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Response of Gaucher bone disease to enzyme replacement therapy

L W Poll, MD1, M Maas, MD2, M R Terk, MD3, M Roca-Espiau, MD4, B Bembi, MD5, G Ciana, MD5 and N J Weinreb, MD6

1 Institute of Diagnostic Radiology, Heinrich Heine University, Düsseldorf, Germany, 2 Department of Radiology, Academic Medical Centre, Amsterdam, The Netherlands, 3 Keck School of Medicine, University of Southern California, USA, 4 Department of Radiology, Musculoskeletal MRI Section, Miguel Servet Hospital, Zaragoza, Spain, 5 Burlo Garofolo Institute, Trieste, Italy and 6 University Gaucher Research Foundation Inc., University Gaucher Treatment Center, Tamarac, Florida, USA



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  		Figure 1. Coronal T1-weighted magnetic resonance images of the femurs, TR/TE 615/25, of a patient with Gaucher disease from a study conducted at Heinrich Heine University Düsseldorf: (a) before starting enzyme replacement therapy (ERT); (b) after 9 months of ERT (60 U/kg/2 weeks).

 


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  		Figure 2. Plot of changes in T1-weighted marrow images against spleen volume changes for patients studied at The Keck School of Medicine of the University of Southern California. Mean volumes are shown numerically. Reproduced from Skeletal Radiol 2000;291:563–71 [11] with permission of the International Skeletal Society.

 


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  		Figure 3. Patterns of bone marrow involvement on magnetic resonance images, TR/TE 600/20 from patients studied at Miguel Servet Hospital: (a) homogenous morphology or pattern of involvement on a sagittal T1-weighted MRI of the spine; (b) non-homogenous diffuse morphology on a sagittal T1-weighted magnetic resonance image of the spine; (c) non-homogenous mottled morphology on a coronal T1-weighted magnetic resonance image of the pelvis; (d) non-homogenous reticular morphology on a coronal T1-weighted magnetic resonance image of the femurs.

 


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  		Figure 4. Colour representation of the change in the bone marrow fat fraction (as measured by QCSI every year) in the lumbar spine of a patient with Gaucher disease receiving ERT. The darker colours represent a lower fat fraction as shown on the scale.

 


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  		Figure 5. Change with time in VDR score in patients with Gaucher disease: (a) untreated patients studied at the Academic Medical Centre in Amsterdam; (b) patients receiving enzyme replacement therapy.

 


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  		Figure 6. Change after 1 year of enzyme replacement therapy in the functional status of patients in the Gaucher Registry with restricted mobility at baseline.

 




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