1. Solid organ transplantation for non-TTR hereditary amyloidosis: report from the 1st International Workshop on the Hereditary Renal Amyloidoses
- Author
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Reinhold P. Linke, Steven R. Zeldenrust, Arie J. Stangou, Bernard Portmann, Maria M. Picken, Bo Goran Ericzon, John O'Grady, Gerd Otto, Luísa Lobato, Henryk Wilczek, Gilles Grateau, Mohamed Rela, Ole B. Suhr, Daniel Azoulay, Nigel Heaton, Isabel Conceição, and Merrill D. Benson
- Subjects
medicine.medical_specialty ,Pathology ,Systemic disease ,medicine.medical_treatment ,Liver transplantation ,Organ transplantation ,Renal amyloidosis ,Internal Medicine ,Medicine ,Humans ,biology ,Apolipoprotein A-I ,business.industry ,Amyloidosis ,Fibrinogen ,Organ Transplantation ,medicine.disease ,Liver Transplantation ,Transplantation ,Transthyretin ,Treatment Outcome ,Immunology ,biology.protein ,business ,Nephrotic syndrome ,Amyloidosis, Familial - Abstract
Fibrinogen A α-chain (AFib) and apolipoprotein AI (AApoAI) amyloidosis due to variants in the AFib and ApoAI genes are the most common types of hereditary amyloidosis in Europe and the United States. Liver is the exclusive source of the aberrant amyloidogenic protein in AFib and responsible for supplying approximately half of the circulating variant ApoAI. Nephrotic syndrome and renal impairment due to renal amyloidosis are common disease manifestations; however, recent research provides evidence to support a more diverse and systemic disease phenotype, which in turn has implications in the management of the hereditary amyloidoses with solid organ transplantation and, in particular, liver transplantation.
- Published
- 2012