Cerebral and muscle microvascular oxygenation in children with sickle cell disease: Influence of hematology, hemorheology and vasomotion

Charlot, Keyne; Antoine‐Jonville, Sophie; Moeckesch, Berenike; Jumet, Stéphane; Romana, Marc; Waltz, Xavier; Divialle‐Doumdo, Lydia; Hardy‐Dessources, Marie‐Dominique; Petras, Marie; Tressières, Benoît; Tarer, Vanessa; Hue, Olivier; Etienne‐Julan, Maryse; Connes, Philippe

doi:10.1016/j.bcmd.2017.03.015

Cited by 25 publications

(22 citation statements)

References 44 publications

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“…Some data implicate mechanical stress and RBC deformation as stimuli for local NO . Our observation that RBC deformability is a powerful predictor of %rSO 2 is consistent with the findings of Charlot and colleagues but cannot distinguish among these possible mechanisms.…”

Section: Discussionsupporting

confidence: 84%

“…There is an acute on chronic vascular disease phenomenon occurring in patients with SCD, and the chronic vasculopathy is becoming more apparent as survival improves. All levels of the vasculature exhibit functional changes in patients with sickle cell disease, including conduit arteries, arterial microcirculation, venous microcirculation and the capillary bed leading to multiple organ dysfunction. Small vessel damage causes silent strokes in the brain, pulmonary hypertension in the lungs, and focal glomerular sclerosis in the kidney .…”

Section: Discussionmentioning

confidence: 99%

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Sickle cell microvascular paradox—oxygen supply‐demand mismatch

et al. 2019

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We have previously demonstrated that sickle cell disease (SCD) patients maintain normal global systemic and cerebral oxygen delivery by increasing cardiac output. However, ischemic end‐organ injury remains common suggesting that tissue oxygen delivery may be impaired by microvascular dysregulation or damage. To test this hypothesis, we performed fingertip laser Doppler flowmetry measurements at the base of the nailbed and regional oxygen saturation (rSO2) on the dorsal surface of the same hand. This was done during flow mediated dilation (FMD) studies in 26 chronically transfused SCD, 75 non‐transfused SCD, and 18 control subjects. Chronically transfused SCD patients were studied prior to and following a single transfusion and there was no acute change in rSO2 or perfusion. Laser Doppler estimates of resting perfusion were 76% higher in non‐transfused and 110% higher in transfused SCD patients, compared to control subjects. In contrast, rSO2 was 12 saturation points lower in non‐transfused SCD patients, but normal in the transfused SCD patients. During cuff occlusion, rSO2 declined at the same rate in all subjects suggesting similar intrinsic oxygen consumption rates. Upon cuff release, laser doppler post occlusive hyperemia was blunted in SCD patients in proportion to their resting perfusion values. Transfusion therapy did not improve the hyperemia response. FMD was impaired in SCD subjects but partially ameliorated in transfused SCD subjects. Taken together, non‐transfused SCD subjects demonstrate impaired conduit artery FMD, impaired microcirculatory post‐occlusive hyperemia, and resting hypoxia in the hand despite compensated oxygen delivery, suggesting impaired oxygen supply‐demand matching. Transfusion improves FMD and oxygen supply‐demand matching but not microcirculation hyperemic response.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Sickle cell microvascular paradox—oxygen supply‐demand mismatch

et al. 2019

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“…Hb < 12 g/dL and ferritin < 15 ng/mL were considered IDA, whereas vitamin B12 < 200 pg/mL was considered B12DA. 5 The right eye of 35 females with IDA was assigned to group 1, and the right eye of 35 females with combined IDA-B12DA was assigned to group 2. The right eye of 35 healthy females was assigned to group 3 (control group).…”

Section: Methodsmentioning

confidence: 99%

The Evaluation of Ophthalmic Findings in Women Patients With Iron and Vitamin B12 Deficiency Anemia

Coşkun

Sevencan

2018

Trans. Vis. Sci. Tech.

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PurposeTo determine whether there are differences in the central corneal thickness (CCT), retinal nerve fiber layer (RNFL), and ganglion cell-inner plexiform layer (GC-IPL) in women with iron deficiency anemia (IDA) and vitamin B12 deficiency anemia (B12DA).MethodsThe patients were divided and evaluated in three groups as IDA (group 1), combined IDA-B12DA (group 2), and healthy females (group 3). Each group constituted 35 subjects. CCT, endothelial cell density (CD), the coefficient of variation (CV), and hexagonality (HEX) values were evaluated. Superior, nasal, inferior, and temporal retinal nerve fiber analysis were done, and mean thickness of GC-IPL complex was also evaluated.ResultsCCT was found to be less (thin CCT) in group 2 as compared to group 1 and group 3 (P = 0.016 and P = 0.013, respectively). While CD measurements did not vary significantly between the groups, CV value was found to be lower in the control group as compared to that in the other groups (P = 0.001 and P = 0.002, respectively). HEX and the thicknesses of all RNFL and GC-IPL were lower in both the anemic groups as compared to the control group (P = 0.012 and P = 0.013, respectively); however, no significant difference was determined between the anemia groups.ConclusionsIt should be noted that anemia is a common clinical picture particularly seen in women, and there may be a tendency toward corneal and retinal disorders in these patients. The present study found that presence in the patients with IDA and with combined IDA-B12DA may have influenced endothelial functions; also the patients with anemia had significantly thinner CCT than healthy controls.Translational RelevanceOptical coherence tomography and specular microscopy measurements may provide important data for early diagnosis and treatment of both corneal and retinal diseases according to the type of anemia in anemic women.

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“…The vessels supplying the cerebral parenchyma exhibit the most curious behavior termed vasomotion, involving the regular and rhythmic alternating contraction and relaxation of the vascular smooth muscle cells, which may play a critical role in modulating the local cerebral blood flow, [171][172][173][174][175][176][177][178][179][180][181] evidenced by alterations of these mechanisms in various pathophysiologic states. These oscillations manifest in the spectra of the cerebral blood flow, demonstrating frequency bands with peaks correlated to the central tendencies of respirophasic Traube-Hering-Meyer, arterial pressure-cardiac interval-sympathetic nerve coherent, and endogenously generated vasogenic autorhythmicity waves.…”

Section: Cerebral Vasomotionmentioning

confidence: 99%

“…[287][288][289] Most interestingly, patients suffering from sickle cell disease evidence enhanced cerebral vasomotion, not precisely correlating with the magnitude of anemia, though varying according to parameters of red blood cell aggregability and rheologic properties of the blood. 172,176,177 Hemoglobin could consequently reduce buffering and inhibit the cerebral vasomotion by scavenging nitric oxide. 290,291 Enhanced blood viscosity could alternatively reflexively augment the cerebral vasomotion.…”

Section: Sickle Cell Diseasementioning

confidence: 99%

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

Ghali

Styler

2020

Journal of Pediatric Neurology

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The posterior reversible encephalopathy syndrome was characterized by Hinchey and colleagues in the 1990s. The condition frequently afflicts patients suffering from hematologic and solid organ malignancy and individuals undergoing transplantation. Cases are more frequently described in the adult population compared with children. In the pediatric population, malignancy, transplantation, renal disease, and hypertension represent the most common etiologies. Theories on pathogenesis have centered upon cerebrovascular dysautoregulation with increases in blood–brain barrier permeability. This generates vasogenic edema of the cerebral parenchyma and consequent neurologic deficits. The parietal and occipital lobes are affected with greatest prevalence, though frontal and temporal lobe involvement is frequent, and that of the contents of the infratentorial posterior cranial fossa are occasionally described. The clinical presentation involves a characteristic constellation of neurologic signs and symptoms, most typically inclusive of headache, visual-field disturbances, abnormalities of visual acuity, and seizures. Supportive care, withdrawal of the offending agent, antihypertensive therapy, and prophylactic anticonvulsants affect convalescence in majority of cases. The principal challenge lies in identifying the responsible agent precipitating the condition in patients with malignancy and those having undergone transplantation and thus deciding which medication among a multidrug treatment regimen to withhold, the duration of drug cessation required to effect clinical resolution, and the safety of resuming treatment with the compound. We accordingly reviewed and evaluated the literature discussing the posterior reversible encephalopathy syndrome in children.

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Cerebral and muscle microvascular oxygenation in children with sickle cell disease: Influence of hematology, hemorheology and vasomotion

Cited by 25 publications

References 44 publications

Sickle cell microvascular paradox—oxygen supply‐demand mismatch

Sickle cell microvascular paradox—oxygen supply‐demand mismatch

The Evaluation of Ophthalmic Findings in Women Patients With Iron and Vitamin B12 Deficiency Anemia

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

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