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https://www.readbyqxmd.com/read/28817400/liver-xenotransplantation
#1
Madhukar S Patel, Nathan Louras, Parsia A Vagefi
PURPOSE OF REVIEW: There continues to be an inadequate organ supply and lack of effective temporary support, for patients with liver failure. The purpose of this review is to discuss recent progress in the field of orthotopic pig-to-nonhuman primate (NHP) liver xenotransplantation (LXT). RECENT FINDINGS: From 1968 to 2012, survival in pig-to-NHP LXT was limited to 9 days, initially due to hyperacute rejection which has been ameliorated through use of genetically engineered donor organs, but ultimately because of profound thrombocytopenia, thrombotic microangiopathy, and bleeding...
August 16, 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28814708/-a-case-of-pulmonary-tumor-thrombotic-microangiopathy-in-castration-resistant-prostate-cancer
#2
Kodai Hattahara, Akihiro Hamada, Rie Oyama, Kimihiko Masui, Yasumasa Shichiri
A man aged 83 years under treatment with enzalutamide for castration-resistant prostate cancer presented with general malaise and exertional dyspnea. The underlying cause could not be identified by further investigations. On the 5th hospital day, he died due to a sudden exacerbation of dyspnea. The results of an autopsy indicated tumor emboli and stenosis of small pulmonary arteries with the fibrocellular intimal thickening, and therefore our final diagnosis was pulmonary tumor thrombotic microangiopathy.
July 2017: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/28814429/nintedanib-macrophage-activation-and-ameliorates-vascular-and-fibrotic-manifestations-in-the-fra2-mouse-model-of-systemic-sclerosis
#3
Jingang Huang, Christiane Maier, Yun Zhang, Alina Soare, Clara Dees, Christian Beyer, Ulrike Harre, Chih-Wei Chen, Oliver Distler, Georg Schett, Lutz Wollin, Jörg H W Distler
BACKGROUND: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc). METHODS: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed...
August 16, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28812187/thrombotic-microangiopathy-and-breastfeeding-where-is-the-link-answers
#4
Jean Daniel Delbet, Tim Ulinski
No abstract text is available yet for this article.
August 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28812099/thrombotic-microangiopathy-and-breastfeeding-where-is-the-link-questions
#5
Jean Daniel Delbet, Tim Ulinski
No abstract text is available yet for this article.
August 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28806758/developmental-vascular-remodeling-defects-and-postnatal-kidney-failure-in-mice-lacking-gpr116-adgrf5-and-eltd1-adgrl4
#6
Shun Lu, Shuya Liu, Astrid Wietelmann, Baktybek Kojonazarov, Ann Atzberger, Cong Tang, Ralph Theo Schermuly, Hermann-Josef Gröne, Stefan Offermanns
GPR116 (ADGRF5) and ELTD1 (ADGRL4) belong to different subfamilies of the adhesion G-protein-coupled receptor group but are both expressed in endothelial cells. We therefore analyzed their functions in mice lacking these receptors. While loss of GPR116 or ELTD1 alone had no obvious effect on cardiovascular or kidney function, mice lacking both, GPR116 and ELTD1, showed malformations of the aortic arch arteries and the cardiac outflow tract leading to perinatal lethality in about 50% of the mutants. In addition to cardiovascular malformations, surviving mice developed renal thrombotic microangiopathy as well as hemolysis and splenomegaly, and their lifespan was significantly reduced...
2017: PloS One
https://www.readbyqxmd.com/read/28802699/relationship-between-limited-joint-mobility-of-hand-and-carotid-atherosclerosis-in-patients-with-type-2-diabetes
#7
Yusuke Mineoka, Michiyo Ishii, Yoshitaka Hashimoto, Muhei Tanaka, Naoto Nakamura, Yasukazu Katsumi, Motohide Isono, Michiaki Fukui
AIM: Limited joint mobility (LJM) of hand, which is one of a complication of diabetic hand, has a close association with diabetic microangiopathy. However, it remains to be elucidated about the relationships between LJM of hand and subclinical atherosclerosis in patients with type 2 diabetes. Therefore, we conducted a cross-sectional study to evaluate the relationships between LJM of hand and carotid intima-media thickness (IMT) and plaque score in patients with type 2 diabetes. METHODS: We evaluated the relationships between LJM of hand, and carotid IMT and plaque score, evaluated by carotid ultrasound examination, in 341 consecutive patients with type 2 diabetes...
July 20, 2017: Diabetes Research and Clinical Practice
https://www.readbyqxmd.com/read/28802086/acute-kidney-injury-is-common-with-intravenous-abuse-of-extended-release-oral-oxymorphone-and-delayed-renal-recovery-rates-are-associated-with-increased-kdigo-staging
#8
Alex K Bonnecaze, Matthew Whitaker Wilson, Ajay Dharod, Alison Fletcher, P J Miller
BACKGROUND: Prescription opioid abuse poses a serious problem in the United States, representing 615 per 100,000 deaths annually. Extended-release oxymorphone (Opana-ER) is an oral opioid pain medication that has recently been found to cause thrombotic microangiopathy when intravenously abused. In this retrospective study, prevalence and outcomes of AKI among patients intravenously abusing extended-release oral oxymorphone were analyzed. METHODS: A query of electronic medical records for "drug abuse" at an academic medical center during January 2012 to December 2015 was performed and yielded 2350 patients...
August 12, 2017: Nephrology
https://www.readbyqxmd.com/read/28801959/outcomes-of-patients-with-atypical-hemolytic-uremic-syndrome-with-native-and-transplanted-kidneys-treated-with-eculizumab-a-pooled-post-hoc-analysis
#9
Christophe M Legendre, Josep M Campistol, Thorsten Feldkamp, Giuseppe Remuzzi, John F Kincaid, Åsa Lommelé, Jimmy Wang, Laurent E Weekers, Neil S Sheerin
Atypical hemolytic uremic syndrome (aHUS) often leads to end-stage renal disease (ESRD) and kidney transplantation; graft loss rates are high due to disease recurrence. A post hoc analysis of four prospective clinical trials in aHUS was performed to evaluate eculizumab, a terminal complement inhibitor, in patients with native or transplanted kidneys. The trials included 26-week treatment and extension periods. Dialysis, transplant, and graft loss were evaluated. Study endpoints included complete thrombotic microangiopathy (TMA) response, TMA event-free status, hematologic and renal parameters, and adverse events...
August 12, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28801815/plasma-levels-of-complement-activation-fragments-c3b-and-sc5b-9-significantly-increased-in-patients-with-thrombotic-microangiopathy-after-allogeneic-stem-cell-transplantation
#10
Jiaqian Qi, Jie Wang, Jia Chen, Jian Su, Yaqiong Tang, Xiaojin Wu, Xiao Ma, Feng Chen, Changgeng Ruan, X Long Zheng, Depei Wu, Yue Han
Transplantation-associated thrombotic microangiopathy (TA-TMA) is an uncommon but severe complication in patients undergoing allogeneic stem cell transplantation (allo-SCT). However, the mechanism is unclear. From 2011 to 2014, 20 patients with TA-TMA, 20 patients without, and 54 patients with various other complications, including veno occlusive disease (VOD), graft-versus-host disease (GVHD), and infection, were recruited in the study. Plasma vWF antigen (vWFAg), vWF activity (vWFAc), and ADAMTS13 activity were determined in these patients by ELISAs and FRETS-vWF73 assay, respectively...
August 11, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28798244/atypical-haemolytic-uremic-syndrome-ahus-and-membranoproliferative-glomerulonephritis-mpgn-different-diseases-or-a-spectrum-of-complement-mediated-glomerular-diseases
#11
Ghada A Ankawi, William F Clark
Historically, patients with kidney diseases caused by genetic or acquired dysregulation of the complement alternative pathway have been grouped into clinical syndromes, C3 glomerulopathy (C3GN/DDD) and thrombotic microangiopathy (TMA), specifically atypical haemolytic uremic syndrome (aHUS). Recent data suggested that these diseases share a common pathophysiology and that patients can transition between glomerulopathies in this spectrum. Histopathologically, the main difference cited is the immunofluorescence (IF) findings, with C3 predominance in C3 glomerulopathy (compared with immunoglobulins and complements in immune complex-mediated membranoproliferative glomerulonephritis (MPGN)) and negative IF in TMA...
August 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28796639/development-and-validation-of-a-multivariable-prediction-rule-for-detecting-a-severe-acquired-adamts13-activity-deficiency-in-patients-with-thrombotic-microangiopathies
#12
Jorge M Nieto, Félix De La Fuente-Gonzalo, Fernando A González, Ana Villegas, Rafael Martínez, Manuel E Fuentes, Paloma Ropero
BACKGROUND: Thrombotic microangiopathies (TMAs) are a group of diseases that have different aetiologies and treatments, but a clinical differential diagnosis remains difficult. Among TMAs, thrombotic thrombocytopenic purpura (TTP) is characterised by a severe ADAMTS13 functional deficiency. However, assays exploring ADAMTS13 activity are limited to some specialised laboratories. Our objective was to develop and validate a diagnostic method for TTP in adult patients with TMA. METHODS: We generated a multivariable model (four predictors) on a cohort of 174 TMA patients in order to predict an ADAMTS13 activity deficiency (AUC of 0...
August 10, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28795840/a-complete-histologic-approach-to-gastrointestinal-biopsy-from-hematopoietic-stem-cell-transplant-patients-with-evidence-of-transplant-associated-gastrointestinal-thrombotic-microangiopathy
#13
Mikako Warren, Sonata Jodele, Christopher Dandoy, Kasiani C Myers, Gregory Wallace, Adam Nelson, Javier El-Bietar
CONTEXT: - Transplant-associated thrombotic microangiopathy is a serious complication of hematopoietic stem cell transplant that may progress to multi-organ dysfunction. Transplant-associated thrombotic microangiopathy may involve the intestinal vasculature (intestinal transplant-associated thrombotic microangiopathy [iTMA]), causing patients to experience debilitating symptoms of ischemic colitis, including disproportionately severe abdominal pain and gastrointestinal bleeding, requiring heavy narcotic use and frequent transfusion support...
August 10, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28794515/the-complement-system-as-a-potential-therapeutic-target-in-rheumatic-disease
#14
REVIEW
Leendert A Trouw, Matthew C Pickering, Anna M Blom
Complement activation is associated with common rheumatic diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and systemic vasculitis. Evidence linking complement activation to these diseases includes the presence of complement deposition in affected tissues, decreased levels of complement proteins and high levels of complement activation fragments in the blood and/or synovial fluid of patients with these diseases, as well as data from experimental models. Eculizumab, a monoclonal antibody that inhibits the complement component C5, is now approved for the treatment of rare conditions involving complement hyperactivation, and the success of this therapy has renewed interest in understanding the utility of complement inhibition in rheumatological practice, particularly for SLE...
August 10, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28794069/phase-i-study-of-the-anti-cd22-immunotoxin-moxetumomab-pasudotox-for-childhood-acute-lymphoblastic-leukemia
#15
Alan S Wayne, Nirali N Shah, Deepa Bhojwani, Lewis B Silverman, James A Whitlock, Maryalice Stetler-Stevenson, Weili Sun, Meina Liang, Jie Yang, Robert J Kreitman, Mark C Lanasa, Ira Pastan
Novel therapies are needed to overcome chemotherapy resistance for children with relapsed/refractory acute lymphoblastic leukemia (ALL). Moxetumomab pasudotox is a recombinant anti-CD22 immunotoxin. A multicenter, phase I study was conducted to determine the maximum tolerated cumulative dose (MTCD) and evaluate safety, activity, pharmacokinetics, and immunogenicity of moxetumomab pasudotox in children, adolescents, and young adults with ALL (n=55). Moxetumomab pasudotox was administered as a 30-minute intravenous infusion at doses of 5 to 50 µg/kg every other day (QOD) for six (Cohorts A and B) or 10 (Cohort C) doses, on 21-day cycles...
August 9, 2017: Blood
https://www.readbyqxmd.com/read/28792176/chikungunya-fever-presenting-as-life-threatening-thrombotic-thrombocytopenic-purpura
#16
Vimal Kumar, Rujul Jain, Arvind Kumar, Neeraj Nischal, Pankaj Jorwal, Manish Soneja, Sudheer Arava, Naveet Wig
It is well known for Chikungunya fever to present as myriad of skin rash along with usual joint pain and fever, but probably this is the first case report of Chikungunya fever presenting as severe life threatening thrombotic microangiopathy, thrombotic thrombocytopenic purpura leading to multiple areas of skin necrosis, peripheral digital gangrene, haemolytic anemia, renal failure and severe thrombocytopenia with bleeding. This complication was most likely due to inhibitor autoantibody formation against ADAMTS13 triggered by chikungunya virus leading to thrombotic thrombocytopenic purpura...
July 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28791309/research-progress-on-mechanism-of-podocyte-depletion-in-diabetic-nephropathy
#17
REVIEW
Haoran Dai, Qingquan Liu, Baoli Liu
Diabetic nephropathy (DN) together with glomerular hyperfiltration has been implicated in the development of diabetic microangiopathy in the initial stage of diabetic diseases. Increased amounts of urinary protein in DN may be associated with functional and morphological alterations of podocyte, mainly including podocyte hypertrophy, epithelial-mesenchymal transdifferentiation (EMT), podocyte detachment, and podocyte apoptosis. Accumulating studies have revealed that disruption in multiple renal signaling pathways had been critical in the progression of these pathological damages, such as adenosine monophosphate-activated kinase signaling pathways (AMPK), wnt/β-catenin signaling pathways, endoplasmic reticulum stress-related signaling pathways, mammalian target of rapamycin (mTOR)/autophagy pathway, and Rho GTPases...
2017: Journal of Diabetes Research
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#18
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28790784/a-clinical-correlation-of-conjunctival-microangiopathy-with-grades-of-retinopathy-in-type-2-diabetes-mellitus
#19
M A Khan, Manasa Katta, V S Gurunadh, P S Moulick, Sandeep Gupta, Alok Sati
BACKGROUND: Conjunctival microangiopathy has been described among diabetics similar to retinal vessel angiopathy. Correlation of these conjunctival microangiopathy changes with retinopathy may form the basis of screening by external examination without expert fundus evaluation. METHODS: Conjunctival vessels widths and tortuous segment length of conjunctival vessels of 96 patients with type 2 diabetes mellitus were photographed and measured by the Zeiss Fundus camera Visupac software...
July 2017: Medical Journal, Armed Forces India
https://www.readbyqxmd.com/read/28782988/express-insights-on-pulmonary-tumor-thrombotic-microangiopathy-a-7-patient-case-series
#20
Rohit Godbole, Rajan Saggar, Alexander Zider, Jaime Betancourt, William Dean Wallace, Robert Daniel Suh, Nader Kamangar
No abstract text is available yet for this article.
January 1, 2017: Pulmonary Circulation
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