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https://www.readbyqxmd.com/read/29329518/the-use-of-eculizumab-in-gemcitabine-induced-thrombotic-microangiopathy
#1
Vinod Krishnappa, Mohit Gupta, Haikoo Shah, Abhijit Das, Natthavat Tanphaichitr, Robert Novak, Rupesh Raina
BACKGROUND: Thrombotic microangiopathy (TMA) secondary to gemcitabine therapy (GiTMA) is a very rare pathology that carries a poor prognosis, with nearly half of the cases progressing to end stage renal disease. GiTMA is most commonly associated with adenocarcinomas, most notably pancreatic cancers. The mainstay of management is withdrawal of the offending drug and supportive care. Plasmapheresis has a limited role and hemodialysis may help in the management of fluid overload secondary to renal failure...
January 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29326807/heavy-chain-diseases-and-myeloma-associated-fanconi-syndrome-an-update
#2
REVIEW
Roberto Ria, Franco Dammacco, Angelo Vacca
The heavy chain diseases (HCDs) are rare B-cell malignancies characterized by the production of a monoclonal immunoglobulin heavy chain without an associated light chain. There are three types of HCD, defined by the class of immunoglobulin heavy chain produced: IgA (α-HCD), IgG (γ-HCD), and IgM (μ-HCD). Alpha-HCD is the most common and usually occurs as intestinal malabsorption in a young adult from a country of the Mediterranean area. Gamma- and μ-HCDs are rarer and associated with a B-cell non-Hodgkin lymphoma that produces an abnormal Ig heavy chain...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29323186/combination-of-high-fat-high-fructose-diet-and-low-dose-streptozotocin-to-model-long-term-type-2-diabetes-complications
#3
David André Barrière, Christophe Noll, Geneviève Roussy, Farah Lizotte, Anissa Kessai, Karyn Kirby, Karine Belleville, Nicolas Beaudet, Jean-Michel Longpré, André C Carpentier, Pedro Geraldes, Philippe Sarret
The epidemic of type 2 diabetes mellitus (T2DM) is fueled by added fructose consumption. Here, we thus combined high-fat/high-fructose diet, with multiple low-dose injections of streptozotocin (HF/HF/Stz) to emulate the long-term complications of T2DM. HF/HF/Stz rats, monitored over 56 weeks, exhibited metabolic dysfunctions associated with the different stages of the T2DM disease progression in humans: an early prediabetic phase characterized by an hyperinsulinemic period with modest dysglycemia, followed by a late stage of T2DM with frank hyperglycemia, normalization of insulinemia, marked dyslipidemia, hepatic fibrosis and pancreatic β-cell failure...
January 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29322327/combined-and-sequential-liver-kidney-transplantation-in-children
#4
Ryszard Grenda, Piotr Kaliciński
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms...
January 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29322198/association-of-cardiovascular-biomarkers-with-incident-heart-failure-with-preserved-and-reduced-ejection-fraction
#5
Rudolf A de Boer, Matthew Nayor, Christopher R deFilippi, Danielle Enserro, Vijeta Bhambhani, Jorge R Kizer, Michael J Blaha, Frank P Brouwers, Mary Cushman, Joao A C Lima, Hossein Bahrami, Pim van der Harst, Thomas J Wang, Ron T Gansevoort, Caroline S Fox, Hanna K Gaggin, Willem J Kop, Kiang Liu, Ramachandran S Vasan, Bruce M Psaty, Douglas S Lee, Hans L Hillege, Traci M Bartz, Emelia J Benjamin, Cheeling Chan, Matthew Allison, Julius M Gardin, James L Januzzi, Sanjiv J Shah, Daniel Levy, David M Herrington, Martin G Larson, Wiek H van Gilst, John S Gottdiener, Alain G Bertoni, Jennifer E Ho
Importance: Nearly half of all patients with heart failure have preserved ejection fraction (HFpEF) as opposed to reduced ejection fraction (HFrEF), yet associations of biomarkers with future heart failure subtype are incompletely understood. Objective: To evaluate the associations of 12 cardiovascular biomarkers with incident HFpEF vs HFrEF among adults from the general population. Design, Setting, and Participants: This study included 4 longitudinal community-based cohorts: the Cardiovascular Health Study (1989-1990; 1992-1993 for supplemental African-American cohort), the Framingham Heart Study (1995-1998), the Multi-Ethnic Study of Atherosclerosis (2000-2002), and the Prevention of Renal and Vascular End-stage Disease study (1997-1998)...
January 10, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/29319775/primary-hiperoxaluria-diagnosed-after-kidney-transplantation-report-of-2-cases-and-literature-review
#6
John Fredy Nieto Rios, Monica Zuluaga, Lina Maria Serna Higuita, Adriana Florez, Diana Carolina Bello-Marquez, Arbey Aristizábal, Catalina Ocampo Kohn, Gustavo Adolfo Zuluaga
Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29318530/a-novel-heterozygous-gli2-mutation-in-a-patient-with-congenital-urethral-stricture-and-renal-hypoplasia-dysplasia-leading-to-end-stage-renal-failure
#7
Toshihiko Shirakawa, Yumiko Nakashima, Satoshi Watanabe, Sadatomo Harada, Mariko Kinoshita, Toshiharu Kihara, Yuko Hamasaki, Seiichiro Shishido, Koh-Ichiro Yoshiura, Hiroyuki Moriuchi, Sumito Dateki
Glioblastoma 2 (GLI2) is a mediator of Sonic hedgehog signaling pathway that plays an important role in development of the central nervous system and limbs. Heterozygous GLI2 mutations have been associated with postaxial polydactyly, various pituitary dysfunction, and holoprosencephaly-like phenotype. Herein, we report a Japanese boy who presented with isolated growth hormone deficiency with ectopic posterior pituitary, postaxial polydactyly, atrioventricular septal defect, intellectual disability and dysmorphic facial features including mid-facial hypoplasia...
January 9, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29315693/nacseld-acute-on-chronic-liver-failure-nacseld-aclf-score-predicts-30-day-survival-in-hospitalized-patients-with-cirrhosis
#8
Jacqueline G O'Leary, K Rajender Reddy, Guadalupe Garcia-Tsao, Scott W Biggins, Florence Wong, Michael B Fallon, Ram M Subramanian, Patrick S Kamath, Paul Thuluvath, Hugo E Vargas, Benedict Maliakkal, Puneeta Tandon, Jennifer Lai, Leroy R Thacker, Jasmohan S Bajaj
NACSELD (North American Consortium for the Study of End-Stage Liver Disease) definition of acute-on-chronic liver failure (NACSELD-ACLF) as ≥2 extra-hepatic organ failures has been proposed as a simple bedside tool to assess risk of mortality in hospitalized patients with cirrhosis. We validated NACSELD-ACLF's ability to predict 30-day survival (defined as in-hospital death or hospice discharge) in a separate multicenter prospectively enrolled cohort of both infected and uninfected hospitalized patients with cirrhosis...
January 8, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29313137/fungal-peritonitis-in-the-standardizing-care-to-improve-outcomes-in-pediatric-end-stage-renal-disease-scope-collaborative
#9
Raj Munshi, Christine B Sethna, Troy Richardson, Jonathan Rodean, Samhar Al-Akash, Sushil Gupta, Alicia M Neu, Bradley A Warady
BACKGROUND: Fungal peritonitis is a serious complication among peritoneal dialysis (PD) patients. The Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative is a North American multicenter quality improvement initiative with the primary aim to reduce catheter-related infections in children on chronic dialysis. OBJECTIVE: To describe the epidemiology of fungal peritonitis and outcomes of affected patients among pediatric subjects receiving chronic PD and enrolled in SCOPE...
January 8, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29312859/recurrence-of-primary-glomerulonephritis-review-of-the-current-evidence
#10
REVIEW
Fedaey Abbas, Mohsen El Kossi, Jon Kim Jin, Ajay Sharma, Ahmed Halawa
In view of the availability of new immunosuppression strategies, the recurrence of allograft glomerulonephritis (GN) are reported to be increasing with time post transplantation. Recent advances in understanding the pathogenesis of the GN recurrent disease provided a better chance to develop new strategies to deal with the GN recurrence. Recurrent GN diseases manifest with a variable course, stubborn behavior, and poor response to therapy. Some types of GN lead to rapid decline of kidney function resulting in a frustrating return to maintenance dialysis...
December 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/29301167/acute-kidney-injury-after-total-knee-arthroplasty-a-clinical-review
#11
Ahmed Siddiqi, Peter B White, Jennifer I Etcheson, Nicole E George, Chukwuweike U Gwam, Jaydev B Mistry, Nirav G Patel, Hephizibah Adam, Ronald E Delanois
INTRODUCTION: Patients who develop acute kidney injury (AKI) have an increased risk for progression to chronic kidney disease, end-stage renal disease, and increased mortality. The outcomes of total knee arthroplasty (TKA) patients who develop AKI have remained controversial. The purpose of this review was to summarize and identify the current literature focused on 1) major risk factors, 2) short-term outcomes, and 3) costs associated with the development of perioperative AKI after TKA...
December 22, 2017: Surgical Technology International
https://www.readbyqxmd.com/read/29299604/association-of-acylcarnitines-with-left-ventricular-remodeling-in-patients-with-severe-aortic-stenosis-undergoing-transcatheter-aortic-valve-replacement
#12
Sammy Elmariah, Laurie A Farrell, Deborah Furman, Brian R Lindman, Xu Shi, Jordan E Morningstar, Eugene P Rhee, Robert E Gerszten
Importance: Clinical practice guidelines currently endorse a reliance on clinical symptoms of overt left ventricular (LV) failure to time aortic valve replacement for severe aortic stenosis; however, delayed aortic valve replacement can result in irreversible LV injury and adverse outcomes. Blood metabolomic signatures possess prognostic value in heart failure; this study assesses whether they are informative in aortic stenosis. Objective: To evaluate the value of metabolomic signatures in reflecting the extent of maladaptive LV remodeling in patients with end-stage aortic stenosis undergoing transcatheter aortic valve replacement, and to assess whether this procedure reverses metabolomic aberrations...
January 3, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/29298216/the-utility-of-myocardial-perfusion-imaging-before-renal-transplantation-a-retrospective-analysis
#13
Paul D Callan, Sunil Bhandari, Andrew L Clark, David Eadington, Emmanouil Papadopoulos, Ann C Tweddel
BACKGROUND: Renal transplantation (RT) reduces morbidity and mortality in patients with end-stage renal failure. Myocardial perfusion imaging provides prognostic information in patients with renal failure, but its role before transplantation remains unclear. We performed a retrospective review assessing the prognostic value of technetium-99m sestamibi myocardial perfusion imaging at a tertiary UK centre. PATIENTS AND METHODS: We included scans performed between 2005 and 2012...
January 2, 2018: Nuclear Medicine Communications
https://www.readbyqxmd.com/read/29296707/cyclophosphamide-improves-engraftment-in-patients-with-scd-and-severe-organ-damage-who-undergo-haploidentical-pbsct
#14
Courtney D Fitzhugh, Matthew M Hsieh, Tiffani Taylor, Wynona Coles, Katherine Roskom, Delon Wilson, Elizabeth Wright, Neal Jeffries, Christopher J Gamper, Jonathan Powell, Leo Luznik, John F Tisdale
Peripheral blood stem cell transplantation (PBSCT) offers a curative option for sickle cell disease (SCD). Although HLA-matched sibling transplantation is promising, the vast majority of patients lack such a donor. We sought to develop a novel nonmyeloablative HLA-haploidentical PBSCT approach that could safely be used for patients with severe organ damage. Based on findings in our preclinical model, we developed a phase 1/2 trial using alemtuzumab, 400 cGy total body irradiation, and escalating doses of posttransplant cyclophosphamide (PT-Cy): 0 mg/kg in cohort 1, 50 mg/kg in cohort 2, and 100 mg/kg in cohort 3...
April 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29291386/nifuroxazide-a-stat3-inhibitor-mitigates-inflammatory-burden-and-protects-against-diabetes-induced-nephropathy-in-rats
#15
Eman Said, Sawsan A Zaitone, Mohamed Eldosoky, Nehal M Elsherbiny
Diabetic nephropathy (DN) is a serious complication of diabetes mellitus. Moreover,it is amongst the most common causes of end-stage renal failure. Inflammation is a crucial player in both development and progression of DN. JAK2/STA3 is a pleotropic cascade reported to regulate diverse inflammatory events. Previous studies reported involvement of JAK2/STA3 signal transduction pathway in diabetes-associated renal injury. In the current study, the inhibitory effect of nifuroxazide (25 mg/kg/day, orally) against inflammatory condition associating diabetic kidney progression in rats was evaluated...
December 29, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/29289551/role-of-tubulointerstitial-lesions-in-predicting-renal-outcome-among-pediatric-onset-lupus-nephritis-a-retrospective-cohort-study
#16
Chao-Yi Wu, Hui-Ping Chien, Huang-Yu Yang, Tsung-Chieh Yao, Min-Hua Tseng, Mei-Chin Yu, Kuo-Wei Yeh, Jing-Long Huang
BACKGROUND: Raising evidence suggested a prognostic utility of tubulointerstitial lesions in lupus nephritis (LN). The exact prevalence of tubulointerstitial abnormalities and its predictive value among pediatric onset systemic lupus erythematous (pSLE) cases, however, remained unknown. METHODS: Sixty-seven pSLE subjects diagnosed with LN with initial renal samples available were enrolled and followed for an average of 6.49 ± 3.06 years. Renal histology was evaluated according to the International Society of Nephrology/Renal Pathology Society classification, National Institute of Health classification and tubulointerstitial activity index (TIAI)...
December 15, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29288261/selection-and-short-term-outcomes-of-living-kidney-donors-in-singapore-an-analysis-of-the-donor-care-registry
#17
Marc Zj Ho, Huili Zheng, Jeannette Jm Lee, Khuan Yew Chow, Gek Hsiang Lim, Wei Wei Hong, Anantharaman Vathsala
INTRODUCTION: Transplant rates in Singapore have been falling and there is limited information on baseline characteristics and clinical outcomes of living kidney donors nationally. This study aimed to determine the safety of living kidney donor transplant in Singapore by exploring the proportion of donors that meets international selection guidelines and describing short-term clinical outcomes. MATERIALS AND METHODS: We analysed 472 donors who underwent nephrectomies from 1 January 2010 to 31 December 2014 from the Donor Care Registry...
November 2017: Annals of the Academy of Medicine, Singapore
https://www.readbyqxmd.com/read/29284159/characteristics-and-clinical-outcomes-of-living-renal-donors-in-hong-kong
#18
Y L Hong, C H Yee, C B Leung, J Yc Teoh, B Ch Kwan, P Kt Li, S Sm Hou, C F Ng
INTRODUCTION: In Asia, few reports are available on the outcomes for living renal donors. We report the short- and long-term clinical outcomes of individuals following living donor nephrectomy in Hong Kong. METHODS: We retrospectively reviewed the characteristics and clinical outcomes of all living renal donors who underwent surgery from January 1990 to December 2015 at our centre in Hong Kong. Information was obtained from hospital records and territory-wide electronic patient records...
December 29, 2017: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
https://www.readbyqxmd.com/read/29277509/mortality-and-allograft-loss-trends-among-us-pediatric-kidney-transplant-recipients-with-and-without-focal-segmental-glomerulosclerosis
#19
Chia-Shi Wang, Jennifer Gander, Rachel E Patzer, Larry A Greenbaum
BACKGROUND: Pediatric patients with focal segmental glomerulosclerosis (FSGS) have high rates of disease recurrence and allograft failure after kidney transplantation, but there are few data for long-term survival posttransplantation. STUDY DESIGN: Retrospective cohort study. SETTING & PARTICIPANTS: 12,303 pediatric patients (aged <18 years), including 1,408 (11%) patients with FSGS, who received a first kidney transplant in 1990 through 2009 and were followed up through June 2015 were identified from the US Renal Data System database...
December 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29277506/high-output-cardiac-failure-and-coronary-steal-with-an-arteriovenous-fistula
#20
Amtul Aala, Sairah Sharif, Leslie Parikh, Paul C Gordon, Susie L Hu
Creation of an arteriovenous access for hemodialysis can provoke a sequence of events that significantly affects cardiovascular hemodynamics. We present a 78-year-old man with end-stage renal disease and concomitant coronary artery disease previously requiring coronary artery bypass grafting including a left internal mammary graft to the left anterior descending artery, ischemic cardiomyopathy with left ventricular systolic dysfunction, and severe aortic stenosis who developed hypotension unresponsive to medical therapy after recent angioplasty of his ipsilateral arteriovenous fistula for high-grade outflow stenosis...
December 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
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