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Cardiac transplant AND gastrointestinal

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https://www.readbyqxmd.com/read/27881814/chronic-norovirus-infections-in-cardiac-transplant-patients-considerations-for-evaluation-and-management
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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...
August 31, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27366152/cardiac-arrest-and-gastrointestinal-bleeding-a-case-of-medical-heuristics
#9
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
#10
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
#11
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...
2016: BMC Infectious Diseases
https://www.readbyqxmd.com/read/27240092/sublingual-administration-improves-systemic-exposure-of-tacrolimus-in-kidney-transplant-recipients-comparison-with-oral-administration
#12
Stefano Federico, Rosa Carrano, Massimo Sabbatini, Riccardo Nappi, Luigi Russo, Luca Apicella, Mario Maria Balletta, Michele Santangelo, Teresa Mosca, Giovanni Tarantino, Domenico Capone
BACKGROUND: Tacrolimus (TCR) is an immunosuppressive drug used by oral administration. Intravenous (IV) TCR administration is required under conditions of gastrointestinal diseases or abdominal surgery at the onset of paralytic ileus. The infusion formulation needs a large dilution and therefore a careful technical management during continuous infusion by 24 h and may determine anaphylaxis, cardiac arrhythmia, QT prolongation and torsades de pointes. Sublingual (SL) TCR administration was suggested as an alternative route...
July 2016: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27173072/outcomes-on-continuous-flow-left-ventricular-assist-devices-a-single-institutional-9-year-experience
#13
Jeffrey A Morgan, Pauline H Go, Linnea Xuereb, Babbaljeet Kaur, Silvy Akrawe, Hassan W Nemeh, Jamil Borgi, David E Lanfear, Celeste T Williams, Gaetano Paone
BACKGROUND: Continuous-flow left ventricular assist devices (LVADs) have become the standard of care for patients with advanced heart failure. The goal of this study was to review our 9-year institutional experience. METHODS: From March 2006 through May 2015, 231 patients underwent implantation of 240 CF LVADs, HeartMate II LVAD (Thoratec Corp., Pleasanton, CA; n = 205) or HVAD (HeartWare Inc., Framingham, MA; n = 35). Of these, 127 devices (52.9%) were implanted as bridge to transplantation (BTT) and 113 (47...
October 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27154148/timing-and-frequency-of-unplanned-readmissions-after-lung-transplantation-impact-long-term-survival
#14
Entela Lushaj, Walker Julliard, Shahab Akhter, Glen Leverson, James Maloney, Richard D Cornwell, Keith C Meyer, Nilto DeOliveira
BACKGROUND: Adverse events that require hospital readmission frequently occur long after lung transplantation (LT) that has been successfully performed. We sought to identify the causes and rate of unplanned readmissions after LT and to determine whether unplanned readmissions have a significant impact on post-LT survival. METHODS: We retrospectively reviewed the outcomes in 174 LT recipients who underwent LT at our center from June 2005 to May 2014. The median follow-up period was 38 months (range, 17 to 72 months)...
August 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27132122/parenteral-nutrition-improves-nutritional-status-autonomic-symptoms-and-quality-of-life-in-transthyretin-amyloid-polyneuropathy
#15
Massimo Russo, Gian Luca Vita, Claudia Stancanelli, Anna Mazzeo, Giuseppe Vita, Sonia Messina
Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an inherited amyloidosis, leading to death in about ten years in most cases due to cardiac failure or wasting syndrome. Previous studies showed that modified body mass index was related to time before death, duration of gastrointestinal disturbances, malabsorption and functional capacity. We report two patients in whom nutritional status worsened despite diet modification, hypercaloric supplement and two relevant therapeutic approaches such as liver transplant and tafamidis meglumine, respectively...
June 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27109981/ten-year-experience-with-bowel-transplantation-at-seoul-st-mary-s-hospital
#16
H K Chang, S Y Kim, J I Kim, S I Kim, J K Whang, J Y Choi, J M Park, E S Jung, S E Rha, D G Kim, I S Moon, M D Lee
A retrospective review of intestinal transplantation (ITx) at Seoul St. Mary's Hospital was made by collecting clinical data over the past 10 years. Fifteen consecutive cases from 2004 were analyzed. Five children and 10 adults (6 months to 69 years of age) were included. Primary diseases in adults included 4 mesenteric vessel thromboses, 2 strangulations, and 1 each of visceral myopathy, malignant gastrointestinal stromal tumor (GIST), mesenteric lymphangiectasis, and injury. Pediatric cases involved 2 Hirschsprung disease, 2 visceral myopathy, and 1 necrotizing enterocolitis...
March 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27060043/is-four-stage-management-the-future-of-univentricular-hearts-destination-therapy-in-the-young
#17
REVIEW
Robert D B Jaquiss, Hamza Aziz
For the child born with a functionally univentricular heart, multi-stage surgical palliation culminating in the Fontan operation is now well established as the standard of care. Though this is an effective approach, there are several inherent disadvantages to the Fontan circulation: subnormal resting cardiac output, impaired exercise capacity, increased risk of thromboembolism, and increased risk of arrhythmia. Perhaps most importantly, the long-term deleterious effects of substantial systemic venous hypertension, which is necessary to "drive" blood through the pulmonary vascular bed, include progressive dysfunction of other organ systems, particularly renal, lymphatic, gastrointestinal, and hepatic systems...
2016: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/27022892/the-future-of-mechanical-circulatory-support-for-advanced-heart-failure
#18
Karolina K Marinescu, Nir Uriel, Sirtaz Adatya
PURPOSE OF REVIEW: Mechanical circulatory support (MCS) has become the main focus of heart replacement therapy for end stage heart failure patients. Advances in technology are moving towards miniaturization, biventricular support devices, complete internalization, improved hemocompatibility profiles, and responsiveness to cardiac loading conditions. This review will discuss the recent advances and investigational devices in MCS for advanced heart failure. RECENT FINDINGS: The demand for both short-term and long-term durable devices for advanced heart failure is increasing...
May 2016: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/27011917/pentamidine-in-pneumocystis-jirovecii-prophylaxis-in-heart-transplant-recipients
#19
REVIEW
Adem Ilkay Diken, Ozlem Erçen Diken, Onur Hanedan, Seyhan Yılmaz, Ata Niyazi Ecevit, Emir Erol, Adnan Yalçınkaya
Despite advances in transplantation techniques and the quality of post-transplantation care, opportunistic infections remain an important cause of complications. Pneumocystis jirovecii (P. jirovecii) is an opportunistic organism, represents an important cause of infections in heart transplantation patients. Almost 2% to 10% of patients undergoing cardiac transplantation have Pneumocystis pneumonia. Prophylaxis is essential after surgery. Various prophylaxis regimes had been defined in past and have different advantages...
March 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/26886643/child-to-adult-liver-transplantation-with-donation-after-cardiac-death-donors-three-case-reports
#20
Liangshuo Hu, Xuemin Liu, Xiaogang Zhang, Liang Yu, Huanchen Sha, Ying Zhou, Min Tian, Jianhua Shi, Wanli Wang, Chang Liu, Kun Guo, Yi Lv, Bo Wang
Development of organ transplantation is restricted by the discrepancy between the lack of donors and increasing number of patients. The outcome of pediatric donors transplanted into adult recipients especially with donation after circulatory death (DCD) pattern has not been well studied. The aim of this paper is to describe our experience of 3 successful DCD donor child-to-adult liver transplantations lately. Three DCD donors were separately 7, 5, and 8 years old. The ratio between donor graft weight and recipient body weight was 1...
February 2016: Medicine (Baltimore)
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