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  • 1
    ISSN: 1432-2161
    Keywords: Keywords Gaucher disease ; MRI ; Enzyme replacement therapy ; Liver ; Spleen ; Bone marrow
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Purpose. To determine whether T1-weighted magnetic resonance (MR) images can demonstrate response in the marrow of patients with type 1 Gaucher disease treated with enzyme replacement therapy (ERT) and to determine whether a relationship exists between liver and spleen volume reductions and visible marrow changes. Patients. Forty-two patients with type 1 Gaucher disease were evaluated on at least two occasions. Thirty-two patients received ERT. Of these patients, 15 had a baseline examination prior to the initiation of ERT. The remaining 10 patients did not receive ERT. Design. T1-weighted and gradient recalled echo (GRE) coronal images of the femurs and hips were obtained. Concurrently, liver and spleen volumes were determined using contiguous breath-hold axial gradient-echo images. T1-weighted images of the hips and femurs were evaluated to determine change or lack of change in the yellow marrow. Results. Of the 32 patients receiving ERT, 14 (44%) demonstrated increased signal on T1-weighted images suggesting an increase in the amount of yellow marrow. If only the 15 patients with a baseline examination were considered, the response rate to ERT was 67%. Using Student’s t-test a highly significant correlation (P〈0.005) was found between marrow response and reduction in liver and spleen volume. Conclusions. Marrow changes in patients receiving ERT can be detected by T1-weighted images. This response correlated with reductions in visceral volumes (P〈0.0005).
    Type of Medium: Electronic Resource
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