MR-IMAGING OF PATIENTS WITH TYPE-1 GAUCHERS-DISEASE - RELATIONSHIP BETWEEN BONE AND VISCERAL CHANGES

OBJECTIVE. Because treatment is now available for patients with Gauche r's disease, methods of follow-up have become important in making trea tment decisions, Bone abnormalities (infarct and avascular necrosis) t end to be irreversible while visceral volumes are not, suggesting that it may be useful to follow patients with visceral volume determinatio ns, In order to do so, the relationship of one to the other must first be understood. The purpose of this paper is to determine the relation ships between bone and visceral changes identified with MR imaging. MA TERIALS AND METHODS. Sixty-two consecutive patients from 1 to 78 years old with type 1 Gaucher's disease were studied by MR imaging, The exa mination consisted of two parts: T1-weighted MR images and gradient-ec ho coronal MR images of the hips and femurs, and axial breath-holding MR images of the abdomen that allowed measurement of hepatic and splen ic volume. The bone changes were classified and were correlated with t he liver and spleen volumes, Specific imaging features of the liver an d spleen were noted and correlation sought. RESULTS. A statistically s ignificant difference in liver size was found between patients with an d without avascular necrosis, A statistically significant correlation was also found between liver size and avascular necrosis, Patients wit h marrow changes classified as grade 2 or higher were significantly mo re likely to have avascular necrosis, Nineteen percent of patients had splenic nodules and 7% had hepatic nodules; however, there was no cor relation between the nodules and bone changes. CONCLUSION. MR imaging showed a statistical relationship between marrow changes, liver size, and avascular necrosis in patients with type 1 Gaucher's disease, Know ledge of this relationship may be used in dosage and treatment conside rations in the follow-up and clinical management of these patients.