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
URL:
http://dx.doi.org/10.1007/s002560000276