Solid organ transplantation for non-TTR hereditary amyloidosis: report from the 1st International Workshop on the Hereditary Renal Amyloidoses.

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.

• Authors: Arie J Stangou, Luisa Lobato, Steven Zeldenrust, Mohamed Rela, Bernard Portmann, Reinhold P Linke, Isabel Conceicao, Gerd Otto, Henryk Wilczek, Ole Suhr, Daniel Azoulay, Gilles Grateau, Maria Picken, John O'Grady, Nigel Heaton, Bo-Goran Ericzon, Merrill D Benson
• Journal: Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis (Amyloid) Vol. 19 Suppl 1 Pg. 81-4 (Jun 2012) ISSN: 1744-2818 [Electronic] England
• PMID: 22540225 (Publication Type: Journal Article)

Chemical References

• Apolipoprotein A-I
• fibrinogen Aalpha
• Fibrinogen

Topics

• Amyloidosis, Familial (metabolism, surgery)
• Apolipoprotein A-I (metabolism)
• Fibrinogen (metabolism)
• Humans
• Liver Transplantation
• Organ Transplantation
• Treatment Outcome