Genetic amyloidosis

recent advances.

Merrill Benson, M. R. Wallace

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

The hereditary amyloidoses are usually associated with peripheral neuropathy and, for this reason, they have been called familial amyloidotic polyneuropathy (FAP). The neuropathy usually involves both the peripheral sensory motor and autonomic nervous systems, resulting in not only loss of sensation and motor function but also bowel dysfunction and orthostatic hypotension. In addition, most of the FAP syndromes involve other organ systems, in particular the renal and cardiac systems. Renal amyloidosis usually presents as nephrotic syndrome and later terminates in azotemia. Cardiac amyloidosis may present as congestive heart failure or arrhythmia. Whereas the neuropathy of hereditary amyloidosis may be the most prominent clinical feature, it is renal or cardiac disease that often dictates the length of survival.

Original languageEnglish
Pages (from-to)129-137
Number of pages9
JournalAdvances in nephrology from the Necker Hospital
Volume18
StatePublished - 1989

Fingerprint

Amyloidosis
Familial Amyloidosis
Polyneuropathies
Kidney
Azotemia
Orthostatic Hypotension
Autonomic Nervous System
Nephrotic Syndrome
Peripheral Nervous System Diseases
Cardiac Arrhythmias
Heart Diseases
Heart Failure

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Genetic amyloidosis : recent advances. / Benson, Merrill; Wallace, M. R.

In: Advances in nephrology from the Necker Hospital, Vol. 18, 1989, p. 129-137.

Research output: Contribution to journalArticle

@article{2d27731a82984ccb90098cffd899dd4e,
title = "Genetic amyloidosis: recent advances.",
abstract = "The hereditary amyloidoses are usually associated with peripheral neuropathy and, for this reason, they have been called familial amyloidotic polyneuropathy (FAP). The neuropathy usually involves both the peripheral sensory motor and autonomic nervous systems, resulting in not only loss of sensation and motor function but also bowel dysfunction and orthostatic hypotension. In addition, most of the FAP syndromes involve other organ systems, in particular the renal and cardiac systems. Renal amyloidosis usually presents as nephrotic syndrome and later terminates in azotemia. Cardiac amyloidosis may present as congestive heart failure or arrhythmia. Whereas the neuropathy of hereditary amyloidosis may be the most prominent clinical feature, it is renal or cardiac disease that often dictates the length of survival.",
author = "Merrill Benson and Wallace, {M. R.}",
year = "1989",
language = "English",
volume = "18",
pages = "129--137",
journal = "Advances in nephrology from the Necker Hospital",
issn = "0084-5957",
publisher = "Mosby Inc.",

}

TY - JOUR

T1 - Genetic amyloidosis

T2 - recent advances.

AU - Benson, Merrill

AU - Wallace, M. R.

PY - 1989

Y1 - 1989

N2 - The hereditary amyloidoses are usually associated with peripheral neuropathy and, for this reason, they have been called familial amyloidotic polyneuropathy (FAP). The neuropathy usually involves both the peripheral sensory motor and autonomic nervous systems, resulting in not only loss of sensation and motor function but also bowel dysfunction and orthostatic hypotension. In addition, most of the FAP syndromes involve other organ systems, in particular the renal and cardiac systems. Renal amyloidosis usually presents as nephrotic syndrome and later terminates in azotemia. Cardiac amyloidosis may present as congestive heart failure or arrhythmia. Whereas the neuropathy of hereditary amyloidosis may be the most prominent clinical feature, it is renal or cardiac disease that often dictates the length of survival.

AB - The hereditary amyloidoses are usually associated with peripheral neuropathy and, for this reason, they have been called familial amyloidotic polyneuropathy (FAP). The neuropathy usually involves both the peripheral sensory motor and autonomic nervous systems, resulting in not only loss of sensation and motor function but also bowel dysfunction and orthostatic hypotension. In addition, most of the FAP syndromes involve other organ systems, in particular the renal and cardiac systems. Renal amyloidosis usually presents as nephrotic syndrome and later terminates in azotemia. Cardiac amyloidosis may present as congestive heart failure or arrhythmia. Whereas the neuropathy of hereditary amyloidosis may be the most prominent clinical feature, it is renal or cardiac disease that often dictates the length of survival.

UR - http://www.scopus.com/inward/record.url?scp=0024487213&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0024487213&partnerID=8YFLogxK

M3 - Article

VL - 18

SP - 129

EP - 137

JO - Advances in nephrology from the Necker Hospital

JF - Advances in nephrology from the Necker Hospital

SN - 0084-5957

ER -