Prospective Brain Imaging Evaluation of Children with Sickle Cell Trait: Initial Observations

Steen, R. Grant; Hankins, Gisele M.; Xu, Xiaoping; Wang, Winfred C.; Beil, Kenneth; Langston, J. William; Helton, Kathleen J.

doi:10.1148/radiol.2281020600

Cited by 29 publications

(19 citation statements)

References 47 publications

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“…Thirdly, vessel tortuosity at the labeling plane adds an additional path length and angle component to labeling efficiency measurements that was not accounted for in this study. Anemic patients, especially those with sickle cell disease are well known to have tortuous vessels(13) and this fact may partially explain the larger variance observed in the anemic patients in this study. Fourthly, it remains possible that venous outflow is present in other patients but had yet to reach the sagittal sinus at the time of image acquisition.…”

Section: Discussionmentioning

confidence: 77%

“…Anemic subjects have elevated CBF(6), high blood velocities(7), shorter vascular transit times(8) and longer blood relaxation times(9,10). In sickle cell disease (SCD), quantification is complicated further by abnormal blood properties (shorter T1 for a given hematocrit(11)) and cerebrovascular disease (stroke(12,13), vessel tortuosity(14)). As a result, historically there are widely variable reports of ASL CBF values in SCD(8,15–20).…”

Section: Introductionmentioning

confidence: 99%

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Pseudo continuous arterial spin labeling quantification in anemic subjects with hyperemic cerebral blood flow

Bush

Chai

Choi

et al. 2018

Magnetic Resonance Imaging

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Purpose To investigate possible sources of quantification errors in global cerebral blood flow (CBF) measurements by comparing pseudo continuous arterial spin labeling (PCASL) and phase contrast (PC) MRI in anemic, hyperemic subjects. Methods All studies were performed on a Philips 3T Achieva MRI scanner. PC and PCASL CBF examinations were performed in 10 healthy, young adult subjects and 18 young adults with chronic anemia syndromes including sickle cell disease and thalassemia. CBF estimates from single and two compartment ASL kinetic models were compared. Numerical simulation and flow phantom experiments were used to explore the effects of blood velocity and B1+ on CBF quantification and labeling efficiency. Results PCASL CBF underestimated PC in both populations using a single compartment model (30.1±9.2% control, 45.2±17.2% anemia). Agreement substantially improved using a two-compartment model (−8.0 ± 6.0% control, 11.7 ± 12.3% anemia). Four of the anemic subjects exhibited venous outflow of ASL signal, suggestive of cerebrovascular shunt, possibly confounding PC-PCASL comparisons. Additionally, sub-study experiments demonstrated that B1+ was diminished at the labeling plane (82.9 ± 5.1%), resulting in suboptimal labeling efficiency. Correcting labeling efficiency for diminished B1+, PCASL slightly overestimated PC CBF in controls (−15.4 ± 6.8%) and resulted in better matching of CBF estimates in anemic subjects (0.7 ± 10.0% without outflow, 10.5 ± 9.4% with outflow). Conclusions This work demonstrates that a two-compartment model is critical for PCASL quantification in hyperemic subjects. Venous outflow and B1+ under-excitation may also contribute to flow underestimation, but further study of these effects is required.

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Section: Discussionmentioning

confidence: 77%

Section: Introductionmentioning

confidence: 99%

Pseudo continuous arterial spin labeling quantification in anemic subjects with hyperemic cerebral blood flow

Bush

Chai

Choi

et al. 2018

Magnetic Resonance Imaging

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“…Interestingly, in a study of 21 pediatric patients with sickle cell trait, two were classified as having mild abnormalities on MRI and four were identified as having vascular tortuosity. When compared with a population of patients without sickle cell trait, the subjects with sickle cell trait were significantly more likely to have arterial tortuosity and it was correlated with hemoglobin S percent at the time of the evaluation [167]. This was considered mild vasculopathy and has not been replicated.…”

Section: Sickle Cell Traitmentioning

confidence: 99%

Stroke in patients with sickle cell disease

Webb

Kwiatkowski

2013

Expert Review of Hematology

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“…Children with heterozygous sickle cell trait were found to have parenchymal abnormalities on MRI in 10% and vasculopathy manifesting as tortuosity in 19%. 125 The authors of this handbook have seen vasculopathy producing moyamoya syndrome in some patients with sickle cell trait. One study showed sickle cell trait associated with a 15-fold lower risk of ischaemic stroke but 10-fold higher risk of haemorrhagic stroke compared to those without the gene.…”

Section: Sickle Cell Traitmentioning

confidence: 99%

Acute Ischaemic Stroke Agnieszka Anna Ardelt

Harrigan

Deveikis

Ardelt

2012

Handbook of Cerebrovascular Disease and Neurointerventional Technique

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This chapter focuses on acute ischaemic stroke: mechanisms, risk factors, clinical presentation, diagnostic evaluation, and treatment other than thrombolysis and thrombectomy, which are discussed in Chap. 9 . The topics are arranged alphabetically. Ischaemic stroke in paediatric patients is discussed separately in an appendix. Acute Ischaemic Stroke: Burden of DiseaseIn the United States, approximately 795,000 people suffer a stroke annually, and the vast majority of strokes are ischaemic in nature. Over 130,000 people with stroke die each year; only cardiac disease, cancer, and chronic lung diseases result in more deaths than stroke.1 Stroke leaves survivors with disabilities which have both personal and societal implications: 20-30% will need assistance with activities of daily living or walking, and the majority will be unable to return to work.

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Prospective Brain Imaging Evaluation of Children with Sickle Cell Trait: Initial Observations

Abstract: Findings suggest that greater percentage of hemoglobin S is associated with mild vasculopathy. This vasculopathy may explain some of the excess risk of stroke among African Americans.

Cited by 29 publications

References 47 publications

Pseudo continuous arterial spin labeling quantification in anemic subjects with hyperemic cerebral blood flow

Pseudo continuous arterial spin labeling quantification in anemic subjects with hyperemic cerebral blood flow

Stroke in patients with sickle cell disease

Acute Ischaemic Stroke Agnieszka Anna Ardelt

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