1984
DOI: 10.1080/00365521.1984.12005756
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Familial Amyloidosis with Polyneuropathy

Abstract: Fifty-two patients with a confirmed diagnosis of familial amyloidosis with polyneuropathy living in northern Sweden were investigated. Gastrointestinal symptoms, mainly in the form of changed bowel habits, were found in 47 patients. Symptoms of polyneuropathy of the peripheral nerves usually preceded the gastrointestinal symptoms, but in 12 patients gastrointestinal dysfunction started either before or simultaneously with the neurological symptoms. Steatorrhea was found in 30 patients (58%) and an impaired D-x… Show more

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Cited by 13 publications
(5 citation statements)
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“…Symptoms of peripheral and autonomic neuropathy, typical for FAP patients, were evaluated pre‐ and postoperatively. A modified polyneuropathy disability (PND) score was introduced to assess the neuropathy of the FAP patients (12). On this scale, a score of I indicates sensory disturbances in extremities but preserved walking capacity; a score of II indicates difficulties in walking but without the need for a walking stick; a score of IIIa indicates one stick or one crutch required for walking; a score of IIIb indicates two sticks or two crutches required for walking; and a score of IV indicates a patient confined to a wheelchair or to bed.…”
Section: Methodsmentioning
confidence: 99%
See 1 more Smart Citation
“…Symptoms of peripheral and autonomic neuropathy, typical for FAP patients, were evaluated pre‐ and postoperatively. A modified polyneuropathy disability (PND) score was introduced to assess the neuropathy of the FAP patients (12). On this scale, a score of I indicates sensory disturbances in extremities but preserved walking capacity; a score of II indicates difficulties in walking but without the need for a walking stick; a score of IIIa indicates one stick or one crutch required for walking; a score of IIIb indicates two sticks or two crutches required for walking; and a score of IV indicates a patient confined to a wheelchair or to bed.…”
Section: Methodsmentioning
confidence: 99%
“…A common initial symptom is a painful sensory motor peripheral polyneuropathy that initially affects the lower extremities and later on the upper extremities. The autonomic nervous system is also often affected with symptoms such as constipation, diarrhea, impotence and orthostatic hypotension; and in the later stages, visceral organs such as the gastrointestinal tract, kidneys and heart are involved (10–13). The disease progresses relentlessly, and patients usually die 9–13 years after onset (13,14).…”
Section: Introductionmentioning
confidence: 99%
“…Fat malabsorption measured by faecal fat determination is rarely performed today, but steatorrhoea is a common finding in ATTRv patients with diarrhoea. In fact, it was found in 30 of 47 patients (58%) examined in a Swedish cross-sectional study [50], and in seven of 16 patients under evaluation for liver transplantation [49]. In a study from Switzerland, six of seven ATTRv patients suffering from diarrhoea were found to have steatorrhoea [48].…”
Section: Commentsmentioning
confidence: 99%
“…Systemic amyloidosis is further divided into several types, including light-chain (AL or primary), amyloid A (AA or secondary), dialysis-related, hereditary, and wild-type transthyretin amyloidosis. 3,4 Transthyretin (TTR) amyloidosis relates to the progressive deposition of abnormal TTR protein in organs, resulting from failure of homeostatic mechanisms in wild-type ATTR amyloidosis (ATTRwt) or destabilising mutations in variant ATTR (ATTRv) amyloidosis. 5,6 ATTRwt more specifically refers to the deposition of otherwise normal (wild-type) transthyretin (TTR) in myocardium and other sites; a condition previously known as systemic senile amyloidosis (SSA).…”
Section: Discussionmentioning
confidence: 99%