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Titolo:
Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement.
Autore:
Poll, LW; Dahl, SV; Koch, JA; Borner, D; Willers, R; Cohnen, M; Jung, G; Scherer, A; Niederau, C; Haussinger, D; Modder, U;
Indirizzi:
Univ Dusseldorf, Inst Diagnost Radiol, D-40225 Dusseldorf, Germany Univ Dusseldorf Dusseldorf Germany D-40225 , D-40225 Dusseldorf, Germany Univ Dusseldorf, Klin Gastroenterol Hepatol & Infektiol, D-40225 Dusseldorf, Germany Univ Dusseldorf Dusseldorf Germany D-40225 , D-40225 Dusseldorf, Germany Univ Dusseldorf, Rechenzentrum, D-40225 Dusseldorf, Germany Univ Dusseldorf Dusseldorf Germany D-40225 , D-40225 Dusseldorf, Germany Univ Essen Gesamthsch, Akad Lehrkrankenhaus, St Josef Hosp Oberhausen, Innere Abt, D-4300 Essen 1, Germany Univ Essen Gesamthsch Essen Germany 1 nnere Abt, D-4300 Essen 1, Germany
Titolo Testata:
ROFO-FORTSCHRITTE AUF DEM GEBIET DER RONTGENSTRAHLEN UND DER BILDGEBENDEN VERFAHREN
fascicolo: 10, volume: 173, anno: 2001,
pagine: 931 - 937
SICI:
1438-9029(200110)173:10<931:GDMEOB>2.0.ZU;2-H
Fonte:
ISI
Lingua:
GER
Soggetto:
SKELETAL INVOLVEMENT; THERAPY; GLUCOCEREBROSIDASE; RESPONSES; SPLEEN;
Keywords:
MRI; bone marrow; sphingolipidosis; enzyme replacement therapy; Gaucher disease;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
30
Recensione:
Indirizzi per estratti:
Indirizzo: Poll, LW Univ Dusseldorf, Inst Diagnost Radiol, Moorenstr 5, D-40225 Dusseldorf, Germany Univ Dusseldorf Moorenstr 5 Dusseldorf Germany D-40225 , Germany
Citazione:
L.W. Poll et al., "Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement.", ROFO-F RONT, 173(10), 2001, pp. 931-937

Abstract

Purpose: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluatingbone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. Materials and Methods: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T-1/T-2) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. Results: infiltration of bm by Gaucher cells is characterized by a reduction of SI on both T-1- and T-2-weighted sequences. Bone marrow responses were seen in 19 patients (63%) during treatment. Focal bone lesions, surrounded by a rim of reduced SI, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. Conclusion: In adult patients with type I Gaucher disease receiving ERT, treatment effects on bone disease can be demonstrated by MRI using Spin-echo sequences due to the partial reconversion of fat marrow. A non-homogeneous type of signal appearance and a status post splenectomy correlate with the presence of bone infarcts.

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Documento generato il 06/04/20 alle ore 11:57:08