6533b85dfe1ef96bd12be871
RESEARCH PRODUCT
Solid organ transplantation for non-TTR hereditary amyloidosis: report from the 1st International Workshop on the Hereditary Renal Amyloidoses.
Reinhold P. LinkeSteven R. ZeldenrustArie J. StangouBernard PortmannMaria M. PickenBo Goran EriczonJohn O'gradyGerd OttoLuísa LobatoHenryk WilczekGilles GrateauMohamed RelaOle B. SuhrDaniel AzoulayNigel HeatonIsabel ConceiçãoMerrill D. Bensonsubject
medicine.medical_specialtyPathologySystemic diseasemedicine.medical_treatmentLiver transplantationOrgan transplantationRenal amyloidosisInternal MedicineMedicineHumansbiologyApolipoprotein A-Ibusiness.industryAmyloidosisFibrinogenOrgan Transplantationmedicine.diseaseLiver TransplantationTransplantationTransthyretinTreatment OutcomeImmunologybiology.proteinbusinessNephrotic syndromeAmyloidosis Familialdescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2012-04-27 | Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis |