Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Clin Imaging. 2022 Sep;89:147-154. doi: 10.1016/j.clinimag.2022.06.022. Epub 2022 Jul 8.
Both congestive (patients post-Fontan hepatopathy) and congenital (patients with ARPKD) disease can lead to hepatic fibrosis and portal hypertension with eventual development of splenomegaly. We investigated liver and spleen stiffness as measured by MRE between post-Fontan, ARPKD patients and controls independent of organ volume.
Our study included 122 subjects (70 Fontan patients, 14 ARPKD patients, and 38 controls). The mean MRE liver and spleen stiffness values of Fontan patients and patients with ARPKD were compared to controls. Similarly, the liver and spleen volumes of the Fontan patients and patients with ARPKD were then compared to the volumes of controls.
Post-Fontan and ARPKD patients, mean liver stiffness, mean liver volume as well as mean spleen stiffness and mean spleen volume were higher than mean liver stiffness, mean liver volume, mean spleen stiffness, and mean spleen volume of controls. While liver stiffness correlated to liver volume in controls, we found no correlation between stiffness and volume in either Fontan or ARPKD patients, which indicates MRE's ability to act as an independent biomarker. However, these findings are not true in the spleen, where there is significant association between volume and stiffness in patients with ARPKD, but not in Fontan patients or controls.
Liver and spleen stiffness and volumes are significantly different among Fontan patients, ARPKD patients, and controls. Our findings suggest that beyond diagnosing fibrosis, MRE cut-off values could be disease-specific since not only the severity but the underlying pathology causing organ congestion or fibrosis influences MRE results.
充血性心力衰竭（Fontan 肝病史患者）和先天性疾病（ARPKD 患者）均可导致肝纤维化和门静脉高压，最终导致脾肿大。我们研究了磁共振弹性成像（MRE）测量的 Fontan 后患者、ARPKD 患者和对照组之间的肝脏和脾脏硬度，独立于器官体积。
我们的研究包括 122 名受试者（70 名 Fontan 患者、14 名 ARPKD 患者和 38 名对照组）。比较 Fontan 患者和 ARPKD 患者的平均 MRE 肝脏和脾脏硬度值与对照组。同样，Fontan 患者和 ARPKD 患者的肝脏和脾脏体积与对照组的体积进行了比较。
Fontan 后和 ARPKD 患者的平均肝脏硬度、平均肝脏体积以及平均脾脏硬度和平均脾脏体积均高于对照组的平均肝脏硬度、平均肝脏体积、平均脾脏硬度和平均脾脏体积。虽然在对照组中肝脏硬度与肝脏体积相关，但我们发现 Fontan 或 ARPKD 患者的硬度与体积之间没有相关性，这表明 MRE 可以作为独立的生物标志物。然而，这些发现不适用于脾脏，在 ARPKD 患者中，脾脏体积和硬度之间存在显著相关性，但在 Fontan 患者或对照组中则没有。
Fontan 患者、ARPKD 患者和对照组之间的肝脏和脾脏硬度和体积存在显著差异。我们的研究结果表明，除了诊断纤维化之外，MRE 截止值可能是疾病特异性的，因为不仅是严重程度，而且导致器官充血或纤维化的潜在病理也会影响 MRE 结果。
