Dillman Jonathan R, Serai Suraj D, Trout Andrew T, Singh Ruchi, Tkach Jean A, Taylor Amy E, Blaxall Burns C, Fei Lin, Miethke Alexander G
Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229-3026, USA.
Center for Autoimmune Liver Disease (CALD), Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Pediatr Radiol. 2019 Mar;49(3):332-341. doi: 10.1007/s00247-018-4319-1. Epub 2019 Jan 3.
Primary sclerosing cholangitis, autoimmune hepatitis and autoimmune sclerosing cholangitis are forms of chronic, progressive autoimmune liver disease (AILD) that can affect the pediatric population.
To determine whether quantitative MRI- and laboratory-based biomarkers are associated with conventional imaging findings of portal hypertension (radiologic portal hypertension) in children and young adults with AILD.
Forty-four patients with AILD enrolled in an institutional registry underwent a research abdominal MRI examination at 1.5 tesla (T). Five quantitative MRI techniques were performed: liver MR elastography, spleen MR elastography, liver iron-corrected T1 mapping, liver T2 mapping, and liver diffusion-weighted imaging (DWI, quantified as apparent diffusion coefficients). Two anatomical sequences were used to document splenomegaly, varices and ascites. We calculated aspartate aminotransferase (AST)-to-platelet ratio index (APRI) and fibrosis-4 (FIB-4) scores - laboratory-based biomarkers of liver fibrosis. We used receiver operating characteristic (ROC) curve analyses to establish the diagnostic performance of quantitative MRI and laboratory biomarkers for indicating the presence of radiologic portal hypertension.
Twenty-three (52%) patients were male; mean age was 15.2±4.0 years. Thirteen (30%) patients had radiologic portal hypertension. Liver and spleen stiffness demonstrated the greatest diagnostic performance for indicating the presence of portal hypertension (area-under-the-ROC-curve [AUROC]=0.98 and 0.96, respectively). The APRI and FIB-4 scores also demonstrated good diagnostic performance (AUROC=0.87 and 0.88, respectively).
MRI-derived measures of liver and spleen stiffness as well as laboratory-based APRI and FIB-4 scores are highly associated with imaging findings of portal hypertension in children and young adults with AILD and thus might be useful for predicting portal hypertension impending onset and directing personalized patient management.
原发性硬化性胆管炎、自身免疫性肝炎和自身免疫性硬化性胆管炎是慢性进行性自身免疫性肝病（AILD）的几种形式，可影响儿童群体。
确定基于定量MRI和实验室的生物标志物是否与患有AILD的儿童和年轻人门静脉高压的传统影像学表现（放射学门静脉高压）相关。
44例登记在机构注册表中的AILD患者在1.5特斯拉（T）下接受了腹部研究性MRI检查。采用了五种定量MRI技术：肝脏磁共振弹性成像、脾脏磁共振弹性成像、肝脏铁校正T1映射、肝脏T2映射和肝脏扩散加权成像（DWI，以表观扩散系数量化）。使用两个解剖序列记录脾肿大、静脉曲张和腹水情况。我们计算了天冬氨酸转氨酶（AST）与血小板比值指数（APRI）和纤维化-4（FIB-4）评分——基于实验室的肝纤维化生物标志物。我们使用受试者操作特征（ROC）曲线分析来确定定量MRI和实验室生物标志物对提示放射学门静脉高压存在的诊断性能。
23例（52%）患者为男性；平均年龄为15.2±4.0岁。13例（30%）患者有放射学门静脉高压。肝脏和脾脏硬度在提示门静脉高压存在方面表现出最大的诊断性能（ROC曲线下面积[AUROC]分别为0.98和0.96）。APRI和FIB-4评分也表现出良好的诊断性能（AUROC分别为0.87和0.88）。
MRI得出的肝脏和脾脏硬度测量值以及基于实验室的APRI和FIB-4评分与患有AILD的儿童和年轻人门静脉高压的影像学表现高度相关，因此可能有助于预测门静脉高压即将发生，并指导个性化的患者管理。
