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Cardiac transplant AND gastrointestinal

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
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...
August 27, 2016: Molecular Genetics and Metabolism
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
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
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
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
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
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
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
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
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
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
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
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
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)
Francesca Gay, Stefania Oliva, Maria Teresa Petrucci, Concetta Conticello, Lucio Catalano, Paolo Corradini, Agostina Siniscalchi, Valeria Magarotto, Luděk Pour, Angelo Carella, Alessandra Malfitano, Daniela Petrò, Andrea Evangelista, Stefano Spada, Norbert Pescosta, Paola Omedè, Philip Campbell, Anna Marina Liberati, Massimo Offidani, Roberto Ria, Stefano Pulini, Francesca Patriarca, Roman Hajek, Andrew Spencer, Mario Boccadoro, Antonio Palumbo
BACKGROUND: High-dose melphalan plus autologous stem-cell transplantation (ASCT) is the standard approach in transplant-eligible patients with newly diagnosed myeloma. Our aims were to compare consolidation with high-dose melphalan plus ASCT versus chemotherapy (cyclophosphamide and dexamethasone) plus lenalidomide, and maintenance with lenalidomide plus prednisone versus lenalidomide alone. METHODS: We did an open-label, randomised, multicentre, phase 3 study at 59 centres in Australia, Czech Republic, and Italy...
December 2015: Lancet Oncology
Zuoyi Wang, J J He, X Y Liu, Bingyi Lin, Lin Zhang, Lei Geng, Shusen Zheng
OBJECTIVE: The management of liver receivers requires intense immunosuppression to prevent graft rejection. Mycophenolate mofetil (MMF) is a common immunosuppressant but frequently suffer dose reduction for gastrointestinal adverse reactions (GI). Hence, the enteric-coated mycophenolate sodium (EC-MPS) is introduced as a substitute for MMF to reduce GI. The study was designed to investigate the efficacy, safety and exposure equation of EC-MPS in liver transplant patients in China. METHODS: Ninety-two liver receivers who administered EC-MPS or MMF as a primary immunosuppressant were enrolled in this single-center study and divided into MMF group and EC-MPC group, respectively...
2015: Immunopharmacology and Immunotoxicology
Patrick R Vargo, Nicholas K Schiltz, Douglas R Johnston, Nicholas G Smedira, Nader Moazami, Eugene H Blackstone, Edward G Soltesz
A growing number of solid organ transplant survivors require surgery for cardiac disease. We examined the effect of having a previous transplant on outcomes after cardiac surgery in these patients from a population-based perspective. Of 1,709,735 patients who underwent coronary artery bypass grafting, valve, or thoracic aorta surgery from 2004 to 2008 in the Nationwide Inpatient Sample, 3,535 patients (0.21%) had a previous organ transplant (2,712 kidney, 738 liver, 300 pancreas). Multivariate logistic regression analysis and propensity score matching were used to determine the effect of a previous solid organ transplant on outcomes...
December 15, 2015: American Journal of Cardiology
Christopher T Holley, Laura Harvey, Samit S Roy, Rebecca Cogswell, Peter Eckman, Kenneth Liao, Ranjit John
Gastrointestinal bleeding (GIB) remains a significant problem after continuous-flow left ventricular assist device (CF-LVAD) implantation. We hypothesized that the subsequent need for blood transfusions in patients with GIB may reduce rates of cardiac transplantation. We performed a retrospective review of 232 patients implanted with the HeartMate II (HM II) CF-LVAD from June 2005 through May 2013 at our center to determine risk factors for GIB and assess its effect on cardiac transplantation. Over a total LVAD follow-up time of 364 person-years, 62 GIB episodes occurred in 49 patients (27%), for an event rate of 0...
November 2015: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Paul Waltz, Daniel Escobar, Ana Maria Botero, Brian S Zuckerbraun
SIGNIFICANCE: Nitric oxide (NO) is a critical signaling molecule marked by complex chemistry and varied biological responses depending on the context of the redox environment. In the setting of inflammation, NO can not only contribute to tissue injury and be causative of oxidative damage but can also signal as an adaptive molecule to limit inflammatory signaling in multiple cell types and tissues. RECENT ADVANCES: An advance in our understanding of NO biology was the recognition of the nitrate-nitrite-NO axis, whereby nitrate (predominantly from dietary sources) could be converted to nitrite and nitrite could be reduced to NO...
August 1, 2015: Antioxidants & Redox Signaling
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