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Transthyretin

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https://www.readbyqxmd.com/read/29217108/improved-outcomes-after-heart-transplantation-for-cardiac-amyloidosis-in-the-modern-era
#1
Arnt V Kristen, Michael M Kreusser, Patrick Blum, Stefan O Schönland, Lutz Frankenstein, Andreas O Dösch, Benjamin Knop, Matthias Helmschrott, Bastian Schmack, Arjang Ruhparwar, Ute Hegenbart, Hugo A Katus, Philip W J Raake
BACKGROUND: Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTR amyloidosis, and changed chemotherapy regimens for AL amyloidosis...
November 15, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29211930/estimating-the-global-prevalence-of-transthyretin-familial-amyloid-polyneuropathy
#2
Hartmut H Schmidt, Márcia Waddington-Cruz, Marc F Botteman, John A Carter, Avijeet S Chopra, Markay Hopps, Michelle Stewart, Shari Fallet, Leslie Amass
Objective To estimate the global prevalence of transthyretin familial amyloid polyneuropathy (ATTR-FAP). METHODS: Prevalence estimates and information supporting prevalence calculations was extracted from records yielded by reference-databases searches (2005-2016), conference proceedings, and non-peer reviewed sources. Prevalence was calculated as prevalence rate multiplied by general-population size, then extrapolated to countries without prevalence estimates but with reported cases...
December 6, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/29209781/age-dependent-cognitive-dysfunction-in-untreated-hereditary-transthyretin-amyloidosis
#3
Ana Martins da Silva, Sara Cavaco, Joana Fernandes, Raquel Samões, Cristina Alves, Márcio Cardoso, Jeffery W Kelly, Cecília Monteiro, Teresa Coelho
Central nervous system (CNS) involvement in hereditary transthyretin (TTR) amyloidosis has been described in patients whose disease course was modified by liver transplant. However, cognitive dysfunction has yet to be investigated in those patients. Moreover, CNS involvement in untreated patients or asymptomatic mutation carriers remains to be studied. A series of 340 carriers of the TTRVal30Met mutation (180 symptomatic and 160 asymptomatic) underwent a neuropsychological assessment, which included the Dementia Rating Scale-2 (DRS-2), auditory verbal learning test, semantic fluency, phonemic fluency, and trail making test...
December 5, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29202938/prevalence-of-monoclonal-gammopathy-in-wild-type-transthyretin-amyloidosis
#4
Hallie I Geller, Avinainder Singh, Tara M Mirto, Robert Padera, Richard Mitchell, Jacob P Laubach, Rodney H Falk
OBJECTIVE: To evaluate the prevalence of monoclonal gammopathy (MG) in patients with wild-type transthyretin amyloidosis (ATTRwt) (formerly known as senile amyloidosis). PATIENTS AND METHODS: We retrospectively analyzed the serum protein electrophoresis and serum immunofixation results, free light chain (FLC) levels, and renal function of 113 consecutive patients with ATTRwt seen at the Brigham and Women's Hospital's Cardiac Amyloidosis Program between February 21, 2006, and November 9, 2016...
December 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29199249/utility-of-single-photon-emission-computed-tomography-computed-tomography-fusion-imaging-with-99-mtc-pyrophosphate-scintigraphy-in-the-assessment-of-cardiac-transthyretin-amyloidosis
#5
Noriko Tsuda, Shinya Shiraishi, Seitaro Oda, Koji Ogasawara, Fumi Sakamoto, Seiji Tomiguchi, Koichiro Fujisue, Seiji Takashio, Yasuhiro Izumiya, Kenichi Tsujita, Yukio Ando, Yasuyuki Yamashita
No abstract text is available yet for this article.
December 2, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29191088/the-delaying-effect-of-alpha-glycerophosphocholine-on-senescence-transthyretin-deposition-and-osteoarthritis-in-senescence-accelerated-mouse-prone-8-mice
#6
Kiminori Matsubara, Mayumi Okuda, Sachi Shibata, Shigeru Miyaki, Takeshi Ohkubo, Hanae Izu, Tsutomu Fujii
Administration of alpha-glycerophosphocholine (GPC), a choline compound in food, is expected to contribute to human health. In this study, we evaluated its effect on aging in senescence-accelerated mouse prone 8 (SAMP8) mice. Male SAMP8 mice had free access to a commercial stock diet and drinking water with or without GPC (0.07 mg/ml). Mice in the GPC group had significantly lower total senescence grading score than that of the control group at 36 weeks of age. Administration of GPC decreased the deposition of transthyretin (TTR), an amyloidogenic protein, in the brain...
December 1, 2017: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/29190982/diagnostic-performance-enhancement-of-pancreatic-cancer-using-proteomic-multimarker-panel
#7
Jiyoung Park, Yonghwan Choi, Junghyun Namkung, Sung Gon Yi, Hyunsoo Kim, Jiyoung Yu, Yongkang Kim, Min-Seok Kwon, Wooil Kwon, Do-Youn Oh, Sun-Whe Kim, Seung-Yong Jeong, Wonshik Han, Kyu Eun Lee, Jin Seok Heo, Joon Oh Park, Joo Kyung Park, Song Cheol Kim, Chang Moo Kang, Woo Jin Lee, Seungyeoun Lee, Sangjo Han, Taesung Park, Jin-Young Jang, Youngsoo Kim
Due to its high mortality rate and asymptomatic nature, early detection rates of pancreatic ductal adenocarcinoma (PDAC) remain poor. We measured 1000 biomarker candidates in 134 clinical plasma samples by multiple reaction monitoring-mass spectrometry (MRM-MS). Differentially abundant proteins were assembled into a multimarker panel from a training set (n=684) and validated in independent set (n=318) from five centers. The level of panel proteins was also confirmed by immunoassays. The panel including leucine-rich alpha-2 glycoprotein (LRG1), transthyretin (TTR), and CA19-9 had a sensitivity of 82...
November 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29187090/outcomes-of-patients-with-familial-transthyretin-amyloidosis-after-liver-transplantation
#8
Dipti Banerjee, Lindsey E Roeker, Martha Grogan, Paul Swiecicki, John Poterucha, Julie Heimbach, Steve Zeldenrust, Morie Gertz, Brooks Edwards, Richard Daly, Kyle W Klarich, Angela Dispenzieri
BACKGROUND: Familial transthyretin amyloidosis is a disease caused by misfolded transthyretin aggregates that can impair multiple organ systems. Liver transplantation is the first-line treatment for familial transthyretin amyloidosis. RESEARCH QUESTION: Our objective is to study outcomes and survival among patients with familial transthyretin amyloidosis after transplantation. DESIGN: All patients undergoing orthotopic liver transplant for familial transthyretin amyloidosis at Mayo Clinic between 1997 and 2012 were reviewed...
September 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/29185488/microrna-regulation-of-transthyretin-in-trophoblast-differentiation-and-intra-uterine-growth-restriction
#9
Sarbani Saha, Shreeta Chakraborty, Agnihotri Bhattacharya, Arati Biswas, Rupasri Ain
Placental trophoblast cells produce various cytokines, transporters vital to normal embryogenesis. Transthyretin (TTR) aids trans-placental passage of maternal thyroxin (TH) to fetal circulation. Inadequate TH delivery leads to developmental abnormality. Regulation of TTR biosynthesis in placenta is critical for normal embryo development. We showed here that TTR transcripts were expressed more in fetal placenta. Using bioinformatic analysis and confirmation with dual-luciferase reporter assays, we found that miR-200a-3p and miR-141-3p inhibited TTR expression by directly binding to the 3'UTR of TTR, which is reversed by mutation in the microRNA binding site...
November 29, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29184431/update-on-the-clinical-utility-of-an-rna-interference-based-treatment-focus-on-patisiran
#10
REVIEW
Malak Rizk, Şükrü Tüzmen
RNA interference (RNAi) is a naturally existing endogenous mechanism for post-transcriptional gene regulation, nowadays commonly utilized for functional characterization of genes and development of potential treatment strategies for diseases. RNAi-based studies for therapy, after being examined for over a decade, are finally in the pipeline for developing a potential treatment for the mutated transthyretin (TTR) gene, which gives rise to a dysfunctional TTR protein. This dysfunctional protein causes TTR amyloidosis (ATTR), an inherited, progressively incapacitating, and often fatal genetic disorder...
2017: Pharmacogenomics and Personalized Medicine
https://www.readbyqxmd.com/read/29177547/genetic-and-clinical-characteristics-of-hereditary-transthyretin-amyloidosis-in-endemic-and-non-endemic-areas-experience-from-a-single-referral-center-in-japan
#11
Taro Yamashita, Mitsuharu Ueda, Yohei Misumi, Teruaki Masuda, Toshiya Nomura, Masayoshi Tasaki, Kotaro Takamatsu, Keiko Sasada, Konen Obayashi, Hirotaka Matsui, Yukio Ando
Hereditary transthyretin (ATTR) amyloidosis is a life-threatening, autosomal dominant, systemic amyloidosis caused by mutant transthyretin. In addition to ATTRV30M in endemic and non-endemic areas, more than 140 non-V30M mutations occur worldwide. The aim of this study was to analyze the clinical characteristics and genetic frequencies of hereditary ATTR amyloidosis. Diagnostic results and clinical manifestations of hereditary ATTR amyloidosis from April 1, 2012, to March 31, 2017, at Amyloidosis Medical Practice Center, Kumamoto University Hospital were analyzed...
November 24, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29161598/roles-and-potential-mechanisms-of-selenium-in-countering-thyrotoxicity-of-dehp
#12
Pei Zhang, Xie Guan, Min Yang, Li Zeng, Changjiang Liu
Di-(2-ethylhexyl) phthalate (DEHP) as a ubiquitous environmental contaminant could disturb thyroid hormone (TH) homeostasis. Selenium as an essential trace element has protective effects on thyroids. To verify roles of selenium in countering thyrotoxicity of DEHP and elucidate potential mechanisms, Sprague-Dawley rats and Nthy-ori 3-1 cells were treated with DEHP or/and selenomethionine (SeMet). Results showed that selenium supplementation elevated plasma free thyroxine (FT4) that was decreased by DEHP, and free triiodothyronine (FT3) and thyroid stimulating hormone (TSH) levels were also partially recovered...
November 18, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/29155738/imaging-amyloid-tissues-stained-with-luminescent-conjugated-oligothiophenes-by-hyperspectral-confocal-microscopy-and-fluorescence-lifetime-imaging
#13
Sofie Nyström, Marcus Bäck, K Peter R Nilsson, Per Hammarström
Proteins that deposit as amyloid in tissues throughout the body can be the cause or consequence of a large number of diseases. Among these we find neurodegenerative diseases such as Alzheimer's and Parkinson's disease afflicting primarily the central nervous system, and systemic amyloidosis where serum amyloid A, transthyretin and IgG light chains deposit as amyloid in liver, carpal tunnel, spleen, kidney, heart, and other peripheral tissues. Amyloid has been known and studied for more than a century, often using amyloid specific dyes such as Congo red and Thioflavin T (ThT) or Thioflavin (ThS)...
October 20, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29154979/small-fiber-neuropathy-diagnosis-causes-and-treatment
#14
Damien Sène
Small fiber neuropathy, which affects the sensory A≏ and C fibers, is now a major diagnostic and therapeutic challenge. Nearly 7% of the general population have chronic neuropathic pain responsible for severe quality-of-life impairments. Awareness must therefore be raised among clinicians of the somatosensory and autonomic symptoms that can reveal small fiber neuropathy, appropriate diagnostic investigations, most common causes, and best treatment options for each patient profile. To help achieve this goal, the present review article discusses the clinical presentation of neuropathic pain and paresthesia and/or autonomic dysfunction due to involvement of nerves supplying exocrine glands and smooth muscle; normal findings from standard electrophysiological investigations; most informative diagnostic tests (epidermal nerve fiber density in a skin biopsy, laser-evoked potentials, heat- and cold-detection thresholds, electrochemical skin conductance); main causes, which consist chiefly of metabolic diseases (diabetes mellitus, glucose intolerance), dysimmunity syndromes (Sjögren's syndrome, sarcoidosis, monoclonal gammopathy), and genetic abnormalities (familial amyloidosis due to a transthyretin mutation, Fabry disease, sodium channel diseases); and the available symptomatic and etiological treatments...
November 15, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29153245/frequency-of-and-prognostic-significance-of-cardiac-involvement-at-presentation-in-hereditary-transthyretin-derived-amyloidosis-and-the-value-of-n-terminal-pro-b-type-natriuretic-peptide
#15
Sebastiaan H C Klaassen, Jasper Tromp, Hans L A Nienhuis, Peter van der Meer, Maarten P van den Berg, Hans Blokzijl, Dirk J van Veldhuisen, Bouke P C Hazenberg
The aim of this study is to assess the prevalence of cardiac involvement in hereditary transthyretin-derived (ATTRm) amyloidosis at the time of diagnosis and to determine the diagnostic and clinical value of N-terminal pro-B-type natriuretic peptide (NT-proBNP). The University Medical Center Groningen is the national center of expertise for amyloidosis. All consecutive patients between 1994 and 2016 with ATTRm amyloidosis were followed prospectively. Baseline was set at the time of the first positive biopsy...
October 14, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29151280/oral-nutritional-supplementation-in-patients-undergoing-peritoneal-dialysis-a-randomised-crossover-pilot-study
#16
Karen M Salamon, Kelly Lambert
BACKGROUND: Malnutrition is a significant problem in those undergoing peritoneal dialysis (PD). Factors such as gastrointestinal (GI) symptoms and the need for a fluid reduced diet can limit tolerance and thereby the efficacy of oral nutritional supplements to treat malnutrition. OBJECTIVES: To evaluate the acceptability and impact of two different forms of oral nutrition supplementation for 16 weeks on nutritional markers and quality of life of malnourished patients undergoing PD...
November 19, 2017: Journal of Renal Care
https://www.readbyqxmd.com/read/29135289/-what-should-we-know-about-cardiac-amyloidosis-from-clinical-signs-to-treatment
#17
Dóra Földeák, Attila Nemes, Anita Kalapos, Péter Domsik, Árpád Kormányos, László Krenács, Enikő Bagdi, Zita Borbényi
Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment...
November 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/29133141/abdominal-fat-pad-excisional-biopsy-for-the-diagnosis-and-typing-of-systemic-amyloidosis
#18
Yessica Garcia, A Bernard Collins, James R Stone
In the past, the diagnosis and typing of amyloidosis often required an invasive biopsy of an internal organ, such as the heart or kidneys. Abdominal fat pad excisional biopsy (FPEB) offers a less invasive approach, but the sensitivity of this technique has been unclear. To determine the sensitivity of FPEB for immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, we performed a retrospective clinicopathologic analysis of 97 patients who had undergone FPEB, of which 16 were positive for amyloid...
November 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/29125088/cerebrospinal-fluid-biomarkers-as-predictors-of-shunt-response-in-idiopathic-normal-pressure-hydrocephalus-a-systematic-review
#19
Tyler Pfanner, Alexandre Henri-Bhargava, Stephanie Borchert
BACKGROUND: The widely accepted treatment for idiopathic normal-pressure hydrocephalus (iNPH) is a cerebrospinal fluid (CSF) diversion shunt procedure, to which approximately 80% of patients will respond. The purpose of this systematic review was to identify which CSF biomarkers have been investigated in predicting shunt responsiveness in iNPH patients, and to analyze the level of evidence for each. METHODS: To find all relevant articles, a comprehensive search of Medline, Embase, and PsycINFO was conducted...
November 10, 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/29124017/optimization-of-an-in-vitro-assay-methodology-for-competitive-binding-of-thyroidogenic-xenobiotics-with-thyroxine-on-human-transthyretin-and-albumin
#20
Katie L Hill, Timo Hamers, Jorke H Kamstra, William G Willmore, Robert J Letcher
Thyroid hormones (THs) are involved in the regulation of many physiological processes in vertebrates. Competition for TH binding sites on serum transport proteins can interfere with delivery of THs to target tissues, and this is a potential mechanism of action of exogenous thyroidogenic substances. To date, detailed accounts of in vitro methods for competitive binding with THs on TH transport proteins (human or wildlife) are sparse. In the limited number of published studies on in vitro radio-labelled TH-TH transport protein interactions, method descriptions were brief and with insufficient details for successful replication...
2017: MethodsX
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