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https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#1
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28430830/assessment-of-global-kidney-health-care-status
#2
Aminu K Bello, Adeera Levin, Marcello Tonelli, Ikechi G Okpechi, John Feehally, David Harris, Kailash Jindal, Babatunde L Salako, Ahmed Rateb, Mohamed A Osman, Bilal Qarni, Syed Saad, Meaghan Lunney, Natasha Wiebe, Feng Ye, David W Johnson
Importance: Kidney disease is a substantial worldwide clinical and public health problem, but information about available care is limited. Objective: To collect information on the current state of readiness, capacity, and competence for the delivery of kidney care across countries and regions of the world. Design, Setting, and Participants: Questionnaire survey administered from May to September 2016 by the International Society of Nephrology (ISN) to 130 ISN-affiliated countries with sampling of key stakeholders (national nephrology society leadership, policy makers, and patient organization representatives) identified by the country and regional nephrology leadership through the ISN...
April 21, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28430300/left-ventricular-assist-pump-pocket-infection-conservative-treatment-strategy-for-destination-therapy-candidates
#3
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/28429043/transsplenic-endovascular-recanalization-and-stenting-of-a-completely-occluded-portal-vein-with-jejunal-variceal-embolization-in-a-pediatric-liver-transplant-recipient
#4
Jeffrey Forris Beecham Chick, Alexandria Jo, Narasimham Dasika, Wael E Saad, Ravi Nara Srinivasa
Portal vein thrombosis occurs in 1.4% of pediatric liver transplant candidates and 3.7% of liver transplant recipients. While portal vein recanalization without and with portal vein stenting has been described in adult transplant candidates and recipients, it has never been described in the pediatric transplant population. This report presents a pediatric liver transplant recipient with portal hypertension secondary to portal vein thrombosis successfully managed with transsplenic access and subsequent portal vein recanalization and stenting...
April 20, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28428885/the-why-what-and-how-of-the-new-fact-standards-for-immune-effector-cells
#5
EDITORIAL
Marcela V Maus, Sarah Nikiforow
Novel cellular therapies outside of traditional hematopoietic stem cell transplantation or hematopoietic progenitor cell (HPC) therapy are currently under evaluation in clinical trials across the United States and around the world. Several cellular products, e.g., CD19-directed Chimeric Antigen Receptor (CAR) T cells, are poised for FDA approval and thus increased use at a wider range of academic centers within the next year, with the likelihood of dissemination to standard oncology practice once safety is confirmed...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28427529/primary-central-nervous-system-lymphoma
#6
REVIEW
Giovanni Citterio, Michele Reni, Gemma Gatta, Andrés José Maria Ferreri
Primary CNS lymphomas (PCNSL) represent a subgroup of malignancies with specific characteristics, aggressive course, and unsatisfactory outcome in contrast with other lymphomas comparable for tumour burden and/or histological type. Despite a high chemo- and radiosensitivity, remissions are frequently shortlasting, mainly because the blood brain-barrier limits the access of many drugs to the CNS. Moreover, survivor patients are at high risk of developing severe treatment-related toxicity, mainly disabling neurotoxicity, raising the question of how to balance therapy intensification with side-effects control...
May 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28425171/how-is-continuity-of-care-experienced-by-people-living-with-chronic-kidney-disease
#7
Sarah Brand, Kristian Pollock
AIMS AND OBJECTIVES: To explore patients' perceptions of continuity of care within a hospital based specialist service. BACKGROUND: Patient journeys through healthcare are becoming increasingly complex. For patients with chronic conditions, the longevity of their illness and common multiple co-morbities make this complexity more pronounced. Continuity of care is most challenging to provide for these patients. A multifaceted model of continuity is widely accepted, but despite this, much literature focuses exclusively on relational aspects...
April 20, 2017: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/28419938/direct-acting-antivirals-the-endgame-for-hepatitis-c
#8
REVIEW
Roberta D'Ambrosio, Elisabetta Degasperi, Massimo Colombo, Alessio Aghemo
Directly-acting antivirals (DAA) have finally allowed all patients to be potentially cured from chronic hepatitis C (HCV) infection. All-oral, Interferon (IFN)-free regimens are based upon the combination of molecules targeting different sites of the HCV replication process. Three classes of DAA exist: protease inhibitors (anti-NS3/4A), RNA-dependent polymerase inhibitors (anti-NS5B) and anti-NS5A inhibitors, which are characterized by different antiviral potency and barrier to resistance and therefore are usually combined in different treatment schedules...
April 15, 2017: Current Opinion in Virology
https://www.readbyqxmd.com/read/28417431/robotic-kidney-transplantation-an-update
#9
REVIEW
V Sankaran, S Sinha
PURPOSE OF REVIEW: Over the last decade, there have been advances in kidney transplantation with introduction of minimally invasive surgery. Robotic surgery is becoming increasingly common across the specialities. There is now increasing experience in robotic kidney transplantation, though it remains a niche procedure. Initial reports suggest that this is a safe, feasible operation when performed by teams familiar with robotic surgery. There have been a few modifications to the initially described procedure, as a result of increasing experience...
June 2017: Current Urology Reports
https://www.readbyqxmd.com/read/28412517/challenges-around-access-to-and-cost-of-life-saving-medications-after-allogeneic-hematopoietic-cell-transplantation-for-medicare-patients
#10
Stephanie Farnia, Alex Ganetsky, Alicia Silver, Theresa Hwee, Jaime Preussler, Joan Griffin, Nandita Khera
Hematopoietic cell transplantation (HCT) is an expensive, medically complicated and potentially life-threatening therapy for multiple hematologic and non-hematologic disorders with a prolonged trajectory of recovery. Similar to financial issues in other cancer treatments, adverse financial consequences of HCT are emerging as an important issue, and may be associated with poor quality of life (QOL) and increased distress in HCT survivors. Prescription medicine coverage for HCT for Medicare and some Medicaid beneficiaries, especially in the long-term remains suboptimal due to inadequate payer formularies or prohibitive co-pays...
April 12, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28410647/disease-trajectories-before-esrd-implications-for-clinical-management
#11
REVIEW
Keiichi Sumida, Csaba P Kovesdy
One of the biggest challenges nephrologists face is to predict complex clinical pathways of kidney disease progression that do not follow a steady linear decline. This unpredictability often becomes a barrier to timely shared decision making between patients and physicians and could lead to adverse patient outcomes. By using numerous analytic approaches to characterize the longitudinal changes in kidney function, emerging evidence has disclosed the heterogeneous trajectories of kidney disease progression, particularly in late-stage chronic kidney disease (CKD) nearing end-stage renal disease (ESRD), and showed their independent associations with various clinical outcomes...
March 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28405876/laparoscopic-sleeve-gastrectomy-in-kidney-transplant-candidates-a-case-series
#12
Sergio Carandina, Laurent Genser, Manuela Bossi, Laura Montana, Alexandre Cortes, Marie Seman, Marc Danan, Christophe Barrat
BACKGROUND: Patients with a body mass index (BMI) >35 kg/m(2) who need kidney transplant present with increased postoperative mortality and reduced kidney graft survival compared to patients with a lower BMI. For this reason, obese patients are often excluded from the transplantation waiting list. The aim of this study was to evaluate the feasibility and the results of laparoscopic sleeve gastrectomy (LSG) for obese patients awaiting a kidney transplant. METHODS: This was a retrospective study on patients with dialysis-dependent renal failure (DDRF) operated on at two first-level bariatric centers in Paris (France)...
April 12, 2017: Obesity Surgery
https://www.readbyqxmd.com/read/28401575/new-insights-into-the-neural-differentiation-potential-of-canine-adipose-tissue-derived-mesenchymal-stem-cells
#13
D Blecker, M I Elashry, M Heimann, S Wenisch, S Arnhold
Adipose tissue-derived stem cells (ASCs) can be obtained from different adipose tissue sources within the body. It is an abundant cell pool, easily accessible, suitable for cultivation and expansion in vitro and preparation for therapeutic approaches. Amongst these therapeutic approaches are tissue engineering and nervous system disorders such as spinal cord injuries. For such treatment, ASCs have to be reliably differentiated in to the neuronal direction. Therefore, we investigated the neural differentiation potential of ASCs using protocols with neurogenic inductors such as valproic acid and forskolin, while dog brain tissue served as control...
April 11, 2017: Anatomia, Histologia, Embryologia
https://www.readbyqxmd.com/read/28396108/augmented-nurse-care-management-in-ckd-stages-4-to-5-a%C3%A2-randomized-trial
#14
Steven Fishbane, Sofia Agoritsas, Alessandro Bellucci, Candice Halinski, Hitesh H Shah, Vipul Sakhiya, Leah Balsam
BACKGROUND: Outcomes for patients with late-stage chronic kidney disease (CKD) in the United States are suboptimal. There is poor education and preparation for end-stage kidney disease, as well as a high rate of hospitalization in this group of patients. STUDY DESIGN: A randomized, parallel-group, 2-arm, controlled trial. SETTING & PARTICIPANTS: The study was conducted at 3 sites: a clinic of an academic medical center, a public hospital academic clinic, and a community-based private practice...
April 7, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28390101/geographic-disparities-in-liver-availability-accidents-of-geography-or-consequences-of-poor-social-policy
#15
Keren Ladin, Gregory Zhang, Douglas W Hanto
Recently, a redistricting proposal intended to equalize MELDs recommended expanding liver sharing to mitigate geographic variation in liver transplantation. Yet, it is unclear whether variation in liver availability is arbitrary and a disparity requiring rectification, or whether it reflects differences in access to care. We evaluate the proposal's claim that organ supply is an "accident of geography" by examining the relationship between local organ supply and the uneven landscape of social determinants and policies that contribute to differential death rates across the United States...
April 8, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28389288/predictors-of-autogenous-arteriovenous-hemodialysis-access-thrombosis-after-renal-transplantation
#16
Sabrina Ben Ahmed, Mohamed Hadj-Abdelkader, Marie Benezit, Patrice Deteix, Anne-Elisabeth Heng, Eugenio Rosset
OBJECTIVES: The fate of autogenous arteriovenous fistula (aAVF) after renal transplantation (RT) remains variable. The aim of this study was to determine the predictors for their thrombosis after renal transplantation. METHODS: We conducted a monocentric retrospective review of prospective clinical records of 145 patients with a functional aAVF who had a RT between January 2004 and December 2009 in the University Hospital of Clermont-Ferrand. Our primary endpoint was the thrombosis of the aAVF...
April 4, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28386787/prospective-evaluation-of-systematic-use-of-peripherally-inserted-central-catheters-picc-lines-for-the-home-care-after-allogeneic-hematopoietic-stem-cells-transplantation
#17
J Cornillon, J A Martignoles, E Tavernier-Tardy, M Gire, P Martinez, C Tranchan, A Vallard, K Augeul-Meunier, B Hacquard, D Guyotat
PURPOSE: Long-term catheters are often necessary for outpatient care after an allogeneic hematopoietic stem cell transplantation (HSCT), However, there is paucity of data on the use of peripherally inserted central catheter (PICC) in post-HSCT setting. METHODS: We prospectively evaluated the systematic use of PICC in 37 consecutive patients returning home after HSCT. RESULTS: In 6 out of 37 patients, the PICC was exclusively used for weekly blood controls...
April 6, 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/28386285/mesenchymal-stem-cells-transplantation-following-partial-hepatectomy-a-new-concept-to-promote-liver-regeneration-systematic-review-of-the-literature-focused-on-experimental-studies-in-rodent-models
#18
REVIEW
Ioannis G Papanikolaou, Charalambos Katselis, Konstantinos Apostolou, Themistoklis Feretis, Maria Lymperi, Manousos M Konstadoulakis, Apostolos E Papalois, George C Zografos
Mesenchymal stem cells (MSCs) are an attractive source for regenerative medicine because they are easily accessible through minimally invasive methods and have the potential to enhance liver regeneration (LG) and improve liver function, following partial hepatectomy (PH) and acute or chronic liver injury. A systematic review of the literature was conducted for articles published up to September 1st, 2016, using the MEDLINE database. The keywords that were used in various combinations were as follows: "Mesenchymal stem cells", "transplantation", "stem cells", "adipose tissue derived stem cells", "bone marrow-derived stem cells", "partial hepatectomy", "acute liver failure", "chronic liver failure", "liver fibrosis", "liver cirrhosis", "rats", "mice", and "liver regeneration"...
2017: Stem Cells International
https://www.readbyqxmd.com/read/28383177/low-socioeconomic-status-adversely-effects-dialysis-survival-in-australia
#19
Rathika Krishnasamy, Nicholas A Gray
BACKGROUND: Low socioeconomic status (SES) is associated with increased incidence of end stage kidney disease and in the USA poorer dialysis survival. All Australians have access to a universal health care system. METHODS: The study included all non-indigenous adult Australians registered with the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA) who commenced dialysis between 2003 and 2013. SES at dialysis start was classified into quartiles of advantaged through to disadvantaged using Australian Bureau of Statistics socio-economic indexes for areas...
April 6, 2017: Nephrology
https://www.readbyqxmd.com/read/28379431/barriers-to-living-donor-kidney-transplantation-in-the-united-kingdom-a-national-observational-study
#20
Diana A Wu, Matthew L Robb, Christopher J E Watson, John L R Forsythe, Charles R V Tomson, John Cairns, Paul Roderick, Rachel J Johnson, Rommel Ravanan, Damian Fogarty, Clare Bradley, Andrea Gibbons, Wendy Metcalfe, Heather Draper, Andrew J Bradley, Gabriel C Oniscu
Background.: Living donor kidney transplantation (LDKT) provides more timely access to transplantation and better clinical outcomes than deceased donor kidney transplantation (DDKT). This study investigated disparities in the utilization of LDKT in the UK. Methods.: A total of 2055 adults undergoing kidney transplantation between November 2011 and March 2013 were prospectively recruited from all 23 UK transplant centres as part of the Access to Transplantation and Transplant Outcome Measures (ATTOM) study...
March 31, 2017: Nephrology, Dialysis, Transplantation
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