Assessment of cerebral blood flow with magnetic resonance imaging in children with sickle cell disease: A quantitative comparison with transcranial Doppler ultrasonography

Croal, Paula; Leung, Jackie; Kosiński, Przemysław; Shroff, Manohar; Odame, Isaac; Kassner, Andrea

doi:10.1002/brb3.811

Cited by 11 publications

(7 citation statements)

References 48 publications

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“…This difference in CVR can be explained by chronically increased resting vessel diameter, as we have shown previously, 21 which leaves these patients with little reserve for further vasodilation. Numerous resting ASL studies in children with SCD have shown that chronic anemia leads to high CBF 20,29–32 and the high flow requirements in SCD could lead to a loss of autoregulatory capacity if dilatory reserve is being used for perfusion. Our dynamic CBF response supports our hypothesis that adult patients with SCD have severely reduced vasodilatory capacity.…”

Section: Discussionmentioning

confidence: 99%

Hemodynamic provocation with acetazolamide shows impaired cerebrovascular reserve in adults with sickle cell disease

et al. 2018

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Sickle cell disease is characterized by chronic hemolytic anemia and vascular inflammation, which can diminish the vasodilatory capacity of the small resistance arteries, making them less adept at regulating cerebral blood flow. Autoregulation maintains adequate oxygen delivery, but when vasodilation is maximized, the low arterial oxygen content can lead to ischemia and silent cerebral infarcts. We used magnetic resonance imaging of cerebral blood flow to quantify whole-brain cerebrovascular reserve in 36 adult patients with sickle cell disease (mean age, 31.9±11.3 years) and 11 healthy controls (mean age, 37.4±15.4 years), and we used high-resolution 3D FLAIR magnetic resonance imaging to determine the prevalence of silent cerebral infarcts. Cerebrovascular reserve was calculated as the percentage change in cerebral blood flow after a hemodynamic challenge with acetazolamide. Co-registered lesion maps were used to demonstrate prevalent locations for silent cerebral infarcts. Cerebral blood flow was elevated in patients with sickle cell disease compared to controls (median [interquartile range]: 82.8 [20.1] vs . 51.3 [4.8] mL/100g/min, P <0.001). Cerebral blood flow was inversely associated with age, hemoglobin, and fetal hemoglobin, and correlated positively with bilirubin, and LDH, indicating that cerebral blood flow may reflect surrogates of hemolytic rate. Cerebrovascular reserve in sickle cell disease was decreased by half compared to controls (34.1 [33.4] vs . 69.5 [32.4] %, P <0.001) and was associated with hemoglobin and erythrocyte count indicating anemia-induced hemodynamic adaptations. In total, 29/36 patients (81%) and 5/11 controls (45%) had silent cerebral infarcts (median volume of 0.34 vs . 0.02 mL, P =0.03). Lesions were preferentially located in the borderzone. In conclusion, patients with sickle cell disease have a globally reduced cerebrovascular reserve as determined by arterial spin labeling with acetazolamide and reflects anemia–induced impaired vascular function in sickle cell disease. This study was registered at clinicaltrials.gov identifier 02824406 .

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Section: Discussionmentioning

confidence: 99%

Hemodynamic provocation with acetazolamide shows impaired cerebrovascular reserve in adults with sickle cell disease

et al. 2018

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“…CBF and TCD do not directly correlate without conversion of the TCD velocity to CBF units, based on arterial size and estimation of tissue weight supplied by the artery, both of which require MRI measurements. 57 TCDs are portable and non-invasive, but limitations include high operator-dependence, inability to detect velocities in all patients, particularly those with poor bone windows, a low specificity for stroke 56 and they do not predict "silent" ischemia. TCD does not have direct correlation with underlying vascular abnormalities, but stenosis may influence TCD values by either raising or significantly lowering velocities.…”

Section: Cerebral Blood Flowmentioning

confidence: 99%

Advances in Understanding Ischemic Stroke Physiology and the Impact of Vasculopathy in Children With Sickle Cell Disease

Guilliams

Fields

Dowling

2019

Stroke

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“…This finding suggests that there are aspects of SCA physiology aside from decreased arterial oxygen content driving the compensatory increase in OEF. Prior literature has established that CBF increases as patients become more anemic, not only in SCA 7,8,14,[55][56][57][58][59] but also in healthy controls 22 and disease states other than SCA with concomitant anemia. 8,20,21,[24][25][26]56 Oxygen delivery normalizes in SCA in the setting of increased CBF, 8 suggesting there is increased demand driving the elevation in OEF in the participants with SCA compared to AC.…”

Section: Statistical Analysesmentioning

confidence: 99%

Cerebral oxygen metabolic stress is increased in children with sickle cell anemia compared to anemic controls

et al. 2022

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Patients with sickle cell anemia (SCA) experience cerebral metabolic stress with an increase in oxygen extraction fraction (OEF) to compensate for reduced oxygen carrying capacity due to anemia. It remains unclear if anemia alone drives this metabolic stress. Using MRI, we collected voxel-wise OEF measurements to test our hypothesis that OEF would be elevated in anemic controls without SCA (AC) compared to healthy controls (HC), but OEF would be even higher in SCA compared to AC. Brain MRIs (N = 159) were obtained in 120 participants (34 HC, 27 AC, 59 SCA). While

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Assessment of cerebral blood flow with magnetic resonance imaging in children with sickle cell disease: A quantitative comparison with transcranial Doppler ultrasonography

Abstract: Introduction: Transcranial Doppler ultrasonography (TCD) is a clinical tool for stratify-

Cited by 11 publications

References 48 publications

Hemodynamic provocation with acetazolamide shows impaired cerebrovascular reserve in adults with sickle cell disease

Hemodynamic provocation with acetazolamide shows impaired cerebrovascular reserve in adults with sickle cell disease

Advances in Understanding Ischemic Stroke Physiology and the Impact of Vasculopathy in Children With Sickle Cell Disease

Cerebral oxygen metabolic stress is increased in children with sickle cell anemia compared to anemic controls

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