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Thrombotic microangiopathy

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https://www.readbyqxmd.com/read/28542627/predictive-features-of-chronic-kidney-disease-in-atypical-haemolytic-uremic-syndrome
#1
Matthieu Jamme, Quentin Raimbourg, Dominique Chauveau, Amélie Seguin, Claire Presne, Pierre Perez, Pierre Gobert, Alain Wynckel, François Provôt, Yahsou Delmas, Christiane Mousson, Aude Servais, Laurence Vrigneaud, Agnès Veyradier, Eric Rondeau, Paul Coppo
Chronic kidney disease (CKD) is a frequent and serious complication of atypical haemolytic uremic syndrome (aHUS). We aimed to develop a simple accurate model to predict the risk of renal dysfunction in aHUS based on clinical and biological features available at hospital admission. Renal function at 1-year follow-up, based on an estimated glomerular filtration rate < 60mL/min/1.73m2 as assessed by the Modification of Diet in Renal Disease equation, was used as an indicator of significant CKD. Prospectively collected data from a cohort of 156 aHUS patients who did not receive eculizumab were used to identify predictors of CKD...
2017: PloS One
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#2
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28522344/acquired-complement-regulatory-gene-mutations-and-hematopoietic-stem-cell-transplant-related-thrombotic-microangiopathy
#3
Gianluigi Ardissino, Stefania Salardi, Silvia Berra, Giacomo Colussi, Massimo Cugno, Marco Zecca, Fabio Giglio, Jacopo Peccatori, Elisa Diral, Francesca Tel, Alberto Clivio, Silvana Tedeschi
Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement dysregulation and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned...
May 15, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28521259/von-willebrand-factor-and-its-cleaving-protease-adamts13-balance-in-coronary-artery-vessels-lessons-learned-from-thrombotic-thrombocytopenic-purpura-a-narrative-review
#4
REVIEW
Nuccia Morici, Silvia Cantoni, Francesco Panzeri, Alice Sacco, Chiara Rusconi, Miriam Stucchi, Fabrizio Oliva, Marco Cattaneo
BACKGROUND: Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a microangiopathic syndrome that presents as an acute medical emergency. In this review we will explore the evidence of a two-way relationship between TTP and ACS. Moreover, we will review the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of ACS. METHODS AND RESULTS: Pubmed, MEDLINE and EMBASE, CINHAL, COCHRANE and Google Scholar databases were searched from inception to January 2017...
May 12, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28512098/a-rare-case-of-thrombotic-microangiopathy-triggered-by-acute-pancreatitis
#5
Kevin Singh, Ahmed Jamal Nadeem, Behzad Doratotaj
Thrombotic microangiopathy (TMA) occurring after acute pancreatitis is rarely described. Without prompt intervention, TMA can be, and often is, lethal, so prompt recognition is important. Here, we present a case of a 61-year-old woman with a history of alcohol misuse who presented with epigastric pain, nausea and vomiting after binge drinking. Elevated serum lipase and imaging were suggestive of acute-on-chronic pancreatitis. Although the patient's symptoms of acute pancreatitis subsided, her anaemia, thrombocytopenia and acute kidney injury worsened...
May 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28509125/a-case-of-pr3-anca-positive-anti-gbm-disease-associated-with-intrarenal-arteritis-and-thrombotic-microangiopathy
#6
Shun Manabe, Mayuko Banno, Marie Nakano, Teruhiro Fujii, Yukio Kakuta, Kosaku Nitta, Michiyasu Hatano
Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) is occasionally reported and termed "double positive" disease. Interestingly, the majority of "double positive" ANCA is myeloperoxidase (MPO)-ANCA, and some of the MPO-ANCA-positive cases reveal intrarenal arteritis indicating an ANCA-associated renal lesion. In contrast, proteinase 3 (PR3)-ANCA-positive "double positive" disease had rarely been reported, and as far as we know, none of the cases showed intrarenal arteritis...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509120/complex-glomerular-pathology-of-thrombotic-microangiopathy-and-focal-segmental-glomerulosclerosis-forms-tumor-like-mass-in-a-renal-transplant-donor-with-severe-renovascular-hypertension
#7
Michio Nagata, Yutaka Yamaguchi, Daisuke Toki, Izumi Yamamoto, Hiroaki Shinmura, Hiroshi Kawaguchi
The pathogenesis of glomerular hypertension-mediated FSGS and its histological variations in humans remains unknown. A 47-year-old man developed nephrotic syndrome, renal dysfunction, and malignant hypertension 2 years after donating a kidney to his son. The donor's remnant kidney developed renal mass at an upper pole which was fed by an aberrant artery that branched from the root of the renal artery. Furthermore, the main non-aberrant renal artery demonstrated severe stenosis that caused renovascular hypertension, resulting in malignant hypertension...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#8
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508588/at-the-cross-section-of-thrombotic-microangiopathy-and-atypical-hemolytic-uremic-syndrome-a-narrative-review-of-differential-diagnostics-and-a-problematization-of-nomenclature
#9
REVIEW
Alexander Åkesson, Eva Zetterberg, Jenny Klintman
Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with high mortality and morbidity. Renal biopsies often indicate thrombotic microangiopathy (TMA). The condition is caused by an excessive activation of the alternative pathway leading to depositions of membrane attack complexes (MAC) on host cells. It may depend on mutations in complement components and regulatory proteins, or the formation of complement-specific antibodies. Mainly, an environmental trigger (e.g. infection) is needed for the excessive response to develop...
May 16, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28501299/severe-hypertension-with-renal-thrombotic-microangiopathy-what-happened-to-the-usual-suspect
#10
Steven Van Laecke, Wim Van Biesen
Patients with atypical hemolytic uremic syndrome (aHUS) and malignant hypertension can both present with concomitant hypertension and thrombotic microangiopathy (TMA), rendering policy decisions complex. Timmermans et al. report that patients with severe hypertension and renal TMA might have unrecognized aHUS with underlying complement abnormalities. Based on this, they assert that all patients presenting with severe hypertension and renal TMA should be evaluated for aHUS. It remains uncertain whether this holds equally true for patients with malignant hypertension and renal TMA...
June 2017: Kidney International
https://www.readbyqxmd.com/read/28499586/-gemcitabine-induced-thrombotic-microangiopathy-can-we%C3%A2-improve-screening-and%C3%A2-treatment
#11
Xavier Charmetant, Anne Jolivot, Thomas Fournier, Jean-Charles Puthet, Philippe Cassier, Sandrine Lemoine, Laurent Juillard
Thrombotic microangiopathy is a rare but severe complication of treatment with gemcitabine. Its prevalence increases because gemcitabine's indications are growing. We report four cases, which presented with common clinical and biological manifestations, i.e. high blood pressure, proteinuria and increasing plasmatic creatinine level. However, severity was not similar, hemodialysis was inconstant. There is no consensus on treatment for this condition. Stopping gemcitabine is essential. Treatment was dispensed considering the severity of the presentation: plasma exchange therapy of variable outcome, and eculizumab, which was efficient when used...
May 9, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28499011/fatal-case-of-chikungunya-and-concomitant-thrombotic-thrombocytopenic-purpura-in-french-guiana-during-air-flight-medical-evacuation
#12
Loïc Epelboin, Bastien Bidaud, Emilie Mosnier, Paul Le Turnier, Guillaume Vesin, Gaëlle Walter, Philippe Abboud, Stéphanie Houcke, Maïa Forgues, Gérald Egmann, Alain Stepanian, Félix Djossou
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated to severe ADAMTS13 deficiency. It has been linked to various viral infections. Among arboviruses, only Crimean-Congo haemorrhagic fever and dengue fever have been linked to this severe disease. We report the first documented case of TTP concomitant to Chikungunya virus infection.
September 1, 2017: Journal of Travel Medicine
https://www.readbyqxmd.com/read/28485097/hematopoietic-stem-cell-transplantation-and-acute-kidney-injury-in-children-a-comprehensive-review
#13
REVIEW
Rupesh Raina, Nicholas Herrera, Vinod Krishnappa, Sidharth Kumar Sethi, Akash Deep, Wei-Ming Kao, Timothy Bunchman, Rolla Abu-Arja
AKI in the setting of HSCT is commonly investigated among adult patients. In the same way, malignancies requiring treatment with HSCT are not limited to the adult patient population, AKI following HSCT is frequently encountered within pediatric patient populations. However, inadequate information regarding epidemiology and pathophysiology specific to pediatric patients prevents development of appropriate and successful therapeutic strategies for those afflicted. Addressing AKI in the context of sinusoidal obstruction syndrome, chemotherapy, thrombotic microangiopathy and hypertension post chemotherapy, glomerulonephritis, and graft versus host disease provides greater insight into renal impairment associated with these HSCT-related ailments...
June 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28485072/therapeutic-plasma-exchange-in-children-one-center-s-experience
#14
Gerard Cortina, Violeta Ojinaga, Thomas Giner, Magdalena Riedl, Siegfried Waldegger, Alejandra Rosales, Raphaela Trojer, Johannes Hofer
BACKGROUND: Therapeutic plasma exchange (TPE) has evolved to an accepted therapy for selected indications. However, it is technically challenging in children. Moreover, data on safety and efficacy are mainly derived from adult series. The aim of this study was to review the procedure in the context of clinical indications, effectiveness, and safety. STUDY DESIGN AND METHODS: All TPE procedures performed at a tertiary care hospital during a 12-year period (2005-2016) were retrospectively evaluated...
May 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28471571/thrombotic-microangiopathy-a-challenging-diagnosis-always
#15
Javier Marco-Hernández, Sergio Prieto-González, Miquel Blasco, Pedro Castro, Joan Cid, Gerard Espinosa
No abstract text is available yet for this article.
July 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28462511/altered-expression-of-enos-prostacyclin-synthase-prostaglandin-g-h-synthase-and-thromboxane-synthase-in-porcine-aortic-endothelial-cells-after-exposure-to-human-serum-relevance-to-xenotransplantation
#16
Pengfei Chen, Hanchao Gao, Ying Lu, Huirong Nie, Zhaoming Liu, Yu Zhao, Nana Fan, Qingjian Zou, Yifan Dai, Aifa Tang, Hidetaka Hara, Zhiming Cai, David K C Cooper, Liangxue Lai, Lisha Mou
Under normal conditions, the activity of platelets is stringently and precisely balanced between activation and quiescent state. This guarantees rapid hemostasis and avoids uncontrolled thrombosis. However, excessive platelet activation and resulting thrombotic microangiopathy are frequently observed in pig-to-primate xenotransplantation models. Endothelium-derived inhibitory mechanisms play an important role in regulation of platelet activation. These mainly include nitric oxide (NO), prostacyclin PGI2 , and adenosine, which are synthesized by endothelial NO synthases (eNOS), prostacyclin synthase, and CD39/CD73, respectively...
April 29, 2017: Cell Biology International
https://www.readbyqxmd.com/read/28460546/an-update-on-chemical-pharmacotherapy-options-for-the-prevention-of-kidney-transplant-rejection-with-a-focus-on-costimulation-blockade
#17
Florian Kälble, Matthias Schaier, Sebastian Schäfer, Caner Süsal, Martin Zeier, Claudia Sommerer, Christian Morath
The introduction of calcineurin inhibitors (CNI) has greatly improved graft survival in the past three decades. However, long-term graft survival is still limited due to chronic allograft injury and side-effects of immunosuppressive medication. Areas covered: The present overview gives an update on pharmacotherapeutic strategies after kidney transplantation. The main focus is on CNI-sparing regimens using co-stimulatory blockade and on new substances on the horizone. Expert opinion: CNI sparing regimens are well-established...
June 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/28458380/pulmonary-tumor-thrombotic-microangiopathy%C3%A3-antemortem-diagnosis-with-pulmonary-artery-wedge-blood-cell-sampling-in-a-recurrent-breast-cancer-patient
#18
Shunichiro Fuchigami, Ryusuke Tsunoda, Hiroshi Shimizu, Masafumi Takae, Hiroki Usuku, Hiromi Yoshimura, Tomokazu Ikemoto, Michiko Nagamine, Teruhiko Ito, Yasuhiro Izumiya, Koichi Kaikita, Seiji Hokimoto, Kenichi Tsujita
No abstract text is available yet for this article.
April 29, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28458317/long-term-eculizumab-treatment-contributes-to-recovery-from-end-stage-renal-disease-caused-by-atypical-hemolytic-uremic-syndrome
#19
Yuji Yamada, Ryohei Abe, Yutaka Okano, Yoshitaka Miyakawa
We experienced a favorable outcome in an adult case of atypical hemolytic uremic syndrome (aHUS) after long-term eculizumab treatment. A 38-year-old Japanese man with a history of central retinal vein occlusion was admitted to our hospital with progressive dyspnea. He was found to have non-immune hemolytic anemia, thrombocytopenia, and acute renal failure two weeks after an episode of the common cold. Plasma exchange was ineffective; therefore, we initiated eculizumab after we excluded other thrombotic microangiopathies...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28454234/gemcitabine-associated-thrombotic-microangiopathy-in-a-patient-with-lung-cancer-a-case-report
#20
Florence Lai-Tiong, Yann Duval, Francois Krabansky
Gemcitabine is frequently used for the treatment of a number of different cancer types. Gemcitabine-related thrombotic microangiopathy (TMA) has rarely been described, but it is a life-threatening complication. The incidence of the complication varies between 0.015 and 1.4%. The present study reports the case of a 63-year-old Caucasian male who was treated with 3 cycles of carboplatin plus gemcitabine, followed by 7 cycles of gemcitabine only, and developed clinical symptoms that, together with laboratory findings, were compatible with a diagnosis of hemolytic uremic syndrome TMA...
March 2017: Oncology Letters
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