Highlights
- •Liver and spleen stiffness and volumes were significantly different among groups; and can be used with longitudinal disease assessments.
- •Liver and spleen stiffness’ and volumes of patients with ARPKD and Fontan are higher than controls.
- •MRE cut-off values could be disease specific as not only the severity but underlying pathology causing organ congestion influences MRE results.
Abstract
Purpose
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.
Methods
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.
Results
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.
Conclusion
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.
Keywords
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Article info
Publication history
Published online: July 07, 2022
Accepted:
June 28,
2022
Received in revised form:
June 27,
2022
Received:
March 28,
2022
Identification
Copyright
© 2022 Elsevier Inc. All rights reserved.
