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https://www.readbyqxmd.com/read/28435766/a-heart-too-stiff-to-beat-a-case-of-familial-transthyretin-amyloidosis-cardiomyopathy
#1
Yan Zhou, Sameen Khalid, Aamer Abbass, Laura Hughes, Marcos Hazday
Heart failure is a common clinical syndrome caused by a variety of cardiac diseases. We report a rare case of familial transthyretin amyloidosis cardiomyopathy to heighten the awareness of this rare but lethal cause of heart failure, as therapeutic interventions such as liver or heart transplant could be curative in selected patients.
March 20, 2017: Curēus
https://www.readbyqxmd.com/read/28434942/ipsc-derived-regulatory-dendritic-cells-inhibit-allograft-rejection-by%C3%A2-generating-alloantigen-specific-regulatory-t-cells
#2
Songjie Cai, Jiangang Hou, Masayuki Fujino, Qi Zhang, Naotsugu Ichimaru, Shiro Takahara, Ryoko Araki, Lina Lu, Ji-Mei Chen, Jian Zhuang, Ping Zhu, Xiao-Kang Li
Regulatory dendritic cell (DCregs)-based immunotherapy is a potential therapeutic tool for transplant rejection. We generated DCregs from murine induced pluripotent stem cells (iPSCs), which could remain in a "stable immature stage" even under strong stimulation. Harnessing this characteristic, we hypothesized that iPS-DCregs worked as a negative vaccine to generate regulatory T cells (Tregs), and induced donor-specific allograft acceptance. We immunized naive CBA (H-2K(k)) mice with B6 (H-2K(b)) iPS-DCregs and found that Tregs (CD4(+)CD25(+)FOXP3(+)) significantly increased in CBA splenocytes...
April 18, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28431981/ventricular-assist-device-elicits-serum-natural-igg-that-correlates-with-the-development-of-primary-graft-dysfunction-following-heart-transplantation
#3
Sarah B See, Kevin J Clerkin, Peter J Kennel, Feifan Zhang, Matthew P Weber, Kortney J Rogers, Debanjana Chatterjee, Elena R Vasilescu, George Vlad, Yoshifumi Naka, Susan W Restaino, Maryjane A Farr, Veli K Topkara, Paolo C Colombo, Donna M Mancini, P Christian Schulze, Bruce Levin, Emmanuel Zorn
BACKGROUND: Pre-transplant sensitization is a limiting factor in solid-organ transplantation. In heart transplants, ventricular assist device (VAD) implantation has been associated with sensitization to human leukocyte antigens (HLA). The effect of VAD on non-HLA antibodies is unclear. We have previously shown that polyreactive natural antibodies (Nabs) contribute to pre-sensitization in kidney allograft recipients. Here we assessed generation of Nabs after VAD implantation in pre-transplant sera and examined their contribution to cardiac allograft outcome...
March 24, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28431443/-surgical-and-interventional-therapy-of-mitral-valve-regurgitation
#4
Wolfgang Schillinger, Miriam Puls, Bernhard C Danner
Depending from the etiology of mitral valve pathology and surgical risk, mitral valve surgery is the therapy of choice for most degenerative mitral valve diseases. In isolated secondary mitral valve regurgitation, the indication for interventional or conservative therapy becomes more important. In severe left ventricular dysfunction heart transplantation has to be taken into consideration. If relevant coronary artery disease is present and bypass surgery is indicated, mitral valve surgery is mandatory in severe regurgitation and is subject to a benefit-risk evaluation in moderate regurgitation...
April 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28431061/high-prevalence-of-arrhythmic-and-myocardial-complications-in-patients-with-cardiac-glycogenosis-due-to-prkag2-mutations
#5
Julien Thevenon, Gabriel Laurent, Flavie Ader, Pascal Laforêt, Didier Klug, Anju Duva Pentiah, Laurent Gouya, Claude Alain Maurage, Salem Kacet, Jean-Christophe Eicher, Juliette Albuisson, Michel Desnos, Eric Bieth, Denis Duboc, Laurent Martin, Patricia Réant, François Picard, Claire Bonithon-Kopp, Elodie Gautier, Christine Binquet, Christel Thauvin-Robinet, Laurence Faivre, Patrice Bouvagnet, Philippe Charron, Pascale Richard
Aims: Mutations in PRKAG2, the gene encoding for the γ2 subunit of 5'-AMP-activated protein kinase (AMPK), are responsible for an autosomal dominant glycogenosis with a cardiac presentation, associating hypertrophic cardiomyopathy (HCM), ventricular pre-excitation (VPE), and progressive heart block. The aim of this study was to perform a retrospective time-to-event study of the clinical manifestations associated with PRKAG2 mutations. Methods and Results: A cohort of 34 patients from 9 families was recruited between 2001 and 2010...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28430300/left-ventricular-assist-pump-pocket-infection-conservative-treatment-strategy-for-destination-therapy-candidates
#6
Elie Haddad, François-Xavier Lescure, Walid Ghodhbane, Laurent Lepage, Camille D'humieres, William Vindrios, Yazdan Yazdanpanah, Patrick Nataf, Matthias Kirsch
BACKGROUND: Heart failure is a major cause of mortality and morbidity, particularly among patients with advanced disease and no access to cardiac transplantation. LVAD implantation is not only a bridge-to-transplantation option for patients awaiting a heart donor, but is often used as bridge-to-destination therapy in patients unsuited for transplantation for various reasons. LVAD infection is considered the second-most common cause of death in patients who survive the initial 6 months on LVAD support...
April 20, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28427830/liver-transplantation-for-hereditary-hemorrhagic-telangiectasia-a-systematic-review
#7
REVIEW
Emanuele Felli, Pietro Addeo, François Faitot, Gennaro Nappo, Constantin Oncioiu, Philippe Bachellier
AIM: To evaluate the indications, timing and results of liver transplantation in patients affected by hereditary hemorrhagic telangiectasia (HHT), by undertaking a systematic review of the current literature. METHODS: Electronic bibliographical databases were searched on MEDLINE and Pubmed according to the PRISMA criteria. A total of 58 articles were initially found, 11 have been excluded because of single center series later included in the European Liver transplant Registry (ELTR), already reported in this study...
April 17, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28425200/high-dose-calcineurin-inhibitor-free-everolimus-as-a-maintenance-regimen-for-heart-transplantation-may-be-a-risk-factor-for%C3%A2-pneumocystis-pneumonia
#8
Yu-Ning Hu, Nan-Yao Lee, Jun-Neng Roan, Chi-Hsin Hsu, Chwan-Yao Luo
BACKGROUND: Everolimus reduces the incidence of cardiac-allograft vasculopathy (CAV) and is less renally toxic than are calcineurin inhibitors (CNIs). We evaluated the safety of CNI-free everolimus for post-heart transplant (HTx) patients. METHODS: We retrospectively reviewed the records of 36 consecutive patients who had undergone an HTx between January 2006 and December 2013 in National Cheng Kung University Hospital. All patients initially had been treated with the standard tacrolimus regimen...
April 20, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28424763/airway-problems-in-neonates-a-review-of-the-current-investigation-and-management-strategies
#9
REVIEW
Quen Mok
Airway problems in the neonatal population are often life threatening and raise challenging issues in diagnosis and management. The airway problems can result from congenital or acquired lesions and can be broadly classified into those causing obstruction or those due to an abnormal "communication" in the airway. Many different investigations are now available to identify the diagnosis and quantify the severity of the problem, and these tests can be simple or invasive. Bronchography and bronchoscopy are essential to determine the extent and severity of the airway problem and to plan treatment strategy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28421570/-real-world-experience-in-cardiac-resynchronization-therapy-at-a-swiss-tertiary-care-center
#10
Stephan Winnik, Christian Elsener, Burkhardt Seifert, Christoph Starck, Agnes Straub, Ardan M Saguner, Alexander Breitenstein, Nazmi Krasniqi, Markus J Wilhelm, Laurent Haegeli, Firat Duru, Stefano Benussi, Francesco Maisano, Thomas F Lüscher, Johannis Holzmeister, David Huerlimann, Frank Ruschitzka, Jan Steffel
BACKGROUND: Based on a reduction in morbidity and mortality, cardiac resynchronisation therapy (CRT) has evolved as a standard therapy for patients with advanced heart failure. OBJECTIVE: To provide insight into patient demographics, safety, echocardiographic remodelling and long-term follow-up of patients treated with CRT in a "real-world" setting at a Swiss tertiary care centre. METHODS: Patients implanted with a CRT device at the University Heart Centre Zurich between 2000 and 2015 were consecutively enrolled...
April 19, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28420862/clinical-characteristics-treatment-and-prognosis-of-patients-with-acute-severe-heart-failure-of-ischemic-and-non-ischemic-etiology-analysis-from-the-commit-ahf-registry
#11
Mateusz Ostręga, Marek J Gierlotka, Grzegorz Słonka, Paweł Nadziakiewicz, Mariusz Gąsior
INTRODUCTION    There are limited data on the impact of ischemic etiology on clinical status and long-term prognosis among patients with acute severe heart failure (ASHF) not associated with acute coronary syndrome (ACS).  OBJECTIVES    To assess clinical characteristics, treatment and the 12-month mortality of patients with no-ACS ASHF according to etiology.  PATIENTS AND METHODS    The data from 112 patients with ASHF that was not associated with ACS were analyzed: 61 with ischemic (IASHF) and 51 with non-ischemic (NIASHF) etiology...
April 18, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28420436/autophagy-mediates-the-beneficial-effect-of-hypoxic-preconditioning-on-bone-marrow-mesenchymal-stem-cells-for-the-therapy-of-myocardial-infarction
#12
Zheng Zhang, Chao Yang, Mingzhi Shen, Ming Yang, Zhitao Jin, Liping Ding, Wei Jiang, Junke Yang, Haixu Chen, Feng Cao, Taohong Hu
BACKGROUND: Stem cell therapy has emerged as a promising therapeutic strategy for myocardial infarction (MI). However, the poor viability of transplanted stem cells hampers their therapeutic efficacy. Hypoxic preconditioning (HPC) can effectively promote the survival of stem cells. The aim of this study was to investigate whether HPC improved the functional survival of bone marrow mesenchymal stem cells (BM-MSCs) and increased their cardiac protective effect. METHODS: BM-MSCs, isolated from Tg(Fluc-egfp) mice which constitutively express both firefly luciferase (Fluc) and enhanced green fluorescent protein (eGFP), were preconditioned with HPC (1% O2) for 12 h, 24 h, 36 h, and 48 h, respectively, followed by 24 h of hypoxia and serum deprivation (H/SD) injury...
April 18, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28416468/cardiac-transplantation-in-children-and-adolescents-with-long-qt-syndrome
#13
Angela M Kelle, J Martijn Bos, Susan P Etheridge, Bryan C Cannon, Randall M Bryant, Jonathan N Johnson, Michael J Ackerman
BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal, yet highly treatable, cardiac channelopathy. Cardiac transplantation has been reported anecdotally for patients with severe LQTS refractory to standard therapies. OBJECTIVE: We evaluated the incidence and risk factors for cardiac transplantation among children evaluated and treated in an LQTS specialty center. METHODS: This was a retrospective review of 349 children with LQTS (mean age at diagnosis, 8...
April 14, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28413579/metabolic-recovery-of-the-failing-heart-emerging-therapeutic-options
#14
Dale J Hamilton
Heart failure has mortality rates that parallel those of breast cancer. Current management strategies include neurohormonal blockade, rate control measures, natriuretic peptide preservation, implantation of mechanical assist devices, and heart transplantation. Despite these strategies, however, the failing myocardium remains energy depleted. New strategies to promote metabolic recovery are being developed to potentially augment current treatment guidelines. For example, an unexpected finding of our own studies showed that mechanical unloading with assist devices in advanced-stage heart failure restored metabolic flux...
January 2017: Methodist DeBakey Cardiovascular Journal
https://www.readbyqxmd.com/read/28413189/heart-transplantation-in-asia
#15
Hae-Young Lee, Byung-Hee Oh
Heart transplantation (HTx) is the effective way to improve quality of life as well as survival in terminal heart failure (HF) patients. Since the first heart transplant in 1968 in Japan and in earnest in 1987 at Taiwan, HTx has been continuously increasing in Asia. Although the current percentage of heart transplants from Asia comprises only 5.7% of cases in the International Society of Heart and Lung Transplantation (ISHLT) registry, the values were under-reported and soon will be greatly increased. HTx in Asia shows comparable with or even better results compared with ISHLT registry data...
April 11, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28413112/the-effect-of-negative-remodeling-on-fractional-flow-reserve-after-cardiac-transplantation
#16
William F Fearon, Robert Felix, Atsushi Hirohata, Ryota Sakurai, Powell O Jose, Masao Yamasaki, Mamoo Nakamura, Peter J Fitzgerald, Hannah A Valantine, Paul G Yock, Alan C Yeung
BACKGROUND: Negative remodeling is a common occurrence early after cardiac transplantation. Its impact on the development of myocardial ischemia is not well documented. The aim of this study is to investigate the impact of negative remodeling on fractional flow reserve after cardiac transplantation. METHODS: Thirty-four cardiac transplant recipients underwent intravascular ultrasound (IVUS) and fractional flow reserve (FFR) assessment soon after transplantation and one year later...
April 8, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28412246/absence-of-nucks-augments-paracrine-effects-of-mesenchymal-stem-cells-mediated-cardiac-protection
#17
Yuelin Zhang, Sinming Chiu, Xiaoting Liang, Yuet-Hung Chai, Yiming Qin, Junwen Wang, Xiang Li, Beiying Qiu, Vinay Tergaonkar, Hung-Fat Tse, Qizhou Lian
Bone marrow-derived mesenchymal stem cells (BM-MSCs) contribute to myocardial repair after myocardial infarction (MI) by secreting a panel of growth factors and cytokines. This study was to investigate the potential mechanisms of the nuclear casein kinase and cyclin-dependent kinase substrate 1 (NUCKS) in regulation of the profiles of BM-MSCs secretion and compare the therapeutic efficacy of NUCKS(-/-)- and wide type-BM-MSCs (WT-BM-MSCs) on MI. The secretion profiles between NUCKS(-/-)- and WT-BM-MSCs under hypoxia (1%O2) were analyzed...
April 12, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28410244/platelet-derived-growth-factor-receptor-alpha-positive-cardiac-progenitor-cells-derived-from-multipotent-germline-stem-cells-are-capable-of-cardiomyogenesis-in-vitro-and-in-vivo
#18
Bang-Jin Kim, Yong-Hee Kim, Yong-An Lee, Sang-Eun Jung, Yeong Ho Hong, Eun-Ju Lee, Byung-Gak Kim, Seongsoo Hwang, Jeong Tae Do, Myung-Geol Pang, Buom-Yong Ryu
Cardiac cell therapy has the potential to revolutionize treatment of heart diseases, but its success hinders on the development of a stem cell therapy capable of efficiently producing functionally differentiated cardiomyocytes. A key to unlocking the therapeutic application of stem cells lies in understanding the molecular mechanisms that govern the differentiation process. Here we report that a population of platelet-derived growth factor receptor alpha (PDGFRA) cells derived from mouse multipotent germline stem cells (mGSCs) were capable of undergoing cardiomyogenesis in vitro...
March 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/28409553/the-thyromimetic-kb2115-eprotirome-induces-rat-hepatocyte-proliferation
#19
Marta Szydlowska, Monica Pibiri, Andrea Perra, Elisabetta Puliga, Sandra Mattu, Giovanna Ledda-Columbano, Amedeo Columbano, Vera Leoni
Although the hepatomitogenic activity of T3 is well established, the wide range of harmful effects exerted by this hormone precludes its use in regenerative therapy. The aim of this study was to investigate whether an agonist of TR, KB2115 (Eprotirome) could exert a mitogenic effect in the liver, without most of the adverse T3/TR-dependent side effects. F-344 rats treated with KB2115 for one week displayed a massive increase in bromodeoxyuridine incorporation (from 20 to 40% vs. 5% of controls), which was associated with increased mitotic activity in the absence of significant signs of liver toxicity...
April 13, 2017: Gene Expression
https://www.readbyqxmd.com/read/28408734/mycophenolate-mofetil-and-pulmonary-fibrosis-after-kidney-transplantation-a-case-report
#20
Kazuhiro Takahashi, Pauline Go, Chad H Stone, Mohamed Safwan, Krishna G Putchakayala, William J Kane, Lauren E Malinzak, Dean Y Kim, Jason E Denny
BACKGROUND Mycophenolate mofetil (MMF) induced lung disease has been described in only a few isolated reports. We report a case of fatal respiratory failure associated with MMF after kidney transplantation. CASE REPORT A 50-year-old Hispanic male with a history of end-stage renal disease secondary to hypertension underwent deceased donor kidney transplantation. His preoperative evaluations were normal except for a chest x-ray which showed bilateral interstitial opacities. Tacrolimus and MMF were started on the day of surgery...
April 14, 2017: American Journal of Case Reports
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