Terk M R, Esplin J, Lee K, Magre G, Colletti P M
Department of Radiology, University of Southern California School of Medicine, Los Angeles, 90033, USA.
AJR Am J Roentgenol. 1995 Sep;165(3):599-604. doi: 10.2214/ajr.165.3.7645477.
Because treatment is now available for patients with Gaucher's disease, methods of follow-up have become important in making treatment decisions. Bone abnormalities (infarct and avascular necrosis) tend to be irreversible while visceral volumes are not, suggesting that it may be useful to follow patients with visceral volume determinations. In order to do so, the relationship of one to the other must first be understood. The purpose of this paper is to determine the relationships between bone and visceral changes identified with MR imaging.
Sixty-two consecutive patients from 1 to 78 years old with type 1 Gaucher's disease were studied by MR imaging. The examination consisted of two parts: T1-weighted MR images and gradient-echo coronal MR images of the hips and femurs, and axial breath-holding MR images of the abdomen that allowed measurement of hepatic and splenic volume. The bone changes were classified and were correlated with the liver and spleen volumes. Specific imaging features of the liver and spleen were noted and correlation sought.
A statistically significant difference in liver size was found between patients with and without avascular necrosis. A statistically significant correlation was also found between liver size and avascular necrosis. Patients with marrow changes classified as grade 2 or higher were significantly more likely to have avascular necrosis. Nineteen percent of patients had splenic nodules and 7% had hepatic nodules; however, there was no correlation between the nodules and bone changes.
MR imaging showed a statistical relationship between marrow changes, liver size, and avascular necrosis in patients with type 1 Gaucher's disease. Knowledge of this relationship may be used in dosage and treatment considerations in the follow-up and clinical management of these patients.
由于目前已有针对戈谢病患者的治疗方法，随访方法在制定治疗决策中变得至关重要。骨异常（梗死和缺血性坏死）往往是不可逆的，而内脏容积并非如此，这表明对患者进行内脏容积测定可能有用。为了做到这一点，必须首先了解两者之间的关系。本文的目的是确定通过磁共振成像（MR成像）识别的骨与内脏变化之间的关系。
对62例年龄在1至78岁之间的1型戈谢病患者进行了MR成像研究。检查包括两部分：髋部和股骨的T1加权MR图像以及梯度回波冠状位MR图像，以及腹部屏气MR图像，以测量肝脏和脾脏容积。对骨变化进行分类，并与肝脏和脾脏容积进行关联。记录肝脏和脾脏的特定成像特征并寻求相关性。
有无缺血性坏死的患者之间肝脏大小存在统计学显著差异。肝脏大小与缺血性坏死之间也存在统计学显著相关性。骨髓变化分类为2级或更高的患者发生缺血性坏死的可能性显著更高。19%的患者有脾结节，7%的患者有肝结节；然而，结节与骨变化之间没有相关性。
MR成像显示1型戈谢病患者的骨髓变化、肝脏大小与缺血性坏死之间存在统计学关系。了解这种关系可用于这些患者随访和临床管理中的剂量和治疗考虑。
