Evaluation of treatment response to enzyme replacement therapy with Velaglucerase alfa in patients with Gaucher disease using whole-body magnetic resonance imaging

2016 ◽  
Vol 57 ◽  
pp. 35-41 ◽  
Author(s):  
K. Laudemann ◽  
L. Moos ◽  
E. Mengel ◽  
A. Lollert ◽  
C. Hoffmann ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Treatment Response ◽  
Gaucher Disease ◽  
Whole Body ◽  
Resonance Imaging ◽  
Enzyme Replacement ◽  
Body Magnetic Resonance Imaging

Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1

2018 ◽  
Vol 231 (02) ◽  
pp. 52-59
Author(s):  
André Lollert ◽  
Katharina Laudemann ◽  
Eugen Mengel ◽  
Christian Hoffmann ◽  
Larissa Moos ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Gaucher Disease ◽  
Disease Type ◽  
Whole Body ◽  
Enzyme Replacement ◽  
Gaucher Disease Type 1 ◽  
Body Magnetic Resonance Imaging

Abstract Purpose We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy. Materials and Methods In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: “early” (age ≤12 years, median 5 years) and “late” (during adulthood, median 32 years). We evaluated occurrence of irreversible avascular necroses (AVN) and applied several semi-quantitative scores, including the Bone-Marrow-Burden (BMB) score, the Düsseldorf-Gaucher score (DGS), the Vertebra-Disc-Ratio (VDR), and the Gaucher disease type 1 Severity Scoring System (GD-DS3). Results MRI assessments showed no AVN in the “early” group. AVN were observed in 2 patients of the “late” group; one also had a splenic Gaucheroma. The follow-up examinations showed slight improvements in the BMB-score, DGS, and VDR, with similar tendencies in both treatment groups. The GD-DS3 score only improved in “late” group. Conclusion This retrospective study supported the ongoing clinical value of enzyme replacement therapy with alglucerase/imiglucerase, as WB-MRI-based scores stayed constant or slightly improved even after long-term treatment. Secondary complications were only observed in the late treatment group. Our results suggest that “early initiation” of enzyme replacement therapy may protect the bone.

scholarly journals Whole-body magnetic resonance imaging (WBMRI) versus whole-body computed tomography (WBCT) for myeloma imaging and staging

2021 ◽  
Author(s):  
Karla M. Treitl ◽  
Jens Ricke ◽  
Andrea Baur-Melnyk
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Bone Marrow ◽  
Magnetic Resonance ◽  
Treatment Response ◽  
Standard Procedure ◽  
Whole Body ◽  
Resonance Imaging ◽  
Mortality And Morbidity ◽  
Body Magnetic Resonance Imaging

AbstractMyeloma-associated bone disease (MBD) develops in about 80–90% of patients and severely affects their quality of life, as it accounts for the majority of mortality and morbidity. Imaging in multiple myeloma (MM) and MBD is of utmost importance in order to detect bone and bone marrow lesions as well as extraosseous soft-tissue masses and complications before the initiation of treatment. It is required for determination of the stage of disease and aids in the assessment of treatment response. Whole-body low-dose computed tomography (WBLDCT) is the key modality to establish the initial diagnosis of MM and is now recommended as reference standard procedure for the detection of lytic destruction in MBD. In contrast, whole-body magnetic resonance imaging (WBMRI) has higher sensitivity for the detection of focal and diffuse plasma cell infiltration patterns of the bone marrow and identifies them prior to osteolytic destruction. It is recommended for the evaluation of spinal and vertebral lesions, while functional, diffusion-weighted MRI (DWI-MRI) is a promising tool for the assessment of treatment response. This review addresses the current improvements and limitations of WBCT and WBMRI for diagnosis and staging in MM, underlining the fact that both modalities offer complementary information. It further summarizes the corresponding radiological findings and novel technological aspects of both modalities.

scholarly journals The effect of GH replacement therapy on different fat compartments: a whole-body magnetic resonance imaging study

2011 ◽  
Vol 164 (1) ◽  
pp. 23-29 ◽  
Author(s):  
A Egger ◽  
T Buehler ◽  
C Boesch ◽  
P Diem ◽  
C Stettler ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Replacement Therapy ◽  
Imaging Study ◽  
Whole Body ◽  
Model Assessment ◽  
Resonance Imaging ◽  
Gh Replacement ◽  
Before And After ◽  
Body Magnetic Resonance Imaging

ObjectivePatients with GH deficiency (GHD) are insulin resistant with an increase in visceral fat mass (FM). Whether this holds true when sedentary control subjects (CS) are matched for waist has not been documented.GH replacement therapy (GHRT) results in a decrease in FM. Whether the decrease in FM is mainly related to a reduction in visceral FM remains to be proven. The aim was to separately assess visceral and subcutaneous FM in relation to insulin resistance (IR) in GHD patients before and after GHRT and in sedentary CS.MethodsTen patients with GHD were investigated before and 6 months after GHRT. Sedentary CS matched for age, gender, body mass index, and waist were assessed. Exercise capacity was measured as VO2maxusing an incremental work load on a treadmill. Visceral and subcutaneous FM were measured using whole-body magnetic resonance imaging and IR by the homeostasis model assessment of IR (HOMA-IR) index.ResultsGHD patients had a non-significantly lower VO2maxbut did not have increased subcutaneous and visceral FM compared with CS. GHRT resulted in a similar relative decrease in subcutaneous and visceral FM. Compared with CS, GHD patients showed a lower HOMA-IR. GHRT tended to increase HOMA-IR.ConclusionMatching for waist and separate assessment of visceral and subcutaneous FM may be critical in the evaluation of body composition and IR in GHD patients before and after GHRT.

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