Background Transthyretin-associated amyloidosis (ATTR) is the commonest cause of hereditary amyloidosis. Age at onset is 50–60 years with average life-expectancy of 7–14 years. Lumbar spinal stenosis also pre
Familial amyloid polyneuropathy is a dominantly inherited neuropathic form of amyloidosis caused by the hepatic production of a mutant transthyretin (TTR).32-34 Because TTR is produced almost exclusively by the liver, liver transplantation eliminates the underlying cause of the disease: production of an...
The most serious complication of FMF is amyloidosis.Serum amyloid Ais anacute phase reactantthat can deposit in many organs, including the kidney, leading to organ failure. Beforecolchicinetherapy, end-stage renal disease occurred in many FMF patients by the age of 40 years. Oral colchic...
Disease-modifying agentTafamidisLiver transplantationHereditary ATTR amyloidosisFamilial amyloid polyneuropathyAmyloidosis neuropathyCarpal tunnel syndromeCardiomyopathyRed-flag symptom clustersHereditary ATTR (ATTRm) amyloidosis (also called transthyretin-type familial amyloid polyneuropathy [ATTR-FAP]) is an autosomal...