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thrombotic microangiopathies

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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/29302904/acute-kidney-injury-in-pregnancy-with-special-reference-to-pregnancy-specific-disorders-a-hospital-based-study-2014-2016
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/29288001/-a-poems-syndrome-revealed-by-a-blue-toe-syndrome
#15
P Decker, J Galland, J Risse, M Claudin, S Mohamed, A Perrot, D Wahl
INTRODUCTION: Arterial and venous thromboses occur in almost one in five patients with POEMS syndrome and usually in macrocirculation. CASE REPORT: We report a 67-year-old male with a POEMS syndrome who presented initially with a blue toe syndrome. He complained of Raynaud's syndrome and left foot paresthesia. Physical examination showed gynecomastia, lymphadenopathies and skin lesions. Cardiovascular investigations excluded atrial fibrillation, unstable atherosclerotic lesions and vascular calcifications...
December 26, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29285109/acute-kidney-injury-in-cancer-patients-and-impedance-cardiography-assisted-renal-replacement-therapy-experience-from-the-onconephrology-unit-of-a-chinese-tertiary-hospital
#16
Tao Wang, Yan Zhang, Qingxia Li, Shumin Jia, Changjin Shi, Kai Niu, Bing Liu
Acute kidney injury (AKI) in cancer patients may disrupt anticarcinogenic treatment and greatly increase associated mortality rates. The present study reported on the management of cancer patients with AKI and, from the nephrologic viewpoint, on the significance of fine volume control during the continuous renal replacement therapy (CRRT). The records of 117 cancer patients with AKI treated over three years were reviewed and their data were compared with those of 120 healthy controls. AKI was defined according to the Kidney Disease: Improving Global Outcomes criteria with serum creatinine levels determined on initial admission and for the diagnosis of AKI...
December 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29282226/maternal-and-fetal-outcomes-of-pregnancies-in-women-with-atypical-hemolytic-uremic-syndrome
#17
Martina Gaggl, Christof Aigner, Dorottya Csuka, Ágnes Szilágyi, Zoltán Prohászka, Renate Kain, Natalja Haninger, Maarten Knechtelsdorfer, Raute Sunder-Plassmann, Gere Sunder-Plassmann, Alice Schmidt
Atypical HUS (aHUS) is a disorder most commonly caused by inherited defects of the alternative pathway of complement, or the proteins that regulate this pathway, and life-threatening episodes of aHUS can be provoked by pregnancy. We retrospectively and prospectively investigated 27 maternal and fetal pregnancy outcomes in 14 women with aHUS from the Vienna Thrombotic Microangiopathy Cohort. Seven pregnancies (26%) were complicated by pregnancy-associated aHUS (p-aHUS), of which three appeared to be provoked by infection, bleeding, and curettage, and three individuals were considered to have preeclampsia/HELLP syndrome before the definitive diagnosis of p-aHUS was made...
December 27, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29281948/a-patient-with-oxaliplatin-immune-induced-syndrome-oiis-who-also-developed-leucovorin-and-palonosetron-associated-thrombocytopenia
#18
S A Curtis, B R Curtis, A I Lee, J E Hendrickson, J Lacy, N A Podoltsev
OBJECTIVES: We report a case of an 83 year old man who developed oxaliplatin immune-induced syndrome (OIIS) after his 19th cycle of FOLFOX (5FU, leucovorin, oxaliplatin). When oxaliplatin was omitted from his next cycle of chemotherapy he continues to show signs of drug-induced immune thrombocytopenia (DITP) and was found to have drug-dependent, platelet-reactive antibodies (DDPA) to leucovorin and palonosetron as well as oxaliplatin. METHODS: The patient was admitted for monitoring but required no transfusions and thrombocytopenia resolved without treatment during his first admission...
December 28, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29273073/pulmonary-tumor-thrombotic-microangiopathy-successfully-treated-with-corticosteroids-a-case-report
#19
Shinichi Miyazaki, Takuya Ikeda, Genshi Ito, Masahide Inoue, Keiji Nara, Yuko Nishinaga, Yoshinori Hasegawa
BACKGROUND: Pulmonary tumor thrombotic microangiopathy is a special type of tumor thromboembolism. We report the case of a patient who developed pulmonary tumor thrombotic microangiopathy with alveolar hemorrhage. Almost all patients with pulmonary tumor thrombotic microangiopathy die within 1 week of the onset of dyspnea; however, the prognosis in this case was better, with 10 weeks of survival from presentation. CASE PRESENTATION: A 62-year-old Japanese man was referred to our hospital with a 4-week history of dyspnea on exertion and severe pulmonary hypertension...
December 23, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29250893/atypical-hemolytic-uremic-syndrome-a-meta-analysis-of-case-reports-confirms-the-prevalence-of-genetic-mutations-and-the-shift-of-treatment-regimens
#20
Vinod Krishnappa, Mohit Gupta, Mohamed Elrifai, Bahar Moftakhar, Michael J Ensley, Tushar J Vachharajani, Sidharth Kumar Sethi, Rupesh Raina
Atypical hemolytic uremic syndrome (aHUS) is a rare life-threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta-analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations...
December 17, 2017: Therapeutic Apheresis and Dialysis
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