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https://www.readbyqxmd.com/read/29024324/clinical-implications-of-gastrointestinal-symptoms-in-systemic-amyloidosis
#1
T Yen, F W Chen, R M Witteles, M Liedtke, L A Nguyen
BACKGROUND: Gastrointestinal (GI) symptoms in systemic amyloidosis patients are poorly characterized. This purpose of this study is to define the epidemiology and clinical implications of such symptoms. METHODS: This was a retrospective cohort study of 583 amyloid patients seen at a tertiary referral center. Of 96 symptomatic patients, 82 received endoscopic biopsies, subsequently grouped into those with histologic evidence of GI amyloid (biopsy proven) vs without (biopsy absent)...
October 10, 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28919633/clinical-presentation-and-outcomes-in-light-chain-amyloidosis-patients-with-non-evaluable-serum-free-light-chains
#2
S Sidana, N Tandon, A Dispenzieri, M A Gertz, F K Buadi, M Q Lacy, D Dingli, A L Fonder, S R Hayman, M A Hobbs, W I Gonsalves, Y L Hwa, P Kapoor, R A Kyle, N Leung, R S Go, J A Lust, S J Russell, S R Zeldenrust, V Rajkumar, S K Kumar
Hematologic response criteria in light chain (AL) amyloidosis require the difference in involved and uninvolved free light chains (dFLC) to be at least 5 mg/dl. We describe the clinical presentation and outcomes of newly diagnosed amyloidosis patients with dFLC<5 mg/dl (non-evaluable dFLC; 14%, n=165) compared to patients with dFLC ⩾5 mg/dl (evaluable dFLC; 86%, n=975). Patients with non-evaluable dFLC had less cardiac involvement (40 vs 80%, P<0.001), less liver involvement (11 vs 17%, P=0.04) and a trend towards less gastrointestinal involvement (18 vs 25%, P=0...
September 18, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28744150/radiofrequency-ablation-using-barrx-%C3%A2-for-the-endoscopic-treatment-of-gastric-antral-vascular-ectasia-a-series-of-three-cases-and-a-review-of-the-literature-on-treatment-options
#3
Anish Patel, Sunil Patel, Prasanna C Wickremesinghe, Deepak Vadada
Gastric antral vascular ectasia (GAVE), also known as "watermelon stomach", is an uncommon condition, which can cause gastrointestinal bleeding due to rupture of blood vessels that line the stomach. The pathogenesis of GAVE remains unclear; however it is thought that hemodynamic changes, mechanical stress, and autoimmune factors all have a part to play. A range of conditions are also commonly associated with the syndrome, such as portal hypertensive gastropathy, liver cirrhosis, and autoimmune disorders. Less commonly, chronic renal failure, cardiac diseases, and bone marrow transplantation have coexisted with GAVE...
2017: Clinical and Experimental Gastroenterology
https://www.readbyqxmd.com/read/28665192/adipose-tissue-derived-stem-cell-sheet-application-for-tissue-healing-in-vivo-a-systematic-review
#4
Panithi Sukho, Abigael Cohen, Jan Willem Hesselink, Jolle Kirpensteijn, Femke Verseijden, Yvonne M Bastiaansen-Jenniskens
Adipose tissue-derived stem cells (ASCs) are known to be tissue-healing promoters due to their cellular plasticity and secretion of paracrine factors. Cultured ASC sheets provide a novel method of ASC application and can retain ASCs at the targeted tissue. The purpose of this systematic review is to evaluate preclinical studies using ASC sheet transplantation therapy for promoting tissue healing. First, we searched databases to identify studies of ASC sheet therapy in different experimental animal models, and then determined the quality score of studies using SYRCLE's risk bias tool...
August 4, 2017: Tissue Engineering. Part B, Reviews
https://www.readbyqxmd.com/read/28652649/bleeding-with-the-artificial-heart-gastrointestinal-hemorrhage-in-cf-lvad-patients
#5
EDITORIAL
Grigoriy E Gurvits, Elena Fradkov
Continuous-flow left ventricular assist devices (CF-LVADs) have significantly improved outcomes for patients with end-stage heart failure when used as a bridge to cardiac transplantation or, more recently, as destination therapy. However, its implantations carries a risk of complications including infection, device malfunction, arrhythmias, right ventricular failure, thromboembolic disease, postoperative and nonsurgical bleeding. A significant number of left ventricular assist devices (LVAD) recipients may experience recurrent gastrointestinal hemorrhage, mainly due to combination of antiplatelet and vitamin K antagonist therapy, activation of fibrinolytic pathway, acquired von Willebrand factor deficiency, and tendency to develop small intestinal angiodysplasias due to increased rotary speed of the pump...
June 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#6
Faisal M Huq Ronny, George Kleinman, Paul James Kurtin, John Thomas Fallon
Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28376837/heartmate-3-fully-magnetically-levitated-left-ventricular-assist-device-for-the-treatment-of-advanced-heart-failure-1%C3%A2-year-results-from-the-ce-mark-trial
#7
Thomas Krabatsch, Ivan Netuka, Jan D Schmitto, Daniel Zimpfer, Jens Garbade, Vivek Rao, Michiel Morshuis, Friedhelm Beyersdorf, Silvana Marasco, Laura Damme, Yuriy Pya
BACKGROUND: The HeartMate 3 Left Ventricular Assist System (LVAS) (St. Jude Medical Inc., St Paul, MN) with full magnetic levitation allows for wide and consistent blood flow paths and an artificial pulse designed for enhanced hemocompatibility. The HeartMate 3 received market approval in the European Union in 2015 following completion of a multicenter study. After reaching the 6-month study endpoint, patients continue to be followed for 2 years with the 1-year results presented herein...
April 4, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28261549/bioengineering-hearts-simple-yet-complex
#8
REVIEW
Doris A Taylor, Rohan B Parikh, Luiz C Sampaio
PURPOSE OF REVIEW: In this review, we focus on the multiple advancements in the field of cardiovascular regenerative medicine and the state-of-the art of building a heart. An organ is comprised of cells, but cells alone do not comprise an organ. We summarize the components needed, the hurdles, and likely translational steps defining the opportunities for discovery. RECENT FINDINGS: The therapies being developed in regenerative medicine aim not only to repair, but also to regenerate or replace ailing tissues and organs...
2017: Current Stem Cell Reports
https://www.readbyqxmd.com/read/28104141/tacrolimus-associated-diffuse-gastrointestinal-ulcerations-and-pathergy-a-case-report
#9
D Pan, O Shaye, J A Kobashigawa
Tacrolimus is a common immunosuppressive modality with a range of therapeutic applications, including for rheumatologic disease, nephrotic syndrome, and inflammatory bowel disease. The medication also plays an integral role in organ transplantation. However, tacrolimus has a significant side effect profile, which commonly includes nephrotoxicity, neurotoxicity, infection risk, and anemia. We describe an unusual case of tacrolimus toxicity in a cardiac transplant patient, manifesting as diffuse gastrointestinal ulcerations and pathergy...
January 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/27881814/chronic-norovirus-infections-in-cardiac-transplant-patients-considerations-for-evaluation-and-management
#10
Paul T Jurgens, Larry A Allen, Amrut V Ambardekar, Colleen K McIlvennan
Norovirus is a common self-limiting gastrointestinal infection, but in transplant recipients, symptoms can last for months and result in serious health complications. As there is currently no established treatment for chronic norovirus infection in transplant patients, management has been directed at symptom control, trial of various antivirals, and ultimately reductions in immunosuppression. We present 3 cases of chronic norovirus infection in cardiac transplant patients to illustrate various approaches to diagnosis, the prolonged nature of disease symptoms, and treatment options...
November 23, 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27864873/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant-is-there-a-right-time-for-liver-transplantation
#11
Yannick D Muller, Roland Oppliger, Romain Breguet, Philippe Meyer, Laura Rubbia-Brandt, Pierre-Auguste Petignat, Thomas Harr, Eric Dayer, Jörg D Seebach
BACKGROUND & AIMS: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. METHODS: Herein, we report the case of a 65-year-old female patient with a 'double Osler syndrome' consisting of hereditary haemorrhagic telangiectasia (HHT) and type I hereditary angioedema diagnosed at the age of 25 and 22 years respectively...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27864871/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant
#12
Carlo Sabbà
The Association of hereditary hemorrhagic telangiectasia (HHT) and type I hereditary angioedema is a very rare condition in medicine. The case reported by Muller et al., describes the coexistence of the two diseases and emphasizes the need for listing HHT patients for liver transplantation in case indications occurs, such as the presence of HCC, abnormally increased cardiac output, and gastrointestinal bleeding as reported for this case. The case described by Mueller et al. is anecdotal for the usefulness of liver transplantation in HHT patients and shows that liver transplantation may be the best supportive care and, sometimes, the unique and final therapeutic option for these patients...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27853374/management-of-extrapulmonary-sarcoidosis-challenges-and-solutions
#13
REVIEW
Khalid Al-Kofahi, Peter Korsten, Christian Ascoli, Shanti Virupannavar, Mehdi Mirsaeidi, Ian Chang, Naim Qaqish, Lesley A Saketkoo, Robert P Baughman, Nadera J Sweiss
BACKGROUND: Sarcoidosis is a chronic multisystem disease of unknown etiology characterized by noncaseating granulomas that most often involves the lungs, but frequently has extrapulmonary manifestations, which might be difficult to treat in individual patients. OBJECTIVE: To review different disease manifestations, focusing on extrapulmonary organ systems, and to provide treatment options for refractory cases. MATERIALS AND METHODS: We performed a literature search using Medline and Google Scholar for individual or combined keywords of "sarcoidosis, extrapulmonary, treatment, kidney, neurosarcoidosis, cardiovascular, gastrointestinal, transplantation, musculoskeletal, rheumatology, arthritis, and skin"...
2016: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/27812432/treatment-of-rapidly-progressive-systemic-sclerosis-current-and-futures-perspectives
#14
Fabian A Mendoza, Maryah Mansoor, Sergio A Jimenez
INTRODUCTION: Systemic Sclerosis (SSc) is a systemic autoimmune disease characterized by severe and often progressive cutaneous, pulmonary, cardiac and gastrointestinal tract fibrosis, cellular and humoral immunologic alterations, and pronounced fibroproliferative vasculopathy. There is no effective SSc disease modifying therapy. Patients with rapidly progressive SSc have poor prognosis with frequent disability and very high mortality. AREAS COVERED: This paper reviews currently available therapeutic approaches for rapidly progressive SSc and discuss novel drugs under study for SSc disease modification...
2016: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/27622143/pregnancy-in-patients-with-mucopolysaccharidosis-a-case-series
#15
Fiona J Stewart, Andrew Bentley, Barbara K Burton, Nathalie Guffon, Susan L Hale, Paul R Harmatz, Susanne G Kircher, Pavan K Kochhar, John J Mitchell, Ursula Plöckinger, Sue Graham, Stephen Sande, Zlatko Sisic, Tracey A Johnston
The mucopolysaccharidoses (MPS disorders) are rare inherited diseases associated with multi-organ accumulation of glycosaminoglycans, leading to musculoskeletal, respiratory, cardiac, neurological, ophthalmological, otolaryngological, and gastrointestinal abnormalities. As a result of improvements in diagnosis, multi-disciplinary care, and therapies such as enzyme replacement therapy and hematopoietic stem cell transplantation, an increasing number of patients with MPS are reaching adulthood and are involved in family planning...
September 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27590926/triheptanoin-treatment-in-patients-with-pediatric-cardiomyopathy-associated-with-long-chain-fatty-acid-oxidation-disorders
#16
J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden
Long-chain fatty acid oxidation disorders (LC-FAOD) can cause cardiac hypertrophy and cardiomyopathy, often presenting in infancy, typically leading to death or heart transplant despite ongoing treatment. Previous data on triheptanoin treatment of cardiomyopathy in LC-FAOD suggested a clinical benefit on heart function during acute failure. An additional series of LC-FAOD patients with critical emergencies associated with cardiomyopathy was treated with triheptanoin under emergency treatment or compassionate use protocols...
November 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27576370/one-year-results-from-a-prospective-randomized-trial-comparing-phlebotomy-with-deferasirox-for-the-treatment-of-iron-overload-in-pediatric-patients-with-thalassemia-major-following-curative-stem-cell-transplantation
#17
RANDOMIZED CONTROLLED TRIAL
Adlette Inati, Mario Kahale, Nada Sbeiti, Maria Domenica Cappellini, Ali T Taher, Suzanne Koussa, Therese A Nasr, Khaled M Musallam, Hussein A Abbas, John B Porter
BACKGROUND: Iron overload is well documented in patients with β-thalassemia major, and patients who have undergone hematopoietic stem cell transplantation (HSCT) remain at risk as a result of pre- and immediate post-HSCT transfusions. PROCEDURE: This is a prospective, randomized, 1-year clinical trial that compares the efficacy and safety of the once-daily oral iron chelator deferasirox versus phlebotomy for the treatment of iron overload in children with β-thalassemia major following HSCT...
January 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27366152/cardiac-arrest-and-gastrointestinal-bleeding-a-case-of-medical-heuristics
#18
Tokunbo Ajayi, Jerome Okudo
Insufficient clinical data from patients is a major cause of errors in medical diagnostics. In an attempt to make a diagnosis, initial clinical information provided to the physician may be overly relied on as the only information required in making diagnosis leading to anchoring. Failure to rely on differential diagnoses in spite of new signs and symptoms or rethinking of initial hypothesis may lead to fixation on a certain diagnosis, which may lead to significant morbidity and mortality. In the event that there is an anchoring heuristic, like in our patient, it is important to consider differential diagnoses; however, it is not wrong to rely on some form of anchor...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27342573/mechanical-circulatory-assist-devices-a-primer-for-critical-care-and-emergency-physicians
#19
REVIEW
Ayan Sen, Joel S Larson, Kianoush B Kashani, Stacy L Libricz, Bhavesh M Patel, Pramod K Guru, Cory M Alwardt, Octavio Pajaro, J Christopher Farmer
Mechanical circulatory assist devices are now commonly used in the treatment of severe heart failure as bridges to cardiac transplant, as destination therapy for patients who are not transplant candidates, and as bridges to recovery and "decision-making". These devices, which can be used to support the left or right ventricles or both, restore circulation to the tissues, thereby improving organ function. Left ventricular assist devices (LVADs) are the most common support devices. To care for patients with these devices, health care providers in emergency departments (EDs) and intensive care units (ICUs) need to understand the physiology of the devices, the vocabulary of mechanical support, the types of complications patients may have, diagnostic techniques, and decision-making regarding treatment...
June 25, 2016: Critical Care: the Official Journal of the Critical Care Forum
https://www.readbyqxmd.com/read/27297224/clinical-features-therapeutic-interventions-and-long-term-aspects-of-hemolytic-uremic-syndrome-in-norwegian-children-a-nationwide-retrospective-study-from-1999-2008
#20
Gaute Reier Jenssen, Line Vold, Eirik Hovland, Hans-Jacob Bangstad, Karin Nygård, Anna Bjerre
BACKGROUND: Hemolytic-uremic syndrome (HUS) is a clinical triad of microangiopathic hemolytic anemia, impaired renal function and thrombocytopenia, primarily affecting pre-school-aged children. HUS can be classified into diarrhea-associated HUS (D(+)HUS), usually caused by Shiga toxin-producing Escherichia coli (STEC), and non-diarrhea-associated HUS (D(-)HUS), both with potentially serious acute and long-term complications. Few data exists on the clinical features and long-term outcome of HUS in Norway...
June 13, 2016: BMC Infectious Diseases
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