title: Bone marrow relaxation times in Gaucher disease before and after enzyme replacement therapy.
abstract: The aim of this work was to monitor the effectiveness of enzyme replacement therapy on the basis of the changes in T1 relaxation times in Gaucher patients. A total of 26 patients underwent MR before enzyme replacement therapy; of them, 18 have been followed-up. A total of 22 age-matched controls underwent the same MR study. Scans were focused on the femoral neck, and T1 relaxation times were measured by means of a mixed spin-echo inversion recovery sequence. The T1 relaxation times in Gaucher patients were significantly longer than normal (p < 0.05). After enzyme replacement therapy, T1 relaxation times gradually became closer to those of control subjects, and there was also a significant decrease (p < 0.01) with respect to values before therapy, probably due to an increase in the fat/water ratio. Evaluation of T1 relaxation time may supply a useful indication of Gaucher disease regression after enzyme replacement therapy particularly in those cases in which a normal skeletal appearance corresponds to prolonged T1 relaxation times.
authors.affiliation: Department of Radiology, University Hospital of Trieste, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Magnaldi
authors.forename: R
authors.initials: R
authors.lastname: Longo
authors.forename: M
authors.initials: M
authors.lastname: Ukmar
authors.forename: M
authors.initials: M
authors.lastname: Zanatta
authors.forename: M
authors.initials: M
authors.lastname: Bottega
authors.forename: G L
authors.initials: GL
authors.lastname: Sottocasa
mesh_terms: D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005269:Femur / Q000473:pathology; D005776:Gaucher Disease / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D005962:Glucosylceramidase / Q000627:therapeutic use*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged
chemical_list: C070292:alglucerase; D005962:Glucosylceramidase