Familial amyloid neuropathy

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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.[1]

"FAP-I" and "FAP-II" are associated with transthyretin.[2] (Senile systemic amyloidosis is also associated with transthyretin.)

"FAP-III" is also known as "Iowa-type", and involves apolipoprotein A1.[2]

"FAP-IV" is also known as "Finnish-type", and involves gelsolin.[3]

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.[4]

References

  1. Delahaye N, Rouzet F, Sarda L; et al. (2006). "Impact of liver transplantation on cardiac autonomic denervation in familial amyloid polyneuropathy". Medicine (Baltimore). 85 (4): 229–38. doi:10.1097/01.md.0000232559.22098.c3. PMID 16862048. Unknown parameter |month= ignored (help)
  2. 2.0 2.1 "Amyloid".
  3. Akiya S, Nishio Y, Ibi K; et al. (1996). "Lattice corneal dystrophy type II associated with familial amyloid polyneuropathy type IV". Ophthalmology. 103 (7): 1106–10. PMID 8684801. Unknown parameter |month= ignored (help)
  4. "BU". Text " Amyloid Treatment & Research Program " ignored (help); Text " Doctors " ignored (help); Text " ATTR Famililial Amyloidosis " ignored (help); Text " Clinical Features " ignored (help)

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Amyloidosis Microchapters

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Overview

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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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