Liver Transplantation for Transthyretin Amyloidosis View Full Text


Ontology type: schema:Chapter     


Chapter Info

DATE

2009-07-29

AUTHORS

Bo-Göran Ericzon , Erik Lundgren , Ole B. Suhr

ABSTRACT

Liver transplantation has until now proved to be the only treatment available that halts the progression of hereditary transthyretin (TTR) associated amyloidosis. The rationale behind the procedure is to replace the liver producing variant TTR with one that produces wild type TTR only, and thereby cease the production of amyloidogenic TTR (ATTR). Even though the transplantation does not improve the patient's symptoms, the progression of the disease comes to a halt for a majority of patients. However, unforeseen complications after the transplantation have emerged, in particular a continuous amyloid formation in the heart observed in non-ATTR Val30Met mutations. Thus, combined liver and heart transplantation has been performed in selected cases.Since the ATTR liver functions normally apart from a synthesis of the variant TTR, utilisation of ATTR-amyloid patients' livers for transplantation of liver disease patients has been performed. In a few patients, development of amyloid disease has been reported, but the procedure remains an important source of organs, especially for patients with hepatocellular cancer. More... »

PAGES

239-260

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-3-642-00646-3_15

DOI

http://dx.doi.org/10.1007/978-3-642-00646-3_15

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1009502106


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