1,078 results match your criteria Amyloid [Journal]


Commentary to Isabel Conceição et al. early diagnosis through targeted follow-up of identified carriers of TTR gene mutations.

Authors:
Ole B Suhr

Amyloid 2019 Feb 26:1-2. Epub 2019 Feb 26.

a Department of Public Health and Clinical Medicine , Umeå University , Umeå , Sweden.

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http://dx.doi.org/10.1080/13506129.2018.1558051DOI Listing
February 2019

Early diagnosis of ATTR amyloidosis through targeted follow-up of identified carriers of TTR gene mutations.

Amyloid 2019 Feb 22:1-7. Epub 2019 Feb 22.

k Department of Neurology , Kumamoto University , Kumamoto , Japan.

Diagnosis in the early stages of hereditary transthyretin (ATTR) amyloidosis is imperative to support timely treatment to prevent or delay disease progression. Genetic testing in the setting of genetic counselling enables identification of carriers of a TTR gene mutation who are therefore at risk of developing TTR-associated disease. Knowledge of different genotypes and how they manifest in symptomatic disease should facilitate development of a structured and targeted approach to enable diagnosis of symptomatic disease in ATTR amyloidosis mutation carriers on the first manifestation of the earliest detectable sign or symptom. Read More

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http://dx.doi.org/10.1080/13506129.2018.1556156DOI Listing
February 2019
1 Read

Electrochemical skin conductance in hereditary amyloidosis related to transthyretin V30M - a promising tool to assess treatment efficacy?

Amyloid 2018 Dec;25(4):267-268

a Departamento de Neurociências , CHLN- Hospital Santa Maria, Lisboa, Portugal; Faculdade de Medicina, Universidade de Lisboa, Instituto de Medicina Molecular , Portugal.

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http://dx.doi.org/10.1080/13506129.2018.1545639DOI Listing
December 2018

Cell assay for the identification of amyloid inhibitors in systemic AA amyloidosis.

Amyloid 2019 Feb 9:1-10. Epub 2019 Feb 9.

a Institute of Protein Biochemistry, Ulm University , Ulm , Germany.

Systemic AA amyloidosis is still, up to this day, a life-threatening complication of chronic inflammatory diseases. Despite the success of anti-inflammatory treatment, the prognosis of some AA patients is still poor, which is why therapies directed at the amyloidogenic pathway in AA amyloidosis are being sought after. The cell culture model of amyloid formation from serum amyloid A1 (SAA1) protein remodels crucial features of AA amyloid deposit formation in vivo. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2019.1
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http://dx.doi.org/10.1080/13506129.2019.1568978DOI Listing
February 2019
7 Reads

Electrophysiological demyelinating features in hereditary ATTR amyloidosis.

Amyloid 2019 Jan 26:1-9. Epub 2019 Jan 26.

a Department of Medicine (Neurology and Rheumatology) , Shinshu University School of Medicine , Matsumoto , Japan.

Objective: To elucidate the electrophysiological demyelinating features in patients with hereditary ATTR amyloidosis that may lead to a misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).

Methods: In 102 patients with hereditary ATTR amyloidosis (85 Val30Met and 17 non-Val30Met; 37 and 65 from endemic and non-endemic areas, respectively), results of motor nerve conduction studies (MNCSs) with a 2-Hz low-cut filter in the unilateral ulnar and tibial nerves were retrospectively investigated to assess whether each MNCS parameter demonstrated demyelinating features that fulfil the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic (EFNS/PNS EDX) criteria for CIDP.

Results: Thirteen patients with low compound muscle action potential (CMAP) amplitude in the tibial nerve (0. Read More

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http://dx.doi.org/10.1080/13506129.2018.1564903DOI Listing
January 2019

Hereditary systemic amyloidosis caused by K19T apolipoprotein C-II variant.

Amyloid 2019 Jan 26:1-2. Epub 2019 Jan 26.

i Department of Clinical Therapeutics, Alexandra Hospital National and Kapodistrian , University of Athens , Athens , Greece.

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http://dx.doi.org/10.1080/13506129.2018.1562442DOI Listing
January 2019
1 Read

Transthyretin deposition in the eye in the era of effective therapy for hereditary ATTRV30M amyloidosis.

Amyloid 2019 Jan 24:1-5. Epub 2019 Jan 24.

o Department of Neurology & Neurobiology of Aging , Kanazawa University Graduate School of Medical Science , Kanazawa , Japan.

Background: Ocular abnormalities have been known to occur in hereditary amyloidotic polyneuropathy since the 1950s. While vitreous opacities and scalloped pupils were described early it has become evident that every component of the eye from the conjunctiva to the retinal vasculature can be involved. Reports from the major centres in Japan, Portugal and Sweden, which primarily treat patients with ATTRV30M, have indicated that with the increased longevity seen in patients treated with liver transplantation the frequency of the more severe eye findings, notably vitreous opacities and subsequent glaucoma, are being detected more frequently. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2018.1
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http://dx.doi.org/10.1080/13506129.2018.1554563DOI Listing
January 2019
9 Reads

Efficacy of lenalidomide as salvage therapy for patients with AL amyloidosis.

Amyloid 2018 Dec 20;25(4):234-241. Epub 2019 Jan 20.

a Department of Clinical Therapeutics , National and Kapodistrian University of Athens , Athens , Greece.

We retrospectively evaluated 55 consecutive patients who received at least one dose of lenalidomide for relapsed/refractory AL amyloidosis. Their median age was 63 years; 72% had heart and 75% kidney involvement and 13% were on dialysis; while 20%, 46% and 34% had Mayo stage -1, -2 and -3 disease, respectively. Median time from start of primary therapy to lenalidomide was 15 months (range 2-100) and median number of prior therapies was 1 (range 1-4); 73% of the patients had prior bortezomib and 42% were bortezomib-refractory. Read More

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http://dx.doi.org/10.1080/13506129.2018.1540410DOI Listing
December 2018
4 Reads

A pilot study demonstrating cardiac uptake with 18F-florbetapir PET in AL amyloidosis patients with cardiac involvement.

Amyloid 2018 Dec 19;25(4):247-252. Epub 2019 Jan 19.

a National Amyloidosis Centre, University College London (Royal Free Campus) , London , UK.

F-florbetapir is a promising tracer in amyloidosis. This study evaluates its use in patients with systemic AL amyloidosis (AL) before and after treatment as well as its serial utility in monitoring. Fifteen AL patients with cardiac involvement underwent F-florbetapir PET imaging and three patients underwent repeat imaging after chemotherapy. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2018.1
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http://dx.doi.org/10.1080/13506129.2018.1552852DOI Listing
December 2018
7 Reads

Dialysis associated β2-microglobulin amyloidosis occurs even in the era of high-flux dialyzers.

Amyloid 2018 Dec 19;25(4):265-266. Epub 2019 Jan 19.

e Department of internal medicine, Diakonessenhuis Utrecht , Bosboomstraat 1 , 3582 KE , Utrecht , The Netherlands.

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http://dx.doi.org/10.1080/13506129.2018.1531391DOI Listing
December 2018
1 Read

Sudoscan in the evaluation and follow-up of patients and carriers with TTR mutations: experience from an Italian Centre.

Amyloid 2018 Dec 14;25(4):242-246. Epub 2019 Jan 14.

b Università Cattolica del Sacro Cuore , Roma , Italia.

Objective: To evaluate the utility of Sudoscan as possible marker of disease progression and disease onset in a cohort of hereditary ATTR amyloidosis (hATTR amyloidosis) polyneuropathy patients and carriers.

Patients And Methods: We regularly performed different clinical scales, nerve conductions studies (NCS), and Sudoscan on a cohort of hATTR amyloidosis patients and carriers from a single centre of central Italy, a non-endemic area, in the last 2 years.

Results: About 18 hATTR amyloidosis patients and 8 asymptomatic carriers were enrolled. Read More

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http://dx.doi.org/10.1080/13506129.2018.1545640DOI Listing
December 2018
1 Read

Cause of death analysis and temporal trends in survival after liver transplantation for transthyretin familial amyloid polyneuropathy.

Amyloid 2018 Dec 11;25(4):253-260. Epub 2019 Jan 11.

j Cardiology department , Bichat Claude Bernard Hospital, AP-HP, University of Paris-Sud , Paris , France.

Background: Hereditary transthyretin amyloidosis (ATTR) is a multisystemic disease involving mainly the peripheral nervous system and the heart. Liver transplantation (LT) is the reference treatment for ATTR neuropathy and preoperative detection of high risk patients is crucial. We aimed to document the causes of death of ATTR patients after LT, their temporal trends, and to evaluate whether the available preoperative tools that predict the risk of death after LT for hereditary ATTR amyloidosis matched with these trends. Read More

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http://dx.doi.org/10.1080/13506129.2018.1550061DOI Listing
December 2018
13 Reads

Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee.

Amyloid 2018 Dec 7;25(4):215-219. Epub 2019 Jan 7.

h Department of Immunology, Genetics and Pathology , Uppsala University , Uppsala , Sweden.

The nomenclature committee of the International Society of Amyloidosis (ISA) meets every second year to discuss and formulate recommendations. The conclusions from the discussion at the XVI International Symposium on Amyloidosis in Kumamoto, Japan, 25-29 March 2018 and afterwards are summarized in this Nomenclature Article. From having recommended the use of the designation "amyloid fibril" for in vivo material only, ISA's nomenclature committee now accepts its use more broadly following the international scientific literature. Read More

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http://dx.doi.org/10.1080/13506129.2018.1549825DOI Listing
December 2018
1 Read

Epidemiology of ATTRV30M neuropathy in Cyprus and the modifier effect of complement C1q on the age of disease onset.

Amyloid 2018 Dec 20;25(4):220-226. Epub 2018 Dec 20.

a Department of Neuropathology/Neurology Clinic A , The Cyprus Institute of Neurology & Genetics , Nicosia , Cyprus.

Background: ATTRV30M amyloidosis is a lethal autosomal dominant sensorimotor and autonomic neuropathy caused by amyloid deposition composed of aggregated misfolded TTR monomers with the V30M mutation. The age of onset in patients with ATTRV30M varies in different foci and the mechanism behind it is still unknown.

Methods: The tertiary neurology center following all ATTRV30M patients in Cyprus was used to collect demographic data to estimate; prevalence, incidence, penetrance, anticipation, time from disease onset to diagnosis and transplantation. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2018.1
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http://dx.doi.org/10.1080/13506129.2018.1534731DOI Listing
December 2018
9 Reads

Haematological response and overall survival in two consecutive Dutch patient cohorts with AL amyloidosis diagnosed between 2008 and 2016.

Amyloid 2018 Dec 4;25(4):227-233. Epub 2018 Dec 4.

a Department of Haematology , Utrecht University, University Medical Centre Utrecht , Utrecht , The Netherlands.

Background: Although survival has improved in recent decades, the short-term prognosis of patients with immunoglobulin light chain (AL) amyloidosis remains grim. We aimed to assess overall survival (OS) of AL amyloidosis patients by comparing cohorts in two consecutive time periods.

Methods: Data were collected and compared on 126 patients from two tertiary referral centres in The Netherlands during the time periods 2008-2012 and 2013-2016. Read More

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http://dx.doi.org/10.1080/13506129.2018.1536043DOI Listing
December 2018
15 Reads

Polymorph-specific distribution of binding sites determines thioflavin-T fluorescence intensity in α-synuclein fibrils.

Amyloid 2018 Sep;25(3):189-196

a Nanobiophysics, MESA + Institute for Nanotechnology, University of Twente , Enschede , The Netherlands.

Thioflavin-T (ThT) is the most commonly used fluorescent dye for following amyloid formation semi-quantitatively in vitro, specifically probing the fibrillar cross-β-sheet content. In recent years, structural polymorphism of amyloid fibrils has been shown to be an important aspect of amyloid formation, both in vitro and in neurodegenerative diseases. Therefore, understanding ThT-amyloid interactions in the context of structural polymorphism of amyloids is necessary for correct interpretation of ThT fluorescence data. Read More

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http://dx.doi.org/10.1080/13506129.2018.1517736DOI Listing
September 2018
1 Read

Origin of sporadic late-onset hereditary ATTR Val30Met amyloidosis in Japan.

Amyloid 2018 Sep;25(3):143-147

a Department of Neurology, Graduate School of Medical Sciences , Kumamoto University , Kumamoto , Japan.

Hereditary transthyretin (ATTRm) amyloidosis, formerly known as familial amyloid polyneuropathy, is a major type of hereditary systemic amyloidosis, in which the disease is caused by mutant transthyretin (TTR). Although more than 140 different point mutations have been identified in the TTR gene, ATTRm amyloidosis patients with the TTR Val30Met mutation are most frequently found worldwide. Interestingly, the onset age of the ATTR Val30Met amyloidosis is highly varied among countries and regions. Read More

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http://dx.doi.org/10.1080/13506129.2018.1531842DOI Listing
September 2018
4 Reads

Quantitation of Tc-DPD uptake in patients with transthyretin-related cardiac amyloidosis.

Amyloid 2018 Sep;25(3):203-210

a National Amyloidosis Centre , UCL Medical School (Royal Free Campus) , London , UK.

Purpose: Transthyretin (ATTR) amyloidosis is a rare but serious infiltrative disease associated with a wide spectrum of morphologic and functional cardiac involvement. Tc-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD), initially developed as a bone-seeking radiotracer, is remarkably sensitive for imaging cardiac ATTR amyloid deposits. Our aim was to investigate the feasibility and utility of estimating Tc-DPD uptake in myocardial tissue; this has the potential to yield reliable quantitative information on cardiac amyloid burden, which is urgently required to monitor disease progression and response to novel treatments. Read More

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http://dx.doi.org/10.1080/13506129.2018.1520087DOI Listing
September 2018
2 Reads

Diflunisal tolerability in transthyretin cardiac amyloidosis: a single center's experience.

Amyloid 2018 Sep 2;25(3):197-202. Epub 2018 Nov 2.

a Department of Cardiovascular Medicine , Cleveland Clinic Foundation , Cleveland , OH , USA.

Objective: Transthyretin (ATTR) amyloidosis is an under-recognized, progressive disease manifesting as cardiomyopathy and/or polyneuropathy. Diflunisal, a nonsteroidal anti-inflammatory drug (NSAID), has demonstrated transthyretin stabilization in vitro and slowing of polyneuropathy progression in the hereditary ATTR subtype (ATTRm). However, the use of diflunisal has only been described in a small cohort of patients with ATTR cardiac amyloidosis (CA). Read More

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http://dx.doi.org/10.1080/13506129.2018.1519507DOI Listing
September 2018
12 Reads
2.010 Impact Factor

Duodenal amyloid A depositions in a patient with refractory Hodgkin's lymphoma: an old complication in the modern treatment era.

Amyloid 2018 Dec 23;25(4):263-264. Epub 2018 Oct 23.

c Department of Hematology , Hadassah - Hebrew University Medical Center , POB 91120 , Jerusalem 12000 , Israel.

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http://dx.doi.org/10.1080/13506129.2018.1517737DOI Listing
December 2018
5 Reads

Peak V'O is an independent predictor of survival in patients with cardiac amyloidosis.

Amyloid 2018 Sep 7;25(3):167-173. Epub 2018 Sep 7.

a Department of Internal Medicine III, Cardiology , Heidelberg University Hospital , Heidelberg , Germany.

Introduction: Cardiopulmonary exercise testing (CPET) has repeatedly been reported to reliably predict adverse outcomes in different forms of heart failure. However, it has not been elucidated in detail in cardiac amyloidosis (CA). Therefore, we evaluated the predictive value of CPET parameters in patients with CA regarding disease severity and prediction of mortality. Read More

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http://dx.doi.org/10.1080/13506129.2018.1496077DOI Listing
September 2018
7 Reads

Right ventricular involvement in transthyretin amyloidosis.

Amyloid 2018 Sep 7;25(3):160-166. Epub 2018 Sep 7.

a Department of Clinical Physiology , Heart Centre, Umeå University , Umeå , Sweden.

Background: The extent of right ventricular (RV) involvement in transthyretin amyloidosis (ATTR) is unknown.

Objectives: This study sought to establish the degree of RV involvement in ATTR amyloidosis, and compare findings with RV involvement in hypertrophic cardiomyopathy (HCM).

Methods: Forty-two patients with ATTR amyloidosis and echocardiographic evidence of cardiac amyloidosis (cardiac ATTR), 19 ATTR patients with normal left ventricular (LV) wall thickness (non-cardiac ATTR), 25 patients with diagnosed HCM and 30 healthy controls were included in this study. Read More

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http://dx.doi.org/10.1080/13506129.2018.1493989DOI Listing
September 2018
2 Reads

Seven factors predict a delayed diagnosis of cardiac amyloidosis.

Amyloid 2018 Sep 31;25(3):174-179. Epub 2018 Aug 31.

a Division of Cardiovascular Pathology, Department of Pathology , Johns Hopkins University , Baltimore , MD , USA.

Introduction: Diagnostic delay of cardiac amyloidosis (CAm) continues to challenge clinicians. We investigated features associated with delay and ascertained if a diagnostic delay had negative implications for the patient.

Methods: We performed a retrospective chart review identifying 82 subjects with biopsy-proven and mass-spectrometry-identified CAm with clinical and epidemiologic data including first potential symptom of amyloidosis. Read More

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http://dx.doi.org/10.1080/13506129.2018.1498782DOI Listing
September 2018
3 Reads
2.010 Impact Factor

Hereditary transthyretin amyloidosis: baseline characteristics of patients in the NEURO-TTR trial.

Amyloid 2018 Sep 31;25(3):180-188. Epub 2018 Aug 31.

m Division of Hematology, Mayo Clinic , Rochester , Minnesota , USA.

Background: Hereditary transthyretin (ATTRm) amyloidosis is a rare, progressive and fatal disease with a range of clinical manifestations.

Objective: This study comprehensively evaluates disease characteristics in a large, diverse cohort of patients with ATTRm amyloidosis.

Methods: Adult patients (N = 172) with Stage 1 or Stage 2 ATTRm amyloidosis who had polyneuropathy were screened and enrolled across 24 investigative sites and 10 countries in the NEURO-TTR trial ( www. Read More

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http://dx.doi.org/10.1080/13506129.2018.1503593DOI Listing
September 2018
6 Reads

A case of transthyretin amyloidosis with myopathy, neuropathy, and cardiomyopathy resulting from an exceedingly rare mutation transthyretin Ala120Ser (c.418G > T, p.Ala140Ser).

Amyloid 2018 09 24;25(3):211-212. Epub 2018 Jul 24.

f Department of Cardiology , Montefiore Medical Center, Albert Einstein College of Medicine , Bronx , NY , USA.

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http://dx.doi.org/10.1080/13506129.2018.1491398DOI Listing
September 2018
4 Reads

A library of ATTR amyloidosis patient-specific induced pluripotent stem cells for disease modelling and in vitro testing of novel therapeutics.

Amyloid 2018 Sep 21;25(3):148-155. Epub 2018 Jul 21.

a Center for Regenerative Medicine , Boston University School of Medicine , Boston , MA , USA.

Hereditary transthyretin amyloidosis (ATTR amyloidosis) is an autosomal dominant protein-folding disorder caused by over 100 distinct mutations in the transthyretin (TTR) gene. In ATTR amyloidosis, protein secreted from the liver aggregates and forms amyloid fibrils in downstream target organs, chiefly the heart and peripheral nervous system. Few animal models of ATTR amyloidosis exist and none recapitulate the multisystem complexity and clinical variability associated with disease pathogenesis in patients. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2018.1
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http://dx.doi.org/10.1080/13506129.2018.1489228DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319917PMC
September 2018
17 Reads

Patient-reported distress is prevalent in systemic light chain (AL) amyloidosis but not determined by severity of disease.

Amyloid 2018 Jun 21;25(2):129-134. Epub 2018 Jul 21.

a Division of Hematology/Oncology, Department of Medicine, Medical College of Wisconsin , Milwaukee , WI , USA.

We conducted this retrospective study to assess patient-reported distress in light chain (AL) amyloidosis, using the Distress Thermometer (DT) survey at first evaluation at our center. Of 78 patients who completed the survey, 75 scored their distress (distress: <4 - low, 4-6 - moderate, >6 - high). Moderate and high distress were self-reported by 30% and 17% patients, respectively. Read More

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http://dx.doi.org/10.1080/13506129.2018.1486298DOI Listing
June 2018
6 Reads

Outcomes of patients with AL amyloidosis and low serum free light chain levels at diagnosis.

Amyloid 2018 Sep 21;25(3):156-159. Epub 2018 Jul 21.

a Amyloidosis Center , Boston University School of Medicine , Boston , MA , USA.

Serum free light chains (sFLC) are independent prognostic markers of disease in light chain (AL) amyloidosis, and are used in the haematologic response criteria for treatment. However, up to 20% of patients have low sFLCs at diagnosis, with a difference between involved and uninvolved free light chains (dFLC) of less than 50 mg/L, making responses to treatment difficult to evaluate. In order to characterize this distinct subgroup of patients, we retrospectively analyzed 123 AL amyloidosis patients with dFLC <50 mg/L who were diagnosed between 2002 and 2013. Read More

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http://dx.doi.org/10.1080/13506129.2018.1490261DOI Listing
September 2018
5 Reads

Cerebrospinal fluid and vitreous body exposure to orally administered tafamidis in hereditary ATTRV30M (p.TTRV50M) amyloidosis patients.

Amyloid 2018 Jun 11;25(2):120-128. Epub 2018 Jul 11.

a Departments of Chemistry and Molecular Medicine , The Scripps Research Institute , La Jolla , CA , USA.

Hereditary transthyretin (TTR) amyloidosis associated with the TTRV30M (p.TTRV50M) mutation presents predominantly as an axonal polyneuropathy, with variable involvement of other organs. Serious central nervous system (CNS) and eye manifestations, including stroke, dementia, vitreous opacities and glaucoma, have been reported in untreated V30M TTR amyloidosis patients, and in these patients after treatment with liver transplantation (LT). Read More

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http://dx.doi.org/10.1080/13506129.2018.1479249DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6177313PMC
June 2018
4 Reads

Systemic angiopathy and axonopathy in hereditary transthyretin amyloidosis with Ala97Gly (p. Ala117Gly) mutation: a post-mortem analysis.

Amyloid 2018 06 1;25(2):141-142. Epub 2018 Jun 1.

a Department of Neurology , Nagoya University Graduate School of Medicine , Nagoya , Japan.

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http://dx.doi.org/10.1080/13506129.2018.1474734DOI Listing
June 2018
5 Reads

Profile of renal AA amyloidosis in older and younger individuals: a single-centre experience.

Amyloid 2018 Jun 18;25(2):115-119. Epub 2018 May 18.

a Department of Nephrology , Ankara University School of Medicine , Ankara , Turkey.

Objective: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis. Read More

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http://dx.doi.org/10.1080/13506129.2018.1474733DOI Listing
June 2018
27 Reads

MRI feature tracking strain is prognostic for all-cause mortality in AL amyloidosis.

Amyloid 2018 Jun 7;25(2):101-108. Epub 2018 May 7.

a Department of Radiology , Mayo Clinic , Rochester , MN , USA.

Objectives: Cardiac involvement is a major determinate of mortality in light chain (AL) amyloidosis. Cardiac magnetic resonance imaging (MRI) feature tracking (FT) strain is a new method for measuring myocardial strain. This study retrospectively evaluated the association of MRI FT strain with all-cause mortality in AL amyloidosis. Read More

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http://dx.doi.org/10.1080/13506129.2018.1465406DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6405287PMC
June 2018
3 Reads

Utility of the F-Florbetapir positron emission tomography in systemic amyloidosis.

Amyloid 2018 Jun 28;25(2):109-114. Epub 2018 Apr 28.

a Internal Medicine Department , Hospital Universitari Vall d'Hebron , Barcelona , Spain.

Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using F-florbetapir. Read More

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http://dx.doi.org/10.1080/13506129.2018.1467313DOI Listing
June 2018
5 Reads

A functional assay to identify amyloidogenic light chains.

Amyloid 2018 Jun 23;25(2):93-100. Epub 2018 Mar 23.

a Department of Medicine , University of Tennessee Medical Center , Knoxville , TN , USA.

Introduction: Multiple myeloma (MM) and light chain monoclonal gammopathy of undetermined significance (LCMGUS) are plasma cell disorders associated with the secretion of monoclonal free light-chain (LC) proteins. Due to the high concentrations of LC in circulation, both of these populations are at risk for developing LC-associated amyloidosis (AL) - a protein misfolding disease characterized by the deposition of LC protein fibrils in organs and tissues, leading to dysfunction and significant morbidity. At present, accurate identification of subjects at risk for developing amyloidosis is not possible, but with the advent of novel, amyloid-targeted therapies, identification of pre-symptomatic individuals is of clinical import. Read More

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http://dx.doi.org/10.1080/13506129.2018.1456425DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333307PMC
June 2018
8 Reads

Response: Atrial impairment in transthyretin cardiac amyloidosis: an early marker of cardiac involvement and a prognostic factor.

Amyloid 2018 Jun 12;25(2):136. Epub 2018 Mar 12.

a Department of Public Health and Clinical Medicine , Umeå University , Umeå , Sweden.

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http://dx.doi.org/10.1080/13506129.2018.1450241DOI Listing
June 2018
7 Reads

Digoxin use in systemic light-chain (AL) amyloidosis: contra-indicated or cautious use?

Amyloid 2018 Jun 12;25(2):86-92. Epub 2018 Mar 12.

b Department of Cardiovascular Diseases , Mayo Clinic , Rochester , MN , USA.

Aim: Digoxin is considered contraindicated in light-chain (AL) amyloidosis, given reports of increased toxicity published 30-50 years ago. We sought to determine the frequency of digoxin toxicity in patients with AL.

Methods: We identified 107 patients with AL amyloidosis who received digoxin between 2000 and 2015. Read More

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http://dx.doi.org/10.1080/13506129.2018.1449744DOI Listing
June 2018
10 Reads
1 Citation
2.010 Impact Factor

Atrial impairment in transthyretin cardiac amyloidosis: an early marker of cardiac involvement and a prognostic factor.

Amyloid 2018 Jun 9;25(2):135. Epub 2018 Mar 9.

b Department of Biomedical and Dental Sciences and Morphofunctional Imaging , University of Messina , Messina , Italy.

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http://dx.doi.org/10.1080/13506129.2018.1450240DOI Listing
June 2018
4 Reads

Once AL amyloidosis: not always AL amyloidosis.

Amyloid 2018 Jun 8;25(2):139-140. Epub 2018 Mar 8.

b Amyloidosis Center , Boston University School of Medicine and Boston Medical Center , Boston , MA , USA.

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http://dx.doi.org/10.1080/13506129.2018.1449104DOI Listing
June 2018
9 Reads

Bone marrow plasma cell infiltration in light chain amyloidosis: impact on organ involvement and outcome.

Amyloid 2018 Jun 26;25(2):79-85. Epub 2018 Feb 26.

a Amyloidosis and Myeloma Unit, Department of Hematology , Hospital Clínic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona , Barcelona , Spain.

Objectives: Prognosis of immunoglobulin light-chain (AL) amyloidosis depends mainly on the presence of cardiac involvement and the disease burden. A higher bone marrow plasma cell (BMPC) burden has been recognized as an adverse prognostic factor. The aim of our study was to analyze the correlation between the BMPC infiltration, clinical features and outcomes in patients with AL amyloidosis. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2018.1
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http://dx.doi.org/10.1080/13506129.2018.1443439DOI Listing
June 2018
8 Reads

Primary adrenal insufficiency due to hereditary apolipoprotein AI amyloidosis: endocrine involvement beyond hypogonadism.

Amyloid 2018 Jun 15;25(2):75-78. Epub 2018 Feb 15.

a Department of Endocrinology and Nutrition , Hospital Clínic , Barcelona , Spain.

Several mutations in the gene encoding apolipoprotein AI (apoAI) have been described as a cause of familial amyloidosis. Individuals with apoAI-derived (AApoAI) amyloidosis frequently manifest with liver, kidney, laryngeal, skin and myocardial involvement. Although primary hypogonadism (PH) is considered almost pathognomonic of this disease, until now, primary adrenal insufficiency (PAI) has not been described as a common clinical feature. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2018.1
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http://dx.doi.org/10.1080/13506129.2018.1438390DOI Listing
June 2018
23 Reads

Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR).

Amyloid 2018 Mar 9;25(1):62-67. Epub 2018 Feb 9.

a Amyloidosis Center , Boston University School of Medicine , Boston , MA , USA.

Objective: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis.

Patients And Methods: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016.

Results: There were a total of 226 patients with ATTRwt and ATTR V122I amyloidosis evaluated during the specified time frame with 155 and 71 patients in each cohort, respectively. Read More

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http://dx.doi.org/10.1080/13506129.2018.1436048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6157907PMC
March 2018
8 Reads

Normal scores of deep breathing tests: beware of dysrhythmia in transthyretin amyloidosis.

Amyloid 2018 Mar 2;25(1):54-61. Epub 2018 Feb 2.

c Department of Surgical and Perioperative Sciences, Clinical Physiology, Heart Centre , Umeå University , Umeå , Sweden.

Background: The heart rate (HR) response to paced deep breathing (DB) is a common test of cardiac autonomic function, where high heart rate variability (HRV) is considered to reflect normal autonomic function. We evaluated the DB test in patients with hereditary transthyretin amyloid (ATTRm) amyloidosis, where autonomic dysregulation and atrial arrhythmias are common.

Methods: Paced DB was performed during one minute (six breaths/min) in 165 recordings in adult ATTRm amyloidosis patients with the TTR Val30Met mutation, 42 hypertrophic cardiomyopathy (HCM) patients and 211 healthy subjects. Read More

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http://dx.doi.org/10.1080/13506129.2018.1434140DOI Listing
March 2018
8 Reads

Reduced left atrial myocardial deformation irrespective of cavity size: a potential cause for atrial arrhythmia in hereditary transthyretin amyloidosis.

Amyloid 2018 Mar 25;25(1):46-53. Epub 2018 Jan 25.

a Public Health and Clinical Medicine , Umeå University , Umeå , Sweden.

Background: Cardiac amyloidosis (CA) is a myocardial disease and commonly under-diagnosed condition. In CA patients, atrial fibrillation might occur in the absence of left atrial (LA) enlargement.

Objectives: The aim of this study is to assess LA size and function, and its relationship with atrial arrhythmia in patients with hereditary transthyretin amyloidosis (ATTR). Read More

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http://dx.doi.org/10.1080/13506129.2018.1430027DOI Listing
March 2018
13 Reads

Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

Amyloid 2018 Mar 24;25(1):37-45. Epub 2018 Jan 24.

a Department of Medicine V, Amyloidosis Center and Division of Hematology, Oncology and Rheumatology , University of Heidelberg , Heidelberg , Germany.

Background: To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis.

Methods: Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. Read More

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http://dx.doi.org/10.1080/13506129.2018.1429391DOI Listing
March 2018
14 Reads

Psychopathological dimensions in subjects with hereditary ATTR V30M amyloidosis and their relation with life events due to the disease.

Amyloid 2018 Mar 22;25(1):26-36. Epub 2018 Jan 22.

a Serviço de Psiquiatria e Saúde Mental , Centro Hospitalar do Porto , Porto , Portugal.

Background: Chronic physical illness has been associated with emotional distress. Chronic diseases may change usual family patterns with economic, social and family losses. Hereditary ATTR V30M amyloidosis is a rare, fatal inherited systemic amyloidosis, with chronic evolution and beginning in adulthood. Read More

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http://dx.doi.org/10.1080/13506129.2018.1428795DOI Listing
March 2018
8 Reads

Treatment patterns and health care resource utilization among patients with relapsed/refractory systemic light chain amyloidosis.

Amyloid 2018 Mar 5;25(1):1-7. Epub 2018 Jan 5.

e Amyloidosis Center , Boston University School of Medicine and Boston Medical Center , Boston , MA , USA.

Background: Treatment for patients with systemic light chain (AL) amyloidosis remains challenging. Our study aims to describe treatment patterns for both newly diagnosed and relapsed/refractory AL (RRAL) amyloidosis, and to assess clinical outcomes, healthcare costs, and resource utilization during the first year following a diagnosis of RRAL amyloidsis.

Methods: This was a retrospective observational study of adult patients with AL amyloidosis using the US Optum administrative claims data during 1/1/2008 to 6/30/2015. Read More

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http://dx.doi.org/10.1080/13506129.2017.1411796DOI Listing
March 2018
8 Reads

Right ventricular longitudinal strain: a tool for diagnosis and prognosis in light-chain amyloidosis.

Amyloid 2018 Mar 20;25(1):18-25. Epub 2017 Dec 20.

a Cardiology Department , CHU de Poitiers , Poitiers , France.

Objectives: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. Read More

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http://dx.doi.org/10.1080/13506129.2017.1417121DOI Listing
March 2018
7 Reads
2.010 Impact Factor

Gastrointestinal perforation in light chain amyloidosis in the era of novel agent therapy - a case series and review of the literature.

Amyloid 2018 Mar 14;25(1):11-17. Epub 2017 Dec 14.

a Department of Haematology , Hadassah-Hebrew University Medical Center , Jerusalem , Israel.

Gastrointestinal (GI) perforation is remarkably rare in patients with light chain (AL) amyloidosis and has not yet been reported in patients with AL amyloidosis treated with novel agents. Only 24 cases of GI perforation have previously been reported in the setting of AL amyloidosis of which 15 had available information in English. All 15 did not receive novel agent therapy and six died early after experiencing GI perforation. Read More

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http://dx.doi.org/10.1080/13506129.2017.1416350DOI Listing
March 2018
13 Reads