Fast Five Quiz: Presentation and Diagnosis of Hereditary (Familial) Amyloid Transthyretin-Related (hATTR) Amyloidosis

Jefferson R. Roberts, MD


July 11, 2019

ATTR is typically fatal within 7-12 years from the onset of symptoms. Death is most often due to cardiac dysfunction, infection, or cachexia. In areas where it is endemic, clinical onset often occurs before the age of 40 years and includes progressive sensorimotor and autonomic neuropathy, which lead to cachexia and eventually death. Cardiac, renal, and ocular involvement are also common.

In nonendemic areas, as well as in endemic regions of Sweden, disease-related symptoms tend to begin later in life in people aged 50 years and older. Symptoms involving sensory and motor neuropathy of the upper and lower extremities are typical and are associated with mild autonomic symptoms.

Learn more about prognosis.


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