What is TTR amyloid?
Transthyretin amyloidosis is a slowly progressive condition characterized by the buildup of abnormal deposits of a protein called amyloid (amyloidosis) in the body’s organs and tissues.
What is hereditary ATTR?
Hereditary ATTR amyloidosis is caused by a fault or mutation in the transthyretin (TTR) gene which is inherited (i.e. runs in families). The mutation results in an abnormal TTR protein that is unstable and readily misfolds, forming aggregates which deposit as amyloid in various organs and tissues in the body.
What is the typical age range for onset of hereditary ATTR cardiomyopathy?
Most individuals develop cardiac symptoms after age 50 years; cardiac amyloidosis generally presents with restrictive cardiomyopathy.
What are the symptoms of TTR?
It can cause a loss of sensation, tingling, numbness, or pain in the hands and feet (also known as peripheral neuropathy). People with this disease also often have damage to the autonomic nervous system (nerves that affect how organs work), digestive tract, and other vital organs, sometimes including the heart.
How do you treat TTR amyloidosis?
Treatment of Amyloid Cardiomyopathy At Penn Medicine, the treatment of TTR amyloid disease involves disease modification with the FDA approved agents, tafamidis, inotersen and patisiran. Note that these medications act by slowing the progress of amyloid disease but are not a cure.
How is TTR amyloidosis diagnosis?
Tests to diagnose transthyretin amyloidosis include: Electrocardiogram (ECG). Cardiac imaging like echocardiograms, MRIs and positron emission tomography (PET) scans. Bone scan (bone scintigraphy) to check for amyloid deposits.
What is the rarest form of amyloidosis?
Hereditary amyloidosis is a rare type of amyloidosis that is caused by an abnormal gene. There are several abnormal genes that can cause hereditary amyloidosis, but the most common type of hereditary amyloidosis is called ATTR and caused by mutations in the transthyretin (TTR) gene.
Can amyloidosis be reversed?
There is not currently a cure for amyloidosis. The amyloid deposits cannot be directly removed. But there are treatments to stop more of the abnormal proteins being produced and treat your symptoms. These treatments can give your body time to gradually clear the deposits before they build up again.
Who gets amyloidosis most commonly?
Most people diagnosed with amyloidosis are between ages 60 and 70, although earlier onset occurs. Sex. Amyloidosis occurs more commonly in men.
What is the best diet for amyloidosis?
While there’s no clear amyloidosis diet, dietary modifications have helped some people feel better. There isn’t a lot of research in this area, but you should stick to a balanced diet. This includes lean protein, healthy fats, fiber, and fruits and vegetables.