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[戈谢病中的骨病]

[Bone disease in Gaucher's disease].

作者信息

Roca Espiau Mercedes

机构信息

Centro de Diagnóstico por Imagen, Zaragoza, Spain.

出版信息

Med Clin (Barc). 2011 Sep;137 Suppl 1:23-31. doi: 10.1016/S0025-7753(11)70013-6.

DOI:10.1016/S0025-7753(11)70013-6
PMID:22230122
Abstract

The exposition aims, is to review the pathophysiological mechanisms of bone marrow involvement and the patterns of marrow infiltration by Gaucher cells. We have reviewed the different methods of assessment of bone marrow infiltration and its temporal development. Qualitative methods include simple radiography, magnetic resonance imaging (MRI), computed tomography (CT) and radioisotope. The simple radiography is the basic element, but its sensitivity is limited and only allows for assessing changes and trabecular bone remodeling MRI allows us to appreciate the bone marrow infiltration, detection of complications and response to therapy. Radioisotopes can contribute to the differential diagnosis of osteomyelitis and bone crises. Among the quantitative methods are the QCSI (quantitative chemical shift imaging) and the dual-energy X-ray absorptiometry (DEXA), as well as new quantitative techniques of CT, MRI and ultrasound densitometry. The QCSI performed an assessment of fat content of bone marrow in the spine. DEXA quantifies bone density by measuring the attenuation coefficient. The semiquantitative methods have various "scores" to establish criteria for generalized bone disease endpoints of disease progression and response to therapy.

摘要

本次论述旨在回顾骨髓受累的病理生理机制以及戈谢细胞的骨髓浸润模式。我们回顾了评估骨髓浸润及其时间发展的不同方法。定性方法包括简单放射照相、磁共振成像(MRI)、计算机断层扫描(CT)和放射性同位素。简单放射照相是基本手段,但其敏感性有限,仅能评估变化和小梁骨重塑。MRI使我们能够了解骨髓浸润情况、检测并发症以及评估治疗反应。放射性同位素有助于骨髓炎和骨危象的鉴别诊断。定量方法包括定量化学位移成像(QCSI)和双能X线吸收法(DEXA),以及CT、MRI和超声骨密度测定的新定量技术。QCSI用于评估脊柱骨髓的脂肪含量。DEXA通过测量衰减系数来量化骨密度。半定量方法有各种“评分”,以建立全身性骨病疾病进展终点和治疗反应的标准。

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1
[Bone disease in Gaucher's disease].[戈谢病中的骨病]
Med Clin (Barc). 2011 Sep;137 Suppl 1:23-31. doi: 10.1016/S0025-7753(11)70013-6.
2
Quantification of skeletal involvement in adults with type I Gaucher's disease: fat fraction measured by Dixon quantitative chemical shift imaging as a valid parameter.I型戈谢病成年患者骨骼受累情况的量化:通过狄克逊定量化学位移成像测量脂肪分数作为有效参数。
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A Comprehensive Study of Bone Manifestations in Adult Gaucher Disease Type 1 Patients in Argentina.阿根廷 1 型戈谢氏病成年患者骨骼表现的综合研究。
Calcif Tissue Int. 2019 Jun;104(6):650-657. doi: 10.1007/s00223-019-00536-x. Epub 2019 Feb 21.

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Bone marrow adipocytes alteration in an model of Gaucher Disease.戈谢病模型中的骨髓脂肪细胞改变
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