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Thrombotic microangiopathy

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https://www.readbyqxmd.com/read/28720207/thrombotic-microangiopathy-a-multidisciplinary-team%C3%A2-approach
#1
Craig E Gordon, Vipul C Chitalia, J Mark Sloan, David J Salant, David L Coleman, Karen Quillen, Katya Ravid, Jean M Francis
Thrombotic microangiopathy (TMA) is characterized by the presence of microangiopathic hemolytic anemia and thrombocytopenia along with organ dysfunction, and pathologically, by the presence of microthrombi in multiple microvascular beds. Delays in diagnosis and initiation of therapy are common due to the low incidence, variable presentation, and poor awareness of these diseases, underscoring the need for interdisciplinary approaches to clinical care for TMA. We describe a new approach to improve clinical management via a TMA team that originally stemmed from an Affinity Research Collaborative team focused on thrombosis and hemostasis...
July 15, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28720077/hemolytic-uremic-syndrome-as-the-presenting-manifestation-of-wt1-mutation-and-denys-drash-syndrome-a-case-report
#2
Joseph L Alge, Scott E Wenderfer, John Hicks, Mir Reza Bekheirnia, Deborah A Schady, Jamey S Kain, Michael C Braun
BACKGROUND: Hemolytic uremic syndrome (HUS) can occur as a primary process due to mutations in complement genes or secondary to another underlying disease. HUS sometimes occurs in the setting of glomerular diseases, and it has been described in association with Denys-Drash syndrome (DDS), which is characterized by the triad of abnormal genitourinary development; a pathognomonic glomerulopathy, diffuse mesangial sclerosis; and the development of Wilms tumor. CASE PRESENTATION: We report the case of a 46, XX female infant who presented with HUS and biopsy-proven thrombotic microangiopathy...
July 18, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28718268/oxymorphone-induced-thrombotic-microangiopathy-mimicking-atypical-haemolytic-uremic-syndrome
#3
Amibhen Gandhi, Saad Ullah, Shani Kotadia, Samer Nasser
Atypical Haemolytic Uremic Syndrome (aHUS) is a rare life threatening entity characterized by thrombocytopenia, haemolytic anaemia and renal dysfunction. It is a thrombotic microangiopathy related to genetic mutations in the alternate complement pathway and has a distinct pathophysiology which makes it harder to distinguish from other microangiopathies. We present a case of a 25-year-old male patient with history of polysubstance abuse who presented with chest pain and dyspnoea. He admitted to using injectable oxymorphone (Opana) two weeks before presentation...
April 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28716778/thrombotic-microangiopathy-associated-with-intravenous-injection-of-extended-release-oxycodone
#4
Kate J Robson, Danielle Clucas, Robin Filshie, Harshal Nandurkar
We describe the case of a 35-year-old man presenting with thrombotic microangiopathy (TMA) and renal impairment following, as he later disclosed, intravenous injection of oral formulation tamper-resistant extended-release oxycodone hydrochloride (Oxycontin). Recurrent misuse of this agent was associated with relapsing TMA despite treatment with terminal complement inhibitor eculizumab. Cases of TMA have been reported in the USA in association with intravenous misuse of extended-release oxymorphone (Opana ER) after the introduction of a new non-crushable formulation in 2012...
July 17, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28716106/acute-interstitial-nephritis-after-sequential-ipilumumab-nivolumab-therapy-of-metastatic-melanoma
#5
Lea Bottlaender, Anne-Laure Breton, Louis de Laforcade, Frederique Dijoud, Luc Thomas, Stephane Dalle
BACKGROUND: The anti-Programmed Death receptor 1 (anti-PD-1) antibodies nivolumab and pembrolizumab are new treatments in metastatic melanoma. Immunotherapies are best known to be responsible for thrombotic microangiopathy. However, immune interstitial nephritis has been described in a patient treated by nivolumab and ipilimumab concomitantly, and three cases of granulomatous interstitial nephritis have been reported with ipilimumab monotherapy. We report herein a case of acute interstitial immune nephritis in a patient treated with nivolumab after ipilimumab for pulmonary metastatic melanoma...
July 18, 2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28711333/b12-deficiency-leading-to-marked-poikilocytosis-versus-true-schistocytosis-a-pernicious-problem
#6
James A Hall, James Mason, Julia Choi, Mark Holguin
Severe vitamin B12 deficiency is caused most commonly by autoimmune atrophic gastritis leading to loss of intrinsic factor. Vitamin B12 deficiency leading to megaloblastic anemia and demyelinating central nervous system disease is well known; however, a rare presentation of B12 deficiency described as pseudothrombotic microangiopathy is not well known. This complication presents with signs of mechanical hemolysis, elevated lactate dehydrogenase (LDH), thrombocytopenia, and a low reticulocyte count, which can be incorrectly diagnosed as thrombotic thrombocytopenic purpura and managed incorrectly...
June 20, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28711159/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-epidemiology-pathophysiology-and-clinics
#7
C Rafat, P Coppo, F Fakhouri, V Frémeaux-Bacchi, C Loirat, J Zuber, E Rondeau
Thrombotic microangiopathies (TMA) represent an eclectic group of conditions, which share hemolytic anemia and thrombocytopenia as a common defining basis. Remarkable breakthroughs in the physiopathological setting have allowed for a thorough recomposition of the disparate syndromes, which form the constellation of TMA. In this view, clinicians now discriminate thrombocytopenic thrombotic purpura (TTP) defined by a severe deficiency in ADAMTS13, which is rarely associated with a severe renal involvement and the hemolytic and uremic syndrome (HUS) in which renal impairment is the most prominent clinical feature...
July 12, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28711077/acute-kidney-injury-in-pregnancy
#8
Belinda Jim, Vesna D Garovic
Pregnancy-related acute kidney injury (AKI) has declined in incidence in the last three decades, although it remains an important cause of maternal and fetal morbidity and mortality. Pregnancy-related causes of AKI such as preeclampsia, acute fatty liver of pregnancy, HELLP (Hemolysis, Elevated Liver function tests, Low Platelets) syndrome, and the thrombotic microangiopathies (thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome [HUS]) exhibit overlapping features and often present as diagnostic dilemmas...
July 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28710195/marantic-endocarditis-and-paraneoplastic-pulmonary-embolism
#9
Tiago Lobo Ferreira, Rosa Alves, Tiago Judas, Maria F Delerue
Cancer is frequently associated with a hypercoagulable state. Almost 15% of patients with cancer will suffer a thromboembolic event during their clinical course. The aetiology of this hypercoagulable state is multifactorial and includes procoagulant factors associated with malignancy as well as the host's inflammatory response. Cancer-associated thrombophilia can present as venous thromboembolism, migratory superficial thrombophlebitis, arterial thrombosis, disseminated intravascular coagulation, thrombotic microangiopathy and rarely non-bacterial thrombotic endocarditis (NBTE)...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28705839/circulating-dsdna-endothelial-injury-and-complement-activation-in-thrombotic-microangiopathy-and-gvhd
#10
Nicholas J Gloude, Pooja Khandelwal, Nathan Luebbering, Dana T Lounder, Sonata Jodele, Matthew N Alder, Adam Lane, Alyss Wilkey, Kelly E Lake, Bridget Litts, Stella M Davies
TA-TMA is a common and poorly recognized complication of HSCT associated with excessive complement activation, likely triggered by endothelial injury. An important missing piece is the link between endothelial injury and complement activation. We hypothesized that neutrophil extracellular traps (NETs) mechanistically link endothelial damage with complement activation and subsequent TA-TMA. Neutrophil activation releases granule proteins together with double stranded DNA (dsDNA), to form extracellular fibers known as NETs...
July 13, 2017: Blood
https://www.readbyqxmd.com/read/28691557/emicizumab-prophylaxis-in-hemophilia-a-with-inhibitors
#11
Johannes Oldenburg, Johnny N Mahlangu, Benjamin Kim, Christophe Schmitt, Michael U Callaghan, Guy Young, Elena Santagostino, Rebecca Kruse-Jarres, Claude Negrier, Craig Kessler, Nancy Valente, Elina Asikanius, Gallia G Levy, Jerzy Windyga, Midori Shima
Background Emicizumab (ACE910) bridges activated factor IX and factor X to restore the function of activated factor VIII, which is deficient in persons with hemophilia A. This phase 3, multicenter trial assessed once-weekly subcutaneous emicizumab prophylaxis in persons with hemophilia A with factor VIII inhibitors. Methods We enrolled participants who were 12 years of age or older. Those who had previously received episodic treatment with bypassing agents were randomly assigned in a 2:1 ratio to emicizumab prophylaxis (group A) or no prophylaxis (group B)...
July 10, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28688917/real-world-economic-burden-associated-with-transplant-related-complications
#12
M A Perales, M Bonafede, Q Cai, P M Garfin, D McMorrow, N C Josephson, A Richhariya
Approximately 20,000 hematopoietic cell transplantation (HCT) procedures are performed in the US annually. Real-world evidence on the costs associated with post-transplant complications are limited. Patients with hematologic malignancies aged ≥18 years undergoing auto-HCT or allo-HCT between January 1, 2011 and June 30, 2014 were identified in the Truven Health MarketScan(®) Research Databases. Patients were required to have 12 months of continuous medical and pharmacy enrollment pre- and post-HCT with the exception that patients with evidence of inpatient death within 12 months post-HCT were also included...
July 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28683654/efficacy-and-safety-of-multitarget-therapy-with-cyclophosphamide-and-tacrolimus-for-lupus-nephritis-a-prospective-single-arm-single-centre-open-label-pilot-study-in-japan
#13
R Sakai, T Kurasawa, E Nishi, T Kondo, Y Okada, A Shibata, K Nishimura, K Chino, A Okuyama, H Takei, H Nagasawa, K Amano
Background Pulsed cyclophosphamide or mycophenolate mofetil for lupus nephritis has limited efficacy. We previously reported a case of mixed-class IV + V lupus nephritis successfully treated with cyclophosphamide and tacrolimus. This study assessed the efficacy and safety of multitarget therapy with cyclophosphamide and tacrolimus for the treatment of lupus nephritis. Methods In a prospective, single-arm, open label pilot study, we recruited 15 patients aged 18-64 years with active lupus nephritis who met the American College of Rheumatology criteria for a diagnosis of systemic lupus erythematosus (1997)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28682944/a-simplified-mortality-score-using-delta-neutrophil-index-and-the-thrombotic-microangiopathy-score-for-prognostication-in-critically-ill-patients
#14
Eun Kyong Goag, Jong Wook Lee, Yun Ho Roh, Ah Young Leem, Song Yee Kim, Joo Han Song, Eun Young Kim, Ji Ye Jung, Moo Suk Park, Young Sam Kim, Se Kyu Kim, Joon Chang, Kyung Soo Chung
BACKGROUND: This study was conducted to assess the prognostic value of a simplified mortality score (SMS) using the delta neutrophil index (DNI) and thrombotic microangiopathy (TMA) score, both easily obtained from the complete blood count, to identify critically ill patients at high risk of death. METHODS: This was a retrospective study performed in the medical ICU at Yonsei University College of Medicine from June 2015 to February 2016. The primary end-point was 28-day all-cause mortality...
July 4, 2017: Shock
https://www.readbyqxmd.com/read/28681548/how-do-we-reduce-plasma-transfusion-in-rhode-island
#15
Christian P Nixon, Maria F Tavares, Joseph D Sweeney
BACKGROUND: Plasma transfusions are given to patients with coagulopathy, either prophylactically, before an invasive procedure; or therapeutically, in the presence of active bleeding; and as an exchange fluid in therapeutic plasma exchange for disorders such as thrombotic thrombocytopenic purpura. There is consensus that many prophylactic plasma transfusions are non-efficacious, and the misdiagnosis of thrombotic thrombocytopenic purpura results in unnecessary therapeutic plasma exchange...
July 5, 2017: Transfusion
https://www.readbyqxmd.com/read/28679995/thrombotic-microangiopathy-due-to-malignant-hypertension-complicated-with-late-onset-bleeding-after-renal-biopsy
#16
Saki Ameda, Hiroyuki Kuroda, Michiko Yamada, Ken Sato, Shogo Miura, Hiroya Sakano, Takanori Shibata, Naoki Uemura, Tomoyuki Abe, Shigeyuki Fujii, Masahiro Maeda, Miri Fujita, Masayoshi Kobune, Junji Kato
A 47-year-old man presented at a local ophthalmological hospital with blurred vision. He had been diagnosed with hypertensive retinopathy and renal failure and was referred to our hospital for treatment. A renal biopsy was done to evaluate pathology of high proteinuria, hematuria, and rapidly progressive glomerulonephritis. Blood pressure remained high despite antihypertensive therapy; anemia and thrombocytopenia gradually progressed. Thrombotic microangiopathy (TMA) was suspected based on red blood cell fragmentation due to hemolytic anemia, thrombocytopenia, and renal failure...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28670985/-changes-in-diagnostic-criteria-of-thrombotic-microangiopathy-after-stem-cell-transplantation
#17
Orsolya Horváth, Zoltán Prohászka, Krisztián Kállay, Csaba Kassa, Anita Stréhn, Katalin Csordás, János Sinkó, Gergely Kriván
Hematopoietic stem cell transplantation associated thrombotic microangiopathy is a multifactorial complication, and has variable incidence in study populations due to different diagnostic criteria. The diversity of activity parameters, like elevated laktát-dehidrogenáz, hematological parameters and kidney function are not specific variables after stem cell transplantation. Dysregulation of the classical and alternative pathway can play an important role in the pathomechanism of thrombotic microangiopathy, but the understanding of the role of complement activation under transplantation conditions requires further investigation...
July 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28662310/thrombotic-thrombocytopenic-purpura-pathogenesis-diagnosis-and-potential-novel-therapeutics
#18
REVIEW
Manish Saha, Jenny K McDaniel, X Long Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is primarily caused by autoantibodies against the von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS13. In general, severe deficiency of plasma ADAMTS13 activity (<10 IU/dL) with or without detectable inhibitory autoantibodies against ADAMTS13 supports the diagnosis of TTP if a patient presents with thrombocytopenia and microangiopathic hemolytic anemia (i.e. schistocytes, elevated serum lactate dehydrogenase, decreased hemoglobin and haptoglobin) without other known etiologies that cause thrombotic microangiopathy (TMA)...
June 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28659364/acute-cor-pulmonale-due-to-pulmonary-tumour-thrombotic-microangiopathy-from-renal-cell-carcinoma
#19
Maria Story, Sook Kyung Kwon, Robert Robinson, Spyridon Fortis
We report the case of a previously healthy man who presented with subacute dyspnoea after a long drive. He developed hypoxic respiratory failure, thought secondary to a massive pulmonary embolism and was treated with tissue plasminogen activator but died in the hospital despite aggressive medical measures. Autopsy revealed pulmonary tumour thrombotic microangiopathy (PTTM) from papillary renal cell carcinoma. PTTM is a rare clinicopathological syndrome that clinically results in symptoms of dyspnoea and right heart failure...
June 28, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28651806/von-willebrand-factor-deposition-and-adamts-13-consumption-in-allograft-tissue-of-thrombotic-microangiopathy-like-disorder-after-living-donor-liver-transplantation-a-case-report
#20
S Nakanuma, T Miyashita, H Hayashi, Y Ohbatake, H Takamura, M Okazaki, T Yamaguchi, S Sakai, I Makino, K Oyama, H Tajima, I Ninomiya, S Fushida, T Ohta
BACKGROUND: Thrombotic microangiopathy (TMA) pathogenesis after living donor liver transplantation (LDLT) is thought to be caused by release of unusually large von Willebrand factor multimers (UL-vWFMs) resulting from sinusoidal endothelial cell damage and induction of platelet adhesion and aggregation. A decrease in a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs-13 (ADAMTS-13) that cleave UL-vWFMs might cause excessive UL-vWFMs activity and result in platelet thrombus formation...
June 23, 2017: Transplantation Proceedings
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