Department of Pediatric Gastroenterology, Çukurova University Medical Faculty, Adana, Turkey.
Department of Radiology, Çukurova University Medical Faculty, Adana, Turkey.
Turk J Gastroenterol. 2022 Feb;33(2):161-167. doi: 10.5152/tjg.2022.21545.
The primary aim of the study was to demonstrate parenchymal changes in the liver and pancreas related to copper accumulation using ultrasound in pediatric patients with Wilson's disease and secondly, to investigate the effectiveness of two-dimensional shear wave elastography in the diagnosis of involvement of these organs.
Patients with Wilson's disease (n = 25) who were treated and followed at our center were evaluated prospectively. In addition to routine clinical assessments, eye examination, laboratory analyses, and abdominal ultrasound imaging, all patients underwent tissue stiffness measurements from the liver and pancreas (head, body and tail) by two-dimensional shear wave elastography. The data obtained from the WD patients were compared with those of age- and sex-matched healthy controls (n = 37).
Liver elastography measurements showed significantly increased tissue stiffness in the patient group than in control subjects (P < .001). While there was no significant difference between the groups in the tissue thickness of pancreatic head, body, and tail, tissue stiffness was significantly reduced in the patient group (P < .001). Disease duration was significantly associated and moderately correlated with liver tissue stiffness (r = 0.417, P = .038) but not significantly associated with pancreatic tissue stiffness.
In the early stages of Wilson's disease, parenchymal changes occur in the liver and pancreas, which cannot be detected by conventional ultrasonography imaging but may be demonstrated by two-dimensional shear wave elastography. Ultrasound elastography is an easy to use, non-invasive, and promising method that provides numerical data on the early changes in tissue stiffness, allowing for objective monitoring of Wilson's disease patients who require lifelong follow-up.
本研究的主要目的是使用超声显示肝和胰腺实质变化，以显示与铜积累相关的威尔逊病患儿的肝和胰腺实质变化，其次，研究二维剪切波弹性成像在诊断这些器官受累中的有效性。
前瞻性评估在我院治疗和随访的威尔逊病患者（n = 25）。除了常规临床评估、眼部检查、实验室分析和腹部超声成像外，所有患者还接受了肝和胰腺（头部、体部和尾部）的组织硬度测量，使用二维剪切波弹性成像。将 WD 患者的数据与年龄和性别匹配的健康对照组（n = 37）的数据进行比较。
肝弹性成像测量显示患者组的组织硬度明显高于对照组（P <.001）。虽然胰头部、体部和尾部的组织厚度在两组之间没有显著差异，但患者组的组织硬度明显降低（P <.001）。疾病持续时间与肝组织硬度显著相关且中度相关（r = 0.417，P =.038），但与胰腺组织硬度无显著相关性。
在威尔逊病的早期阶段，肝脏和胰腺会发生实质变化，常规超声成像无法检测到，但二维剪切波弹性成像可以显示。超声弹性成像是一种简单易用、无创且有前途的方法，它提供了组织硬度早期变化的数值数据，可对需要终身随访的威尔逊病患者进行客观监测。
