Correlation of mri-based bone marrow burden score with gender and spleen status in Gaucher's disease
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منابع مشابه
Correlation of MRI-Based bone marrow burden score with genotype and spleen status in Gaucher's disease.
OBJECTIVE The purpose of this study was to correlate skeletal pathologic findings quantified by MRI-based bone marrow burden score with genotype and spleen status and other clinical parameters, including liver size and duration of enzyme replacement therapy, in patients with Gaucher's disease. MATERIALS AND METHODS Two radiologists retrospectively reviewed MR images of 47 patients with Gauche...
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UNLABELLED Gaucher's disease is a lysosomal storage disorder due to a genetically transmitted deficiency of the enzyme glucocerebrosidase. In the most common form of the disease (type 1), accumulation of glucosylceramide in the reticuloendothelial cells of liver, spleen, and bone marrow leads to visceromegaly, anemia, thrombocytopenia, and osteopenia. Skeletal manifestations secondary to infilt...
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Gaucher disease, the most common lysosomal storage disorder, is caused by β-glucocerebrosidase deficiency. Bone complications are the major cause of morbidity in patients with type 1 Gaucher disease (GD1). Genetic components strongly influence bone remodelling. In addition, chronic inflammation produced by Gaucher cells induces the production of several cytokines, which leads to direct changes ...
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OBJECTIVE To evaluate by magnetic resonance imaging changes in bone marrow of patients undergoing treatment for type I Gaucher's disease. MATERIALS AND METHODS Descriptive, cross-sectional study of Gaucher's disease patients submitted to 3 T magnetic resonance imaging of femurs and lumbar spine. The images were blindly reviewed and the findings were classified according to the semiquantitativ...
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