28(3):305-11, 2018
- Miao X et al: Increased brain iron deposition in patients with sickle cell disease: an MRI quantitative susceptibility mapping study. Blood. 132(15):1618-21, 2018
- Green NS et al: Enhanced long-term brain magnetic resonance imaging evaluation of children with sickle cell disease after hematopoietic cell transplantation. Biol Blood Marrow Transplant. 23(4):670-6, 2017
- Kawadler JM et al: Brain atrophy in paediatric sickle cell anaemia: findings from the silent infarct transfusion (SIT) trial. Br J Haematol. 177(1):151-3, 2017
- Andreotti C et al: The association of cytokine levels with cognitive function in children with sickle cell disease and normal MRI studies of the brain. J Child Neurol. 30(10):1349-53, 2015
- Chen R et al: A prospective longitudinal brain morphometry study of children with sickle cell disease. AJNR Am J Neuroradiol. 36(2):403-10, 2014
- Helton KJ et al: Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 124(6):891-8, 2014
- Mackin RS et al: Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 82(10):835-41, 2014
- Thust SC et al: Neuroimaging findings in sickle cell disease. Br J Radiol. 87(1040):20130699, 2014
- Arkuszewski M et al: Sickle cell anemia: reference values of cerebral blood flow determined by continuous arterial spin labeling MRI. Neuroradiol J. 26(2):191-200, 2013
- Behpour AM et al: Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Pediatr Neurol. 48(3):188-99, 2013
- Kawadler JM et al: Subcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia. Br J Haematol. 163(3):373-6, 2013
- DeBaun MR et al: Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 119(20):4587-96, 2012
- Thangarajh M et al: Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia. Br J Haematol. 159(3):352-9, 2012
- Helton KJ et al: Arterial spin-labeled perfusion combined with segmentation techniques to evaluate cerebral blood flow in white and gray matter of children with sickle cell anemia. Pediatr Blood Cancer. 52(1):85-91, 2009
- Khademian Z et al: Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer. 52(3):373-5, 2009
- Dick MC: Standards for the management of sickle cell disease in children. Arch Dis Child Educ Pract Ed. 93(6):169-76, 2008
- Gold JI et al: Detection and assessment of stroke in patients with sickle cell disease: neuropsychological functioning and magnetic resonance imaging. Pediatr Hematol Oncol. 25(5):409-21, 2008
- Ozsahin H: MRI abnormalities in infants with sickle cell anemia-indication for preemptive therapy? Pediatr Blood Cancer. 51(5):573-4, 2008
- Al-Kandari FA et al: Regional cerebral blood flow in patients with sickle cell disease: study with single photon emission computed tomography. Ann Nucl Med. 21(8):439-45, 2007
- Kral MC et al: Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics. 112(2):324-31, 2003
- Kwiatkowski JL et al: Transcranial Doppler ultrasonography in siblings with sickle cell disease. Br J Haematol. 121(6):932-7, 2003
- Oguz KK et al: Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. Radiology. 227(2):567-74, 2003
- Riebel T et al: Transcranial Doppler ultrasonography in neurologically asymptomatic children and young adults with sickle cell disease. Eur Radiol. 13(3):563-70, 2003
- Steen RG et al: Brain imaging findings in pediatric patients with sickle cell disease. Radiology. 228(1):216-25, 2003
- Steen RG et al: Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. AJNR Am J Neuroradiol. 24(3):382-9, 2003
- Steen RG et al: Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology. 228(1):208-15, 2003
- Oyesiku NM et al: Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75(3):356-63, 1991
- Wiznitzer M et al: Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. J Pediatr. 117(4):551-5, 1990
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Sickle cell disease (SCD)
Definitions
- Abnormality in hemoglobin (Hgb) → change in shape (sickling) → increased "stickiness" of erythrocytes (RBCs) → capillary occlusions, ischemia, infarctions, premature RBC destruction (hemolytic anemia)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Issar P et al: Conventional and advanced brain MR imaging in patients with sickle cell anemia. Indian J Radiol Imaging. 28(3):305-11, 2018
- Miao X et al: Increased brain iron deposition in patients with sickle cell disease: an MRI quantitative susceptibility mapping study. Blood. 132(15):1618-21, 2018
- Green NS et al: Enhanced long-term brain magnetic resonance imaging evaluation of children with sickle cell disease after hematopoietic cell transplantation. Biol Blood Marrow Transplant. 23(4):670-6, 2017
- Kawadler JM et al: Brain atrophy in paediatric sickle cell anaemia: findings from the silent infarct transfusion (SIT) trial. Br J Haematol. 177(1):151-3, 2017
- Andreotti C et al: The association of cytokine levels with cognitive function in children with sickle cell disease and normal MRI studies of the brain. J Child Neurol. 30(10):1349-53, 2015
- Chen R et al: A prospective longitudinal brain morphometry study of children with sickle cell disease. AJNR Am J Neuroradiol. 36(2):403-10, 2014
- Helton KJ et al: Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood. 124(6):891-8, 2014
- Mackin RS et al: Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 82(10):835-41, 2014
- Thust SC et al: Neuroimaging findings in sickle cell disease. Br J Radiol. 87(1040):20130699, 2014
- Arkuszewski M et al: Sickle cell anemia: reference values of cerebral blood flow determined by continuous arterial spin labeling MRI. Neuroradiol J. 26(2):191-200, 2013
- Behpour AM et al: Cerebral blood flow abnormalities in children with sickle cell disease: a systematic review. Pediatr Neurol. 48(3):188-99, 2013
- Kawadler JM et al: Subcortical and cerebellar volumetric deficits in paediatric sickle cell anaemia. Br J Haematol. 163(3):373-6, 2013
- DeBaun MR et al: Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 119(20):4587-96, 2012
- Thangarajh M et al: Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia. Br J Haematol. 159(3):352-9, 2012
- Helton KJ et al: Arterial spin-labeled perfusion combined with segmentation techniques to evaluate cerebral blood flow in white and gray matter of children with sickle cell anemia. Pediatr Blood Cancer. 52(1):85-91, 2009
- Khademian Z et al: Reversible posterior leuko-encephalopathy in children with sickle cell disease. Pediatr Blood Cancer. 52(3):373-5, 2009
- Dick MC: Standards for the management of sickle cell disease in children. Arch Dis Child Educ Pract Ed. 93(6):169-76, 2008
- Gold JI et al: Detection and assessment of stroke in patients with sickle cell disease: neuropsychological functioning and magnetic resonance imaging. Pediatr Hematol Oncol. 25(5):409-21, 2008
- Ozsahin H: MRI abnormalities in infants with sickle cell anemia-indication for preemptive therapy? Pediatr Blood Cancer. 51(5):573-4, 2008
- Al-Kandari FA et al: Regional cerebral blood flow in patients with sickle cell disease: study with single photon emission computed tomography. Ann Nucl Med. 21(8):439-45, 2007
- Kral MC et al: Transcranial Doppler ultrasonography and neurocognitive functioning in children with sickle cell disease. Pediatrics. 112(2):324-31, 2003
- Kwiatkowski JL et al: Transcranial Doppler ultrasonography in siblings with sickle cell disease. Br J Haematol. 121(6):932-7, 2003
- Oguz KK et al: Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. Radiology. 227(2):567-74, 2003
- Riebel T et al: Transcranial Doppler ultrasonography in neurologically asymptomatic children and young adults with sickle cell disease. Eur Radiol. 13(3):563-70, 2003
- Steen RG et al: Brain imaging findings in pediatric patients with sickle cell disease. Radiology. 228(1):216-25, 2003
- Steen RG et al: Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. AJNR Am J Neuroradiol. 24(3):382-9, 2003
- Steen RG et al: Prospective brain imaging evaluation of children with sickle cell trait: initial observations. Radiology. 228(1):208-15, 2003
- Oyesiku NM et al: Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75(3):356-63, 1991
- Wiznitzer M et al: Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. J Pediatr. 117(4):551-5, 1990
