|4.||Orthostatic Hypotension (Postural Hypotension)
|5.||Neuralgia (Stump Neuralgia)
|1.||Ikeda, Shu-ichi: 21 articles (12/2013 - 11/2002)|
|2.||Ando, Yukio: 19 articles (09/2014 - 11/2002)|
|3.||Sekijima, Yoshiki: 14 articles (09/2015 - 03/2003)|
|4.||Koike, Haruki: 12 articles (12/2013 - 11/2002)|
|5.||Sobue, Gen: 12 articles (12/2013 - 11/2002)|
|6.||Kelly, Jeffery W: 11 articles (12/2013 - 04/2002)|
|7.||Ikeda, Shu-Ichi: 11 articles (09/2013 - 03/2003)|
|8.||Conceição, Isabel: 9 articles (12/2015 - 06/2002)|
|9.||Suhr, Ole B: 9 articles (01/2015 - 09/2002)|
|10.||Planté-Bordeneuve, Violaine: 8 articles (11/2015 - 09/2008)|
01/01/1996 - "We have analysed the structure, binding properties, stability and amyloidogenicity of particular transthyretin (TTR) mutations-TTR Met30 and TTR Pro55, both associated with familial amyloid polyneuropathy, and TTR Met119, a non-pathogenic TTR mutation with apparent protective effects on the amyloidogenicity of the Met30 mutation. "
06/01/2014 - "Literature searches were conducted on the topics of transthyretin, familial amyloid polyneuropathy and clinical trials, using PubMed, the United States clinical trials directory, pharmaceutical company websites and news reports. "
01/01/2014 - "Epidemiology of transthyretin-associated familial amyloid polyneuropathy in the Majorcan area: Son Llàtzer Hospital descriptive study."
02/01/2012 - "The objective of this study was to elucidate the natural history of late-onset transthyretin Val30Met-associated familial amyloid polyneuropathy (FAP ATTR Val30Met) in non-endemic areas. "
12/01/2010 - "The purpose of this study was to evaluate cardiac MRI features in a group of patients with transthyretin familial amyloid polyneuropathy (FAP). "
|2.||Amyloid (Amyloid Fibrils)IBA
06/01/2008 - "In humans, the T119M-TTR variant has been shown to be protective against familial amyloid polyneuropathy, a TTR amyloid disease, through kinetic stabilization of the unliganded tetrameric structure. "
12/25/2013 - "International randomized, double-blind, placebo-controlled study conducted among 130 patients with familial amyloid polyneuropathy exhibiting clinically detectable peripheral or autonomic neuropathy at amyloid centers in Sweden (Umeå), Italy (Pavia), Japan (Matsumoto and Kumamoto), England (London), and the United States (Boston, Massachusetts; New York, New York; and Rochester, Minnesota) from 2006 through 2012. "
03/01/2013 - "In this study we tested the hypothesis that ATTR amyloid in the carpal tunnel ligament is most commonly of wildtype origin in a Caucasian population without endemic background of familial amyloid polyneuropathy. "
02/01/2016 - "Left atrium can be involved by amyloid deposition in familial amyloid polyneuropathy (FAP). "
01/01/2014 - "Familial amyloid polyneuropathy is a group of autosomal dominant disorders characterized by extracellular amyloid deposition in several target organs. "
08/01/2014 - "Tafamidis: a review of its use in familial amyloid polyneuropathy."
08/10/2012 - "Tafamidis is the first and currently the only medication approved to treat TTR familial amyloid polyneuropathy. "
06/01/2015 - "The transthyretin (TTR) stabilizer, tafamidis, has demonstrated efficacy and safety in the treatment of TTR familial amyloid polyneuropathy (20 mg day(-1) ). "
12/01/2014 - "Post hoc analysis of nutritional status in patients with transthyretin familial amyloid polyneuropathy: impact of tafamidis."
12/01/2013 - "Effect on disability and safety of Tafamidis in late onset of Met30 transthyretin familial amyloid polyneuropathy."
|4.||Diflunisal (Dolobid)FDA Link
10/15/2014 - "Efficacy of diflunisal on autonomic dysfunction of late-onset familial amyloid polyneuropathy (TTR Val30Met) in a Japanese endemic area."
05/15/2014 - "The systematic study of diflunisal+nicotinamide mixtures presented in this work is of particular interest due to the relevance of diflunisal, both as a non-steroidal anti-inflammatory drug and also due to the potentiality of orally administrated diflunisal in familial amyloid polyneuropathy."
10/15/2014 - "To evaluate the long-term efficacy and safety of diflunisal in late-onset familial amyloid polyneuropathy (FAP) in a Japanese endemic area. "
12/25/2013 - "Among patients with familial amyloid polyneuropathy, the use of diflunisal compared with placebo for 2 years reduced the rate of progression of neurological impairment and preserved quality of life. "
12/25/2013 - "Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial."
01/01/2006 - "DOPS improved orthostatic hypotension in patients with familial amyloid polyneuropathy, congenital deficiency of dopamine beta hydroxylase, pure autonomic failure and multiple system atrophy. "
08/01/1979 - "In order to evaluate the involvement of the peripheral autonomic nervous system in the pathogenesis of type 1 familial amyloid polyneuropathy, the urinary excretion rates of catecholamines and serum dopamine-beta-hydroxylase (DB/) activity were examined in 22 patients at various clinical stages. "
01/01/1997 - "This study attempted to assess myocardial sympathetic innervation using iodine-123 (I-123) metaidobenzylguanidine (MIBG) imaging in patients with familial amyloid polyneuropathy. "
11/01/2013 - "Letter by Minutoli et al regarding article, "Reduced myocardial 123-iodine meta-iodobenzylguanidine uptake: a prognostic marker in familial amyloid polyneuropathy"."
10/01/1995 - "[123I-MIBG myocardial SPECT in two patients with familial amyloid polyneuropathy]."
01/01/1997 - "We performed M-mode, two-dimensional and Doppler echocardiography and I-123 MIBG, Tl-201 and Tc-99m PYP imaging of the heart in 12 patients with familial amyloid polyneuropathy and biopsy-proved cardiac amyloidosis. "
11/01/1997 - "In MIBG myocardial images, 2 patients with familial amyloid polyneuropathy (FAP) showed complete or partial defect and the other 2 with primary amyloidosis had normal myocardial uptake of MIBG. "
|7.||Complementary DNA (cDNA)IBA
08/01/1990 - "The neuropathological changes were examined in 2 cases of type I familial amyloid polyneuropathy (FAP), confirmed by a genetic study with human transthyretin (prealbumin) cDNA. "
01/01/1993 - "Direct sequence analysis of a DNA fragment spanning codon 187 of plasma gelsolin complementary DNA and restriction analysis using a modified polymerase chain reaction demonstrated a single base substitution, guanine to adenine, at nucleotide position 654, which is identical to the mutation in Finnish familial amyloid polyneuropathy type IV. This strongly suggests that the mutation causes the familial amyloid polyneuropathy type IV phenotype regardless of ethnic background."
|8.||Small Interfering RNA (siRNA)IBA
|9.||Genetic Markers (Genetic Marker)IBA
03/01/2003 - "To evaluate the therapeutic efficacy of liver transplantation in patients with ATTR Val30Met familial amyloid polyneuropathy (FAP), were repeatedly examined the neurophysiological function of peripheral nerves in nine patients. "
10/01/1996 - "Therefore, it is thought that liver transplantation is an effective treatment for severe forms of familial amyloid polyneuropathy."
05/08/2015 - "[Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years]."
01/01/2015 - "This paper aims to review the morphological and functional characteristics of patients affected by familial amyloid polyneuropathy (FAP), with greater focus on type I and its progression after liver transplantation. "
07/01/2014 - "[Familial amyloid polyneuropathy: liver transplantation as first-line therapy]."
09/01/2014 - "The 10 cases described in this study are a good example of comorbid physical and mental symptoms occurring after transplant in patients with familial amyloid polyneuropathy. "
10/01/2015 - "This case suggests that an ABO-incompatible living-donor liver transplant may provide greater opportunities for familial amyloid polyneuropathy patients. "
10/01/2015 - "Therefore, before deterioration of digestive function, liver transplants should be considered for familial amyloid polyneuropathy. "
10/01/2015 - "Few cases of ABO-incompatible living-donor liver transplant for familial amyloid polyneuropathy exist. "
10/01/2015 - "Liver transplant is a treatment for familial amyloid polyneuropathy. "
|3.||Transplantation (Transplant Recipients)
06/01/2003 - "Transplantation offers the only cure for the genetic defect that causes familial amyloid polyneuropathy, appears to result in subjective and objective improvement in neurological function, and eliminates the mortality associated with the disease. "
05/01/2015 - "In 1995 Furtado et al performed the first domino transplantation using a donor liver with familial amyloid polyneuropathy (FAP), thereby increasing the pool of donors. "
08/01/2008 - "Ocular manifestations in liver transplant recipients with familial amyloid polyneuropathy."
10/01/2005 - "After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. "
09/01/2002 - "Long-term follow-up of survival of liver transplant recipients with familial amyloid polyneuropathy (Portuguese type)."
06/01/2015 - "Recent favorable outcomes in clinical trials for cancers and genetic diseases such as familial amyloid polyneuropathy and Duchenne muscular dystrophy indicate high clinical potency of oligonucleotide therapeutics. "
10/01/2015 - "Expanding knowledge of the molecular underpinnings and therapeutics of transthyretin familial amyloid polyneuropathy have provided impetus to molecular-specific phenotype characterization, natural history studies, and target-based therapeutic interventions. "
10/01/2015 - "To highlight the advances in the knowledge of the clinical features, diagnostic techniques, clinimetrics, and therapeutics of transthyretin familial amyloid polyneuropathy. "
04/01/1999 - "Cardiac sympathetic denervation in familial amyloid polyneuropathy assessed by iodine-123 metaiodobenzylguanidine scintigraphy and heart rate variability."
09/01/2013 - "We aimed to study the long-term prognostic value of myocardial sympathetic denervation detected by MIBG imaging in transthyretin familial amyloid polyneuropathy. "
09/01/2013 - "Cardiac sympathetic denervation as assessed by MIBG imaging is a useful prognostic marker in transthyretin familial amyloid polyneuropathy."