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

Amyloid. 2012 Jun:19 Suppl 1:81-4. doi: 10.3109/13506129.2012.668503. Epub 2012 Apr 27.

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.

MeSH terms

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

Substances

  • Apolipoprotein A-I
  • fibrinogen Aalpha
  • Fibrinogen