Abstract
Wilson’s disease (WD) is an inherited disorder of copper metabolism that effects liver and brain function. Liver involvement presents as either acute liver failure or chronic hepatitis that may eventually progress to cirrhosis. Systematic data on the use of liver stiffness measurement (LSM) in WD are scarce due to the low prevalence of the disease. Pilot studies and case reports indicate the diagnostic potential of LSM for the detection of advanced liver involvement and monitoring of therapy efficacy. There are first indications that an elevated LS seems to be an early sign of WD. In addition, LS decreases in response to copper-eliminating therapy.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Ferenci P, Czlonkowska A, Stremmel W, Houwen R, Rosenberg W, Schilsky M, et al. EASL Clinical Practice Guidelines: Wilson’s disease. J Hepatol 2012;56(3):671–85. https://doi.org/10.1016/j.jhep.2011.11.007.
Dietrich CF, Bamber J, Berzigotti A, Bota S, Cantisani V, Castera L, et al. EFSUMB guidelines and recommendations on the clinical use of liver ultrasound elastography, update 2017 (long version). Ultraschall Med. 2017;38(4):e16–47.
Bruha R, Marecek Z, Pospisilova L, Nevsimalova S, Vitek L, Martasek P, et al. Long-term follow-up of Wilson disease: natural history, treatment, mutations analysis and phenotypic correlation. Liver Int. 2010;31(1):83–91.
Huster D. Wilson disease. Best Pract Res Clin Gastroenterol. 2010;24(5):531–9.
Stremmel W, Merle U, Weiskirchen R. Clinical features of Wilson disease. Ann Transl Med. 2019;7(Suppl 2):S61.
Karlas T, Pfrepper C, Rosendahl J, Benckert C, Wittekind C, Jonas S, et al. Acoustic radiation force impulse (ARFI) elastography in acute liver failure: necrosis mimics cirrhosis. Z Gastroenterol. 2011;49(04):443–8.
Seo YS, Lee KG, Jung ES, An H, Park S, Keum B, et al. Dynamic changes in liver stiffness during the course of acute hepatitis A. Scand J Gastroenterol. 2010;45(4):449–56.
Mueller S, Sandrin L. Liver stiffness: a novel parameter for the diagnosis of liver disease. Hepat Med. 2010;2:49–67.
Isabelle Mohr KHW. Biochemical markers for the diagnosis and monitoring of Wilson disease. Clin Biochem Rev. 2019;40(2):59–77.
Karlas T, Hempel M, Tröltzsch M, Huster D, Günther P, Tenckhoff H, et al. Non-invasive evaluation of hepatic manifestation in Wilson disease with transient elastography, ARFI, and different fibrosis scores. Scand J Gastroenterol. 2012;47(11):1353–61.
Sini M, Sorbello O, Civolani A, Liggi M, Demelia L. Non-invasive assessment of hepatic fibrosis in a series of patients with Wilson’s disease. Dig Liver Dis. 2012;44(6):487–91.
Stefanescu AC, Pop TL, Stefanescu H, Miu N. Transient elastography of the liver in children with Wilson’s disease: preliminary results. J Clin Ultrasound. 2015;44(2):65–71.
Singh S, Fujii LL, Murad MH, Wang Z, Asrani SK, Ehman RL, et al. Liver stiffness is associated with risk of decompensation, liver cancer, and death in patients with chronic liver diseases: a systematic review and meta-analysis. Clin Gastroenterol Hepatol. 2013;11(12):1573–84.e2.
Mueller S. Personal observation. 2019.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2020 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Karlas, T. (2020). Liver Stiffness in Patients with Wilson’s Disease. In: Mueller, S. (eds) Liver Elastography. Springer, Cham. https://doi.org/10.1007/978-3-030-40542-7_20
Download citation
DOI: https://doi.org/10.1007/978-3-030-40542-7_20
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-40541-0
Online ISBN: 978-3-030-40542-7
eBook Packages: MedicineMedicine (R0)