Liver Transplantation for Transthyretin Amyloidosis
2009 (English)In: Recent Advances in Transthyretin Evolution, Structure and Biological Functions / [ed] Dr. Samantha J. Richardson, Dr. Vivian Cody, New York: Springer Berlin/Heidelberg, 2009, 239-260 p.Chapter in book (Other academic)
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.
Place, publisher, year, edition, pages
New York: Springer Berlin/Heidelberg, 2009. 239-260 p.
Transthyretin, Liver transplantation, Heart complication, Amyloidosis
Endocrinology and Diabetes
IdentifiersURN: urn:nbn:se:umu:diva-76163DOI: 10.1007/978-3-642-00646-3_15ISI: 000272520300016ISBN: 978-3-642-00645-6 (Print)ISBN: 978-3-642-00646-3 (Online)OAI: oai:DiVA.org:umu-76163DiVA: diva2:635735