OBJECTIVE: Since 1991, enzyme replacement therapy (ERT) has been available for @entity1 with @entity1120 in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in @entity1120 . @entity1 AND DESIGN: Thirty @entity1 with @entity1120 had MRI examinations prior to initiation of ERT with @entity15675 / @entity15675 and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. RESULTS: Overall, 19 of 30 @entity1 (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal @entity79 surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting @entity1188 . Of the 11 @entity1 with @entity1188 (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of @entity1 with splenectomy, @entity1188 were seen (P<0.05). CONCLUSIONS: MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in @entity1 receiving ERT. A non-homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of @entity1188 (P=0.0021).

Magnetic resonance imaging of bone marrow changes in XXXX during enzyme replacement therapy: first German long-term results.

@entity1 :: ('9606', 'Species') :: ['patients', 'PATIENTS'] @entity15675 :: ('2629', 'Gene') :: ['imiglucerase', 'alglucerase'] @entity79 :: ('MESH:D001847', 'Disease') :: ['bone lesions'] @entity1188 :: ('MESH:D007238', 'Disease') :: ['bone infarcts'] @entity1120 :: ('MESH:D005776', 'Disease') :: ['Gaucher disease', 'Gaucher disease type I']
@entity1120 :: (MESH:D005776,Disease) :: ['Gaucher disease']
