Poll, L. W., Willers, R., Haussinger, D., Modder, U. and vom Dahl, S. (2010). MRI Bone Marrow Findings in 63 Patients with Type I Gaucher Disease. Rofo-Fortschr. Gebiet Rontgenstrahlen Bildgeb. Verfahr., 182 (11). S. 979 - 986. STUTTGART: GEORG THIEME VERLAG KG. ISSN 2509-8799

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Abstract

Purpose To determine whether MR bone marrow findings in Gaucher patients may help to identify patients at high risk of developing severe Gaucher bone complications exemplified by avascular necrosis (AVN) of the femoral head Materials and Methods MR images were obtained in 63 Type I Gaucher patients through a standard protocol using coronal Ti and T2-weighted sequences of the lower extremities The location and extent of infiltrated marrow was established using a semi-quantitative MRI scoring method (Dusseldorf Gaucher score DGS) and the morphological pattern of bone marrow involvement determined (whether homogeneous type A or non-homogeneous type B) The active marrow process with bone edema and AVN of the femoral head were also analyzed Results Bone marrow involvement was observed in femoral sites more than in tibial sites A high DGS was significantly correlated with type B morphology and femoral AVN (both p < 0 0001) Splenectomized patients showed a significantly higher Dusseldorf Gaucher score and type B morphology than non-splenectomized patients (both p < 0 05) AVN was seen in 46% of patients with type B morphology versus 3% in type A morphology (p < 0 0001) DGS and morphology of bone marrow involvement were not significantly correlated with active marrow processes Conclusion Type B marrow morphology and extensive marrow packing were significantly associated with AVN of the femoral head (both p < 0 0001) These patterns ale considered predictive and may be employed in a disease management context to alert physicians to the need for urgent therapeutic measures

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Poll, L. W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Willers, R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haussinger, D.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Modder, U.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
vom Dahl, S.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-493999
DOI: 10.1055/s-0029-1245410
Journal or Publication Title: Rofo-Fortschr. Gebiet Rontgenstrahlen Bildgeb. Verfahr.
Volume: 182
Number: 11
Page Range: S. 979 - 986
Date: 2010
Publisher: GEORG THIEME VERLAG KG
Place of Publication: STUTTGART
ISSN: 2509-8799
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
ENZYME REPLACEMENT THERAPY; SKELETAL MANIFESTATIONS; INVOLVEMENT; RESPONSES; ADULTSMultiple languages
Radiology, Nuclear Medicine & Medical ImagingMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/49399

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