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Andrew N Rosenbaum, Omar F Abou Ezzeddine, Martha Grogan, Angela Dispenzieri, Sudhir Kushwaha, Alfredo Clavell, Richard C Daly, Brooks S Edwards
BACKGROUND: The true prevalence of heart failure (HF) due to wild type transthyretin amyloidosis (ATTRwt) is likely underestimated. There is a paucity of data with regard to the management of ATTRwt-related advanced HF and the natural history of extracardiac ATTRwt. METHODS: We conducted a retrospective cohort study of patients undergoing cardiac transplant (HTx) for ATTRwt at a single institution. Comprehensive clinical data, including baseline hemodynamic and echocardiographic characteristics, and posttransplant outcomes were obtained...
April 19, 2018: Transplantation
Avinainder Singh, Hallie I Geller, Kevin M Alexander, Robert F Padera, Richard N Mitchell, Sharmila Dorbala, Jorge J Castillo, Rodney H Falk
No abstract text is available yet for this article.
April 19, 2018: Haematologica
Yale Tung-Chen, Miguel-Ángel Arnau
BACKGROUND: Congestive heart failure (CHF) is a very common condition, especially in the elderly, characterized by dyspnea, orthopnea, nocturnal paroxysmal dyspnea, and peripheral edema. CLINICAL PRESENTATION AND INTERVENTION: We report the case of a 76-year-old with CHF symptoms for the last 3 months. The ECG and transthoracic echocardiogram were suggestive of CHF due to amyloid cardiomyopathy. After cardiac MRI, a positive Congo red staining of subcutaneous fat aspiration and a negative genetic testing for mutant transthyretin, senile amyloid cardiomyopathy (ATTRw) was expected...
April 17, 2018: Acta Clinica Belgica
Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypmann, Constantin E Uhlig, Hartmut H-J Schmidt
No abstract text is available yet for this article.
March 2018: Deutsche Medizinische Wochenschrift
T Wagner, J Page, M Burniston, A Skillen, J C Ross, R Manwani, D McCool, P N Hawkins, Ashutosh D Wechalekar
PURPOSE: 18 F-Florbetapir has been reported to show cardiac uptake in patients with systemic light-chain amyloidosis (AL). This study systematically assessed uptake of 18 F-florbetapir in patients with proven systemic amyloidosis at sites outside the heart. METHODS: Seventeen patients with proven cardiac amyloidosis underwent 18 F-florbetapir PET/CT imaging, 15 with AL and 2 with transthyretin amyloidosis (ATTR). Three patients had repeat scans. All patients had protocolized assessment at the UK National Amyloidosis Centre including imaging with 123 I-serum amyloid P component (SAP)...
April 12, 2018: European Journal of Nuclear Medicine and Molecular Imaging
Mio Matsuoka, Shohei Iijima
SUBJECTS AND METHODS: The subjects comprised 21 patients with esophageal cancer who underwent surgery, without adjuvant chemotherapy. Caloric intake, body weight loss, and biochemical parameters(serum albumin[Alb], hemoglobin[Hb], transthyretin[TTR], and total cholesterol[T-Cho])were measured up to 1 year after surgery, and relationships between all parameters were investigated. RESULTS: The caloric intake dropped to about 87% of base-line intake at 1 month after surgery and recovered to 100% at 1 year after the operation...
March 2018: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Cherie N Dahm, R Frank Cornell, Daniel J Lenihan
Systemic amyloidosis represents a complex group of diseases with a common feature characterized by misfolded autologous proteins depositing into tissues or organs throughout the body. Light chain amyloidosis (AL) and transthyretin (TTR) amyloid are the two most prevalent forms of this disease that commonly results in cardiac amyloidosis. In both of these conditions, the myocardium is a frequent site of infiltration and end-organ involvement often with devastating consequences. With cardiac amyloidosis becoming an increasingly identified disease that has previously been under-recognized, the purpose of this comprehensive review is to focus on the diagnosis and treatment of these two types of cardiac amyloidosis including a contemporary update on currently available therapies being investigated in clinical trials...
April 7, 2018: Current Treatment Options in Cardiovascular Medicine
Lorena Saelices, Stuart A Sievers, Michael R Sawaya, David S Eisenberg
Amyloid diseases are characterized by the deposition of proteins in the form of amyloid fibrils, in organs that eventually fail. The development of effective drug candidates follows from the understanding of the molecular processes that lead to protein aggregation. Here we study amyloidogenic segments of transthyretin (TTR). TTR is a transporter of thyroxine and retinol in the blood and cerebrospinal fluid. When mutated and/or as a result of aging, TTR aggregates into amyloid fibrils that accumulate in organs such as the heart...
April 7, 2018: Protein Science: a Publication of the Protein Society
Hannah Rosenblum, Adam Castano, Julissa Alvarez, Jeff Goldsmith, Stephen Helmke, Mathew S Maurer
BACKGROUND: TTR (transthyretin) cardiac amyloidosis is caused by dissociation of TTR into monomers, which misassemble into amyloid fibrils. TTR stabilizers act at the dimer-dimer interface to prevent dissociation. We investigated differences in survival among patients with TTR cardiac amyloidosis on stabilizer medications compared with those not on stabilizers. METHODS AND RESULTS: A retrospective study of patients with TTR cardiac amyloidosis presenting to a single center was conducted...
April 2018: Circulation. Heart Failure
Daniel Serrano, Christopher B Atzinger, Marc F Botteman
INTRODUCTION: Hereditary transthyretin (TTR) amyloidosis with polyneuropathy (hATTR-PN) is a rare, autosomal dominant amyloidosis characterized primarily by progressive ascending sensorimotor neuropathy often associated with  autonomic involvement. hATTR-PN is caused by a mutation in the TTR gene leading to protein misfolding and amyloid accumulation in peripheral nerves and vital organs. The latest global prevalence estimates point to 10,000 cases worldwide, with an upper end of about 40,000...
April 2, 2018: Neurology and Therapy
Xin Zheng, Shulin Chen, Linfang Li, Xiaohua Liu, Xiaomin Liu, Shuqin Dai, Peng Zhang, Huaiwu Lu, Zhongqiu Lin, Yuefei Yu, Guorong Li
OBJECTIVE: Serum human epididymis protein 4 (HE4) and transthyretin (TTR) are new markers for ovarian cancer. We compared HE4 and TTR with the gold marker CA-125 for the diagnosis of ovarian cancer patients. METHODS: 130 serum samples from benign ovarian tumor and 400 serum samples from healthy women were used to set up the cut-off. 126 serum samples from ovarian cancer patients before operation were collected to test the diagnostic value of these ELISA assays. The sensitivity, positive predictive value (PPV) and ROC curves were used to evaluate the diagnostic value...
March 30, 2018: Journal of Gynecology Obstetrics and Human Reproduction
Yuriko Wakita, Takashi Sato, Keisuke Chosa, Mary Ann Suico, Ryoko Sasaki, Shingo Kawano, Nami Hashimoto, Yuriko Teranishi, Yoshiki Imai, Hiroshi Morioka, Tsuyoshi Shuto, Hirofumi Kai
Transthyretin (TTR) is a tetrameric beta-sheet-rich protein that is important in the plasma transport of thyroxine and retinol. Mutations in the TTR gene cause TTR tetramer protein to dissociate to monomer, which is the rate-limiting step in familial amyloid polyneuropathy. Amyloidogenicity of individual TTR variants depends on the types of mutation that induce significant changes in biophysical, biochemical and/or biological properties. G101S TTR variant was previously identified in a Japanese male without amyloidotic symptom, and was considered as a non-amyloidogenic TTR variant...
2018: Biological & Pharmaceutical Bulletin
Meenakshi Verma, Amandeep Girdhar, Basant Patel, Nirmal K Ganguly, Ritushree Kukreti, Vibha Taneja
Interactions amongst different amyloid proteins have been proposed as a probable mechanism of aggregation and thus an important risk factor for the onset as well as progression of various neurodegenerative disorders including Alzheimer's, Parkinson's, Huntington's, and Amyotrophic Lateral Sclerosis. Evidences suggest that transthyretin (TTR), a plasma protein associated with transthyretin amyloidosis or familial polyneuropathy (FAP) interacts with heterologous amyloid proteins including amyloid beta and islet amyloid polypeptide...
2018: Frontiers in Molecular Neuroscience
Susana Lêdo, Ana Ramires, Ângela Leite, Maria Alzira P Dinis, Jorge Sequeiros
This longitudinal study aimed at determining predicting variables for middle and long-term psychological disturbance due pre-symptomatic testing (PST) for two late-onset neurological diseases, Huntington disease (HD) and TTR (transthyretin protein) familial amyloid polyneuropathy (FAP) Val30Met (now classified as Val50Met). 196 clinical records of persons who performed PST at least three years ago and answered to the two stages of evaluation (before PST and least 3 years after disclosure of results) were analysed...
March 23, 2018: European Journal of Medical Genetics
Renata Germano Borges de Oliveira Nascimento Freitas, Gabriel Hessel, Ana Carolina Junqueira Vasques, Roberto José Negrão Nogueira
Nutritional support is an effective strategy to restore or maintain nutritional status, to reduce clinical complications, hospitalization period and the morbidity/mortality risk of hospitalized patients. So, a good marker is important to evaluate the nutritional support. OBJECTIVES: This study aims to evaluate the evolution of transthyretin levels in patients receiving parenteral nutrition (PN) during 14 days. DESIGN & METHODS: Longitudinal study of 88 hospitalized patients...
April 2018: Clinical Nutrition ESPEN
Vincent Algalarrondo, Eve Piekarski, Ludivine Eliahou, Dominique Le Guludec, Michel S Slama, François Rouzet
PURPOSE OF REVIEW: Nuclear imaging recently gained a key role in the diagnosis and prognostic assessment of transthyretin (TTR)-related cardiac amyloidosis. This review aims at summarizing the state-of-the art regarding the implementation of nuclear imaging in the management of hereditary mutated TTR-cardiac amyloidosis (mTTR-CA). RECENT FINDINGS: Although cardiac uptake of bone tracers is acknowledged as a specific marker of TTR amyloid cardiac burden, recent studies validated the implementation of bone scan in the flow chart for non-invasive diagnosis and follow-up of CA in multicenter trials...
March 24, 2018: Current Cardiology Reports
Fabiola Escolano-Lozano, Ana Paula Barreiros, Frank Birklein, Christian Geber
Background: Familial transthyretin amyloidosis is a life-threatening disease presenting with sensorimotor and autonomic polyneuropathy. Delayed diagnosis has a detrimental effect on treatment and prognosis. To facilitate diagnosis, we analyzed data patterns of patients with transthyretin familial amyloid polyneuropathy (TTR-FAP) and compared them to polyneuropathies of different etiology for clinical and electrophysiological discriminators. Methods: Twenty-four patients with TTR-FAP and 48 patients with diabetic polyneuropathy (dPNP) were investigated (neurological impairment score NIS; neurological disability score NDS) in a cross-sectional design...
January 2018: Brain and Behavior
Ankita Sharma, Monu, Sagarika Biswas
BACKGROUND: Transthyretin (TTR) is the transporter protein (55 kDA) that carries retinol binding protein and thyroxin (T4) in its functional tetramer form. Presence of mutation in this protein (TTR) may lead to the dissociation of tetramers to monomer which unfolds and self associates to form amyloid aggregates. Aggregation of this protein has been found to be associated with various life threatening disorders such as coronary artery disease (CAD) which is the major cause of mortality and morbidity worldwide...
March 20, 2018: Current Computer-aided Drug Design
Ni Zhang, Sheng Bi
Physical activity or exercise contributes to energy balance via affecting energy expenditure and food intake, but how exercise affects food intake and the neural mechanism of the feeding effect of exercise remain to be determined. Growing evidence indicates that physical exercise exerts a distinct action in the control of food intake and body weight. Studies of exercise in rodent models with voluntary access to running wheels reveal an inhibitory effect of voluntary exercise on food intake and body weight. Voluntary running activity decreases food intake and body weight, prevents obesity, and slows down body weight regain...
March 17, 2018: Physiology & Behavior
Ana Martinez-Naharro, Tushar Kotecha, Karl Norrington, Michele Boldrini, Tamer Rezk, Candida Quarta, Thomas A Treibel, Carol J Whelan, Daniel S Knight, Peter Kellman, Frederick L Ruberg, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
OBJECTIVES: This study evaluated the prognostic potential of native myocardial T1 in cardiac transthyretin amyloidosis (ATTR) and compared native T1 with extracellular volume (ECV) in terms of diagnostic accuracy and prognosis. BACKGROUND: ATTR is an increasingly recognized cause of heart failure that has an overlapping clinical phenotype with hypertrophic cardiomyopathy (HCM). Native T1 mapping by cardiac magnetic resonance (CMR) is useful for diagnosis in cardiac amyloidosis but its prognostic potential has never been assessed...
March 12, 2018: JACC. Cardiovascular Imaging
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