Familial amyloid neuropathy

Familial amyloid neuropathy
ICD-10	E85.1
ICD-9	277.3
MeSH	D028227

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The familial amyloid neuropathies (or familial amyloidotic neuropathies, neuropathic heredofamilial amyloidosis, familial amyloid polyneuropathy) are a rare group of autosomal dominant neuropathies of autonomic and peripheral nerves.

Classification

The precursor proteins are: transthyretin (ATTR, the most commonly implicated protein), apolipoprotein A1, and gelsolin.

Due to the rareness of the other types, it is sometimes associated with transthyretin alone.

"FAP-I" and "FAP-II" are associated with transthyretin. (Senile systemic amyloidosis is also associated with transthyretin.)

"FAP-III" is also known as "Iowa-type", and involves apolipoprotein A1.

"FAP-IV" is also known as "Finnish-type", and involves gelsolin.

Fibrinogen, apolipoprotein A1, and lysozyme are associated with a closely related condition, familial visceral amyloidosis.

Treatment

Liver transplantation has demonstrated moderate improvement in ATTR familial amyloidosis.

External links

• http://cancerweb.ncl.ac.uk/cgi-bin/omd?hereditary+amyloidosis

Amyloidosis Microchapters
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Classification • Primary amyloidosis • Secondary amyloidosis • Familial amyloidosis • Wild-type (senile) amyloidosis • Cardiac amyloidosis • Beta-2 microglobulin related amyloidosis • Gelsolin related amyloidosis • Lysozyme amyloid related amyloidosis • Leucocyte cell-derived chemotaxin 2 related amyloidosis • Fibrinogen A alpha-chain associated amyloidosis
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