Webb Muriel, Zimran Ari, Dinur Tama, Shibolet Oren, Levit Stella, Steinberg David M, Salomon Ophira
Department of Gastroenterology, Tel-Aviv Medical Center, Tel-Aviv, Israel and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Shaare Zedek Medical Center, and Hebrew University-Hadassah Medical School, Jerusalem, Israel.
Blood Cells Mol Dis. 2018 Feb;68:143-147. doi: 10.1016/j.bcmd.2016.12.010. Epub 2016 Dec 23.
Up to now, there are no reliable biochemical markers or imaging that could reveal early tissue damage in Gaucher disease. Therefore, we addressed whether elastography technique can serve as a tool for evaluating patients with Gaucher disease. The study included 42 patients with Gaucher disease type I and 33 patients with liver cirrhosis as well as 22 healthy volunteers. Ultrasound and Doppler examination was performed on each participant prior to apply transient and 2D shear wave elastography. In Gaucher disease the median stiffness of the spleen as assessed by transient elastography (TE) and shear wave elastography (SWE) was 35KPa and 22KPa respectively in contrast to the median stiffness of healthy controls (16.95 and 17.5KPa, p=0.0028 and p=0.0002, respectively) and of patients with cirrhosis (45KPa and 34.5KPa, p=0.015 and p<0.0001 respectively). The liver stiffness in GD as measured by TE and SWE had median values of 7.1KPa and 7KPa respectively, slightly higher than in the healthy controls, but much smaller than for the cirrhotic patients (medians of 24.2KPa and 21KPa). In conclusion, a transient and shear wave elastography show a significant promise as noninvasive and reproducible tools to differentiate Gaucher disease from healthy controls and among those with splenomegaly from cirrhotic patients.
到目前为止，尚无可靠的生化标志物或影像学检查能够揭示戈谢病早期的组织损伤。因此，我们探讨了弹性成像技术是否可作为评估戈谢病患者的一种工具。该研究纳入了42例I型戈谢病患者、33例肝硬化患者以及22名健康志愿者。在应用瞬时弹性成像和二维剪切波弹性成像之前，对每位参与者进行了超声和多普勒检查。在戈谢病患者中，通过瞬时弹性成像（TE）和剪切波弹性成像（SWE）评估的脾脏硬度中位数分别为35KPa和22KPa，相比之下，健康对照组的中位数硬度为（16.95和17.5KPa，p值分别为0.0028和0.0002），肝硬化患者的中位数硬度为（45KPa和34.5KPa，p值分别为0.015和<0.0001）。通过TE和SWE测量的戈谢病患者肝脏硬度中位数分别为7.1KPa和7KPa，略高于健康对照组，但远低于肝硬化患者（中位数分别为24.2KPa和21KPa）。总之，瞬时弹性成像和剪切波弹性成像作为无创且可重复的工具，在区分戈谢病与健康对照组以及区分脾肿大的戈谢病患者与肝硬化患者方面显示出巨大潜力。
