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Improved Turnaround Times | Median time to first decision: 12 days

OtherPediatric Neuroimaging

The Effect of Hydroxyurea on Vasculopathy in a Child with Sickle Cell Disease

Kathleen J. Helton, Winfred C. Wang, Lynn W. Wynn, Raja B. Khan and R. Grant Steen
American Journal of Neuroradiology November 2002, 23 (10) 1692-1696;
Kathleen J. Helton
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Winfred C. Wang
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Lynn W. Wynn
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Raja B. Khan
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R. Grant Steen
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  • Fig 1.
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    Fig 1.

    Serial MR images in a patient receiving hydroxyurea. The first examination was performed at another institution.

    A, Examination 1. T1-weighted image shows mild atrophy in the left medial frontal lobe.

    B, Examination 1. Fluid-attenuated inversions recovery image demonstrates a recent infarction in the right middle cerebral artery territory, as well as a remote left anterior cerebral artery infarct, bilateral deep white matter leukoencephalopathy, and a few scattered deep white matter lacunae.

    C, Examination 2. Image obtained at our institution almost 1 year after the beginning of therapy shows further progression of mild diffuse cerebral atrophy.

    D, Examination 5. Approximately 4 years after the beginning of hydroxyurea therapy moderate diffuse cerebral atrophy is present, with prominent sulci and further ex vacuo dilatation of the ventricular system.

  • Fig 2.
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    Fig 2.

    Summary of the clinical history of the patient. Plots show the MR imaging dates, with the percentage of fetal hemoglobin (Hb F), hemoglobin (Hgb), white blood cell (WBC) count, reticulocyte count, mean corpuscular volume (MCV), and platelet count after the initiation hydroxyurea treatment at time 0. HU indicates hydroxyurea.

  • Fig 3.
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    Fig 3.

    Serial MRA images in a patient receiving hydroxyurea. Images show progressive improvement in vascular patency.

    A and B, Examination 2. One year after beginning therapy, an 8-mm severe stenosis is present in the left ICA, with a large left posterior communicating (PCOM) artery. Also present is a 10-mm stenosis in the proximal right A-1 segment of the ACA, as well as proximal segmental stenoses in both middle cerebral arteries (MCAs) (11 mm, right M-1; 9 mm, left M-1). Small moyamoya collaterals are present near the right A-1 and M-1 segments.

    C and D, Examination 3. Eight months later, with the MRA examination further optimized for rapid flow, the left ICA stenosis has nearly resolved, with only a 5-mm region of tapering involving the supraclinoid segment. Stenoses involving the left M-1, right A-1, and right M-1 segments had decreased to 7, 3, and 3 mm, respectively. A short 2-mm proximal stenosis is present in the left A-1 segment. Moyamoya vasculopathy was unchanged.

  • Fig 4.
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    Fig 4.

    Serial MRA images in the patient in Figure 3. Images show progressive improvement in vascular patency.

    A and B, Examination 3. The bilateral A-1 segments have become equivalent in size and the distal left ICA is less tapered. A 2-mm stenosis of the proximal left A-1 and a 1-mm stenosis of the proximal left M-1 segments persist. Also noted are the resolution of the right A-1 and M-1 stenoses and the moyamoya vasculopathy with persistent short-segmental stenoses in the proximal left A-1 and M-1 segments. The right MCA appears to be more patent; small-vessel conspicuity is lost; and both PCOMs have become smaller, suggesting a slower rate of blood flow.

    C and D, Examination 4. Persistent short segment stenoses of the proximal left A-1 and M-1 segments remain.

Tables

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  • Hematologic values in the case patient before and during treatment with hydroxyurea

    ValueBefore TreatmentDuring TreatmentPercentage Change (%)
    Hemoglobin (g/dL)8.89.9 ± 1.3+13
    %Hb F5.931.4 ± 7.0+432
    MCV (fl)88.6116.0 ± 4.0+31
    Platelet count (×10−2/mm3)41.327.1 ± 9.9−34
    White blood cell count (×10−3/mm3)15.85.4 ± 1.3−66
    Reticulocytes (%)14.02.6 ± 2.4−81
    • Note.—All values were abnormal before treatment, but all improved during hydroxyurea therapy. The platelet count, which is usually expressed in units of ×10−3/mm3, are expressed here in units of 10−2/mm3 to plot the results in Figure 2.

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American Journal of Neuroradiology: 23 (10)
American Journal of Neuroradiology
Vol. 23, Issue 10
1 Nov 2002
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Cite this article
Kathleen J. Helton, Winfred C. Wang, Lynn W. Wynn, Raja B. Khan, R. Grant Steen
The Effect of Hydroxyurea on Vasculopathy in a Child with Sickle Cell Disease
American Journal of Neuroradiology Nov 2002, 23 (10) 1692-1696;

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The Effect of Hydroxyurea on Vasculopathy in a Child with Sickle Cell Disease
Kathleen J. Helton, Winfred C. Wang, Lynn W. Wynn, Raja B. Khan, R. Grant Steen
American Journal of Neuroradiology Nov 2002, 23 (10) 1692-1696;
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