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Thrombotic microangiopathy

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https://www.readbyqxmd.com/read/29356300/pathophysiology-of-thrombotic-thrombocytopenic-purpura-and-hemolytic-uremic-syndrome
#1
Johanna A Kremer Hovinga, Silvan R Heeb, Magdalena Skowronska, Monica Schaller
Thrombotic microangiopathies are rare disorders characterized by the concomitant occurrence of severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic end organ damage. The latter particularly affects the brain, the heart and the kidneys. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although in their clinical presentation often overlapping, have distinctive pathophysiologies. TTP is the consequence of a severe ADAMTS13 deficiency, immune-mediated due to circulating autoantibodies (iTTP), or caused by mutations in the ADAMTS13 gene (cTTP)...
January 22, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29353400/a-stard-compliant-prediction-model-for-diagnosing-thrombotic-microangiopathies
#2
Pietro Manuel Ferraro, Gianmarco Lombardi, Alessandro Naticchia, Antonio Sturniolo, Cecilia Zuppi, Valerio De Stefano, Patrizia Bonelli, Ruggero Buonocore, Gianfranco Cervellin, Giuseppe Lippi, Giovanni Gambaro
Aim of the study was the definition of a predictive model for the initial diagnosis of thrombotic microangiopathies (TMA). We retrospectively collected data on all adult patients admitted to the Gemelli Hospital from 2010 to 2014. ICD-9 codes from primary diagnoses were used for TMA diagnosis. Demographic and laboratory characteristics on admission of patients with TMA were then compared with a random sample of 500 patients with other diagnoses. The prediction model was externally validated in a cohort from another hospital...
January 20, 2018: Journal of Nephrology
https://www.readbyqxmd.com/read/29352455/clinical-features-in-a-series-of-258-japanese-pediatric-patients-with-thrombotic-microangiopathy
#3
Akira Ashida, Hideki Matsumura, Toshihiro Sawai, Rika Fujimaru, Yuko Fujii, Akihiko Shirasu, Hyogo Nakakura, Kazumoto Iijima
BACKGROUND: Thrombotic microangiopathy (TMA) includes hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). This study examined the epidemiological characteristics of pediatric patients with TMA classified according to etiology. METHODS: The survey evaluated 258 Japanese pediatric patients diagnosed with TMA between 2012 and 2015. RESULTS: The primary diseases responsible for TMA were categorized as TTP (15 cases), Shiga toxin-producing Escherichia coli-associated HUS (STEC-HUS) (166 cases), atypical HUS (aHUS) (40 cases), and secondary TMA (27 cases)...
January 19, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29350576/posterior-reversible-encephalopathy-syndrome-in-stroke-prone-spontaneously-hypertensive-rats-on-high-salt-diet
#4
Fanny Herisson, Iris Zhou, Jerome Mawet, E Du, Arnavaz H Barfejani, Tao Qin, Marilyn J Cipolla, Philip Z Sun, Natalia S Rost, Cenk Ayata
Stroke-prone spontaneously hypertensive rats (SHRSP) on high-salt diet are characterized by extremely high arterial pressures, and have been endorsed as a model for hypertensive small vessel disease and vascular cognitive impairment. However, rapidly developing malignant hypertension is a well-known cause of posterior reversible encephalopathy syndrome (PRES) in humans, associated with acute neurological deficits, seizures, vasogenic cerebral edema and microhemorrhages. In this study, we aimed to examine the overlap between human PRES and SHRSP on high-salt diet...
January 1, 2018: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/29348275/microangiopathies-in-pregnancy
#5
Jailan Elayoubi, Kavitha Donthireddy, Divyesh Reddy Nemakayala
Thrombotic thrombocytopenic purpura (TTP) is a potentially reversible, life-threatening medical emergency. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis. Negative Coomb's test, thrombocytopenia and placental infarcts raised suspicion for a thrombotic microangiopathy. She was diagnosed with TTP and managed with emergency plasma exchange. Her recovery was immediate.A presumptive diagnosis of TTP should be based on the presence of microangiopathic haemolytic anaemia with thrombocytopenia and plasma exchange should be initiated while complete work up is pending...
January 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29339271/early-increase-in-complement-terminal-pathway-activation-marker-sc5b-9-is-predictive-for-the-development-of-thrombotic-microangiopathy-after-stem-cell-transplantation
#6
Orsolya Horváth, Krisztián Kállay, Dorottya Csuka, Blanka Mező, György Sinkovits, Csaba Kassa, Anita Stréhn, Katalin Csordás, János Sinkó, Zoltán Prohászka, Gergely Kriván
Hematopoietic stem cell transplantation- (HSCT-) associated thrombotic microangiopathy (TA-TMA) is a multifactorial complication, and its prediction is largely unresolved.Our aim was to analyze changes of complement profile after HSCT in order to identify potential markers of TA-TMA development. Thirty- three consecutive pediatric patients (9.6 ± 4.4 years old) were included in this study who underwent allogeneic HSCT due to malignant (n=17) or non malignant (n=16) indications. Graft versus host disease (GVHD) was diagnosed using Glucksberg criteria, viral reactivation was monitored, five different TA-TMA diagnostic criteria were applied and all important clinical and laboratory parameters of TA-TMA activity were registered...
January 12, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#7
REVIEW
Marcel Levi, Marie Scully, Mervyn Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor is most prominent. The multimeric size of von Willebrand factor is an important determinant of a more intense platelet-vessel wall interaction. and is regulated by the von Willebrand factor cleaving protease, ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultra-large von Willebrand factor multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura (TTP), a thrombotic microangiopathy characterized by thrombocytopenia, non-immune haemolysis, and organ dysfunction...
January 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29334938/therapeutic-plasma-exchange-in-a-tertiary-care-center-185-patients-undergoing-912-treatments-a-one-year-retrospective-analysis
#8
Julius J Schmidt, Firas Asper, Gunilla Einecke, Gabriele Eden, Carsten Hafer, Jan T Kielstein
BACKGROUND: Therapeutic plasma exchange (TPE) is increasingly used throughout the world. Although the procedure itself is fairly standardized, it is yet unknown how the underlying disease entities influence the key coordinates of the treatment. METHODS: Retrospective chart review. The treatment indications were clustered into four categories. Data are presented as median and interquartile (25-75%) range [IQR]. RESULTS: Within 1 year, 912 TPE treatments were performed in 185 patients (90 female, 48...
January 15, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29332932/thrombotic-microangiopathy-pregnancy-outcomes-in-ahus
#9
Ellen F Carney
No abstract text is available yet for this article.
January 15, 2018: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/29331477/atypical-presentation-of-pregnancy-related-hemolytic-uremic-syndrome
#10
Salim Baghli, Catherine Abendroth, Umar Farooq, Jennifer A Schaub
The cause of acute kidney injury during pregnancy and in the postpartum period can be particularly challenging to diagnose, especially when it is necessary to differentiate among preeclampsia; eclampsia; hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome; and thrombotic microangiopathies (TMAs). All these disease entities can present with kidney failure, microangiopathic hemolytic anemia, and thrombocytopenia. We present a teaching case of atypical hemolytic uremic syndrome in the postpartum period in a young woman who was found to have mutations of uncertain clinical significance in the complement cascade, including in C3, CFH, and CFI...
January 10, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29329518/the-use-of-eculizumab-in-gemcitabine-induced-thrombotic-microangiopathy
#11
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/29326853/purtscher-like-retinopathy-associated-with-atypical-hemolytic-uremic-syndrome
#12
Melih Ustaoğlu, Feyza Önder, Nilgün Solmaz, Savaş Öztürk, Mesut Ayer
A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure)...
December 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/29313573/p-vivax-malaria-presenting-as-thrombotic-microangiopathy
#13
Dharmendra Bhadauria, Harsh Vardhan, Anupma Kaul, Raj Kumar Sharma, Amit Gupta, Narayan Prasad, Manoj Jain
Introduction: Acute kidney injury (AKI) is reported to occur in patients with falciparum malaria but not uncommon with vivax malaria. AKI, anemia, thrombocytopenia and jaundice is a recurrent finding in severe malaria and can mimic as thrombotic microangiopathy (TMA). Relationship of malaria with TMA is unclear till date however evidences suggest their association. Methods & Material: We reviewed our electronic database to evaluate relationship of malaria with TMA, of cases of malaria, jaundice and AKI...
September 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29312707/rare-causes-of-hyperbilirubinemia-after-lung-transplantation-our-experience-at-a-single-center
#14
Su Hwan Lee, Moo Suk Park, Jin Gu Lee, Joo Han Song, Kyung Soo Chung, Ji Ye Jung, Eun Young Kim, Young Sam Kim, Se Kyu Kim, Joon Chang, Hyo Chae Paik, Song Yee Kim
Background: Lung transplantation is the last treatment option for end-stage lung disease, and the number of lung transplantations has been steadily increasing. Hyperbilirubinemia is a rare complication after lung transplantation. The aim of this study was to review rare causes of hyperbilirubinemia after lung transplantation at our center. Methods: In this single-center study, we retrospectively reviewed the records of 116 consecutive lung transplantation patients who underwent lung transplantation at Severance Hospital and Gangnam Severance Hospital of Yonsei University College of Medicine in South Korea between December 22, 2010 and January 1, 2016...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29308213/case-series-of-3-patients-diagnosed-with-atypical-hemolytic-uremic-syndrome-successfully-treated-with-steroids-plasmapheresis-and-rituximab
#15
Jeffery M Patterson, Lauren Bolster, Loree Larratt
Rationale: Atypical hemolytic uremic syndrome, which has a high probability of chronic kidney disease, morbidity, and mortality, needs to be promptly recognized when patients present with microangiopathic hemolysis. Presenting Concerns of the Patient: Three patients present with laboratory parameters consistent with a thrombotic microangiopathy. With a suspected diagnosis of thrombotic thrombocytopenic purpura, steroids with plasmapheresis were initiated. Diagnoses: With ADAMTS13 levels reported normal, the suspected diagnoses were reevaluated...
2018: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/29302904/acute-kidney-injury-in-pregnancy-with-special-reference-to-pregnancy-specific-disorders-a-hospital-based-study-2014-2016
#16
Jai Prakash, Vivek C Ganiger, Suraj Prakash, Mohammad Iqbal, Deba Prasad Kar, Usha Singh, Ashish Verma
INTRODUCTION: There are numerous reports in the literature describing acute kidney injury in pregnancy (P-AKI) due to various obstetric complications. However, there is a dearth of studies on AKI related to pregnancy-specific disorders from India. We aimed to analyze clinical features and outcome of P-AKI related to pregnancy-specific disorders compared to total pregnancy, in India. METHOD: All pregnant women attending the department of Obstetrics and Gynecology from November 2014 to July 2016 were screened for AKI based on: (1) sudden elevation of serum creatinine ≥ 1 mg/dl; (2) oligoanuria for > 12 h; and (3) need for dialysis...
January 4, 2018: Journal of Nephrology
https://www.readbyqxmd.com/read/29301470/risk-of-complications-of-ultrasound-guided-renal-biopsy-for-adult-and-pediatric-patients-with-systemic-lupus-erythematosus
#17
Y S Sun, I T Sun, H K Wang, A H Yang, C Y Tsai, C J Huang, D F Huang, C C Lai
Objective The objective of this paper is to identify the risk of complications of real-time ultrasound-guided renal biopsy in adult and pediatric patients with systemic lupus erythematosus (SLE). Materials and methods This retrospective study examined outcomes of 296 renal biopsy procedures in 275 SLE patients. Imaging-confirmed symptomatic hematoma was regarded as a major complication when intervention (blood transfusion, angiographic embolization, or surgery) was required or as a minor complication otherwise...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29296809/in-vitro-evidence-of-complement-activation-in-transplantation-associated-thrombotic-microangiopathy
#18
Seth J Rotz, Nathan Luebbering, Bradley P Dixon, Eleni Gavriilaki, Robert A Brodsky, Christopher E Dandoy, Sonata Jodele, Stella M Davies
Transplantation-associated thrombotic microangiopathy is associated with complement activation in vitro.This data further supports the use of eculizumab for the treatment of patients with TA-TMA.
September 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296757/rituximab-prophylaxis-to-prevent-thrombotic-thrombocytopenic-purpura-relapse-outcome-and-evaluation-of-dosing-regimens
#19
John-Paul Westwood, Mari Thomas, Ferras Alwan, Vickie McDonald, Sylvia Benjamin, William A Lester, Gillian C Lowe, Tina Dutt, Quentin A Hill, Marie Scully
Acute antibody-mediated thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with high morbidity and mortality. Rituximab is highly effective as prophylaxis in patients at risk of acute TTP relapse, but the ideal dosing regimen is unknown. A multicenter retrospective cohort study evaluated outcomes of patients given rituximab prophylaxis to prevent TTP relapse. Rituximab was given in 76 episodes to 45 patients (34 women and 11 men). Four once-per-week infusions of standard- (375 mg/m2 [24 episodes]), reduced- (200 mg [19 episodes]), and intermediate- (500 mg [17 episodes]) dose rituximab were given; in the remaining 16 episodes, patients received 100 to 1000 mg rituximab in 1 to 5 doses...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29288923/early-versus-late-institution-of-plasma-exchange-therapy-in-pregnancy-induced-thrombotic-microangiopathy-and-its-effects-on-clinical-outcomes-a-case-series
#20
Anupama Kaul, Kavita Vishwakarma, Dharmendra Singh Bhaduaria, Prabhaker Mishra, Narayan Prasad, Manoj Jain, Venkatesh Thammshetti, Manas R Patel, Amit Gupta, Raj Kumar Sharma
PURPOSE: To look for incidence of pregnancy associated TMA, clinical presentation and impact of early diagnosis and institution of plasma exchange on overall renal outcomes METHODS: -This is a retrospective study among all female patients who presented with acute kidney injury post pregnancy between October 2002 to April 2016 in department of nephrology in a tertiary care hospital in northern India and diagnosed as pregnancy induced TMA. The patient were assessed for duration of onset of renal failure to time of diagnosis of TMA, role of modality of treatment ie plasmpaharesis to outcome...
December 21, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
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