Natural History of Pediatric Patients with Sickle Cell Disease and Concurrent Cerebral Vasculopathies: Report of 62 Cases from a Single Institution

Papadakis, Joanna E.; Piwowarczyk, Paulina; Weber, Daniel S.; Slingerland, Anna L.; Keusch, Dylan S.; Heeney, Matthew M.; Smith, Edward; See, Alfred P.; Archer, Natasha M.

doi:10.1182/blood-2022-170784

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“…Cerebral vasculopathies. Papadakis et al reported in 2022, that in pediatric SCD, clinically evident acute strokes were largely identified in patients with Moyamoya, and that revascularization improves long-term stroke outcomes compared to medical management (34). Based on abnormal TCDs, followed by MRIs, we identified 2 of our patients with silent infarcts who are on escalated pharmacotherapy, and another one with Moyamoya, who underwent a revascularization, is on chronic PRBC transfusion and is being evaluated for HSCT.…”

Section: Chronic Kidney Disease (Ckd)mentioning

confidence: 95%

Sickle Cell Disease Related Vasculopathies and Early Evaluation in a Pediatric Population

PANOSYAN,

CORRAL

et al. 2024

In Vivo

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Background/Aim: Cardiovascular pathologies are ubiquitous in sickle cell disease (SCD). A targeted literature review was conducted to compare the overall epidemiology of selected vasculopathies seen in SCD (SCDVs) compared to the general population. Since many SCDV may originate in childhood, the study also focused on the retrospective investigation of SCDVs in a pediatric cohort at the Harbor-UCLA Medical Center. Patients and Methods: SCDVs were studied along patient age, β-globin genotypes, and fetal hemoglobin (HbF). Urine microalbumin/creatinine ratios (UM/Cr), trans-cranial doppler (TCD) and tricuspid regurgitant jet velocities (TRJV) were analyzed as well. Retinographies and overt vasculopathies were presented descriptively. Results: Among 20 females and 20 males [average 8.3 years (2.3-19 years)], 70% had HbSS/Sβ0, 22.5% HbSC and 7.5%-HbSβ+. The mean(±SD) HbF% was 17.4±12.7% (30% higher in <10 vs. ≥10 y/o, and 3 times higher in SS/Sβ0). Twenty-six patients received hydroxyurea and 13/26, L-glutamine. Thirty-six patients had TCDs within 1.4±0.9 years and all laboratory values were obtained within the last 12 months. TCDs showed low-normal velocities, but 2 were higher for HbSS/Sβ0 vs. HbSC/Sβ+ p=0.03; p<0.001). Nineteen of 28 patients with echocardiograms had

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Section: Chronic Kidney Disease (Ckd)mentioning

confidence: 95%