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https://www.readbyqxmd.com/read/27913483/atypical-hemolytic-uremic-syndrome
#1
Vahid Afshar-Kharghan
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) that affects multiple organs and the kidneys in particular. aHUS can be sporadic or familial and is most commonly caused by dysregulation of the alternative complement pathway. The initial attack of aHUS can occur at any age, and is associated with a high rate of progression to end stage renal disease. Many aHUS patients relapse in the native or transplanted kidneys, and require close monitoring and long-term management. Availability of anticomplement therapy has revolutionized the management of aHUS, and can change the natural course of aHUS by inducing hematologic remission, improving or stabilizing kidney functions, and preventing graft failure...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27892947/danaparoid-reduces-the-incidence-of-hematopoietic-stem-cell-transplantation-associated-thrombotic-microangiopathy
#2
S Machida, M Onizuka, M Toyosaki, Y Aoyama, H Kawai, J Amaki, R Hara, A Ichiki, Y Ogawa, H Kawada, K Ando
No abstract text is available yet for this article.
November 28, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27885340/acute-kidney-injury-in-hematopoietic-stem-cell-transplantation-a-review
#3
REVIEW
Vinod Krishnappa, Mohit Gupta, Gurusidda Manu, Shivani Kwatra, Osei-Tutu Owusu, Rupesh Raina
Hematopoietic stem cell transplantation (HSCT) is a highly effective treatment strategy for lymphoproliferative disorders and bone marrow failure states including aplastic anemia and thalassemia. However, its use has been limited by the increased treatment related complications, including acute kidney injury (AKI) with an incidence ranging from 20% to 73%. AKI after HSCT has been associated with an increased risk of mortality. The incidence of AKI reported in recipients of myeloablative allogeneic transplant is considerably higher in comparison to other subclasses mainly due to use of cyclosporine and development of graft-versus-host disease (GVHD) in allogeneic groups...
2016: International Journal of Nephrology
https://www.readbyqxmd.com/read/27882637/transplantation-associated-thrombotic-microangiopathy-isolated-to-a-congenital-anomaly-of-the-lung
#4
Rebecca T Kummen, Geoffrey D E Cuvelier, Camelia Stefanovici, Anamarija M Perry, Rick Higgins, Rochelle Yanofsky, Suyin A Lum Min, Donna A Wall
TA-TMA is a post-hematopoietic stem cell transplant complication with clinical features of hemolytic anemia and thrombocytopenia. A 26-month-old child who had had an allogeneic transplant for treatment of DBA developed severe TA-TMA with heavy red blood cell and platelet transfusion dependence. Incidentally, he was found to have a lung sequestration. TA-TMA resolved and transfusion dependence resolved after resection of the sequestration. The finding suggests the malformation vasculature was selectively vulnerable to the trigger of TA-TMA-raising perhaps a clue to basic pathophysiology of TA-TMA and/or vascular malformations...
November 24, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27868334/consensus-on-the-standardization-of-terminology-in-thrombotic-thrombocytopenic-purpura-and-related-thrombotic-microangiopathies
#5
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic Microangiopathy is a broad pathophysiological process that leads to microangiopathic hemolytic anemia, thrombocytopenia and involves capillary and small vessel platelet aggregates. The most common cause being disseminated intravascular coagulation (DIC), which may be differentiated by abnormal coagulation. Clinically, a number of conditions present with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT), including cancer, infection, transplantation, drugs, autoimmune disease and pre-eclampsia and HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome in pregnancy...
November 21, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27865549/anaemia-and-fever-in-kidney-transplant-the-role-of-human-parvovirus-b19
#6
Yanet Parodis López, Raquel Santana Estupiñán, Silvia Marrero Robayna, Roberto Gallego Samper, Fernando Henríquez Palop, José Carlos Rivero Vera, Rafael Camacho Galán, María José Pena López, Nery Sablón González, Fayna González Cabrera, Elena Oliva Dámaso, Nicanor Vega Díaz, José Carlos Rodríguez Pérez
Infections remain an issue of particular relevance in renal transplant patients, particularly viral infections. Human parvovirus B19 infection causes severe refractory anaemia, pancytopenia and thrombotic microangiopathy. Its presence is recognized by analysing blood polymerase chain reaction (PCR) and by the discovery of typical giant proerythroblasts in the bone marrow. We report the case of a 65 year-old man with a history of deceased donor renal transplant in September 2014. At 38 days after the transplant, the patient presented progressive anaemia that was resistant to erythropoiesis-stimulating agents...
November 16, 2016: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/27861383/living-donor-renal-transplantation-in-patients-with-antiphospholipid-syndrome-a-case-report
#7
Ji Yoon Choi, Joo Hee Jung, Sung Shin, Young Hoon Kim, Duck Jong Han
INTRODUCTION: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. CLINICAL FINDINGS: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27848226/atypical-hemolytic-uremic-syndrome-in-the-setting-of-complement-amplifying-conditions-case-reports-and-a-review-of-the-evidence-for-treatment-with-eculizumab
#8
REVIEW
Arif Asif, Ali Nayer, Christian S Haas
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, progressive, life-threatening form of thrombotic microangiopathy (TMA) predominantly caused by dysregulation of the alternative pathway of the complement system. Complement-amplifying conditions (CACs), including pregnancy complications [preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome], malignant hypertension, autoimmune diseases, transplantation, and others, are associated with the onset of TMA in up to 69 % of cases of aHUS...
November 15, 2016: Journal of Nephrology
https://www.readbyqxmd.com/read/27830476/production-of-heterozygous-alpha-1-3-galactosyltransferase-ggta1-knock-out-transgenic-miniature-pigs-expressing-human-cd39
#9
Kimyung Choi, Joohyun Shim, Nayoung Ko, Heejong Eom, Jiho Kim, Jeong-Woong Lee, Dong-Il Jin, Hyunil Kim
Production of transgenic pigs for use as xenotransplant donors is a solution to the severe shortage of human organs for transplantation. The first barrier to successful xenotransplantation is hyperacute rejection, a rapid, massive humoral immune response directed against the pig carbohydrate GGTA1 epitope. Platelet activation, adherence, and clumping, all major features of thrombotic microangiopathy, are inevitable results of immune-mediated transplant rejection. Human CD39 rapidly hydrolyzes ATP and ADP to AMP; AMP is hydrolyzed by ecto-5'-nucleotidase (CD73) to adenosine, an anti-thrombotic and cardiovascular protective mediator...
November 9, 2016: Transgenic Research
https://www.readbyqxmd.com/read/27823643/an-overview-of-hematopoietic-stem-cell-transplantation-related-thrombotic-complications
#10
REVIEW
Ugur Sahin, Pinar Ataca Atilla, Erden Atilla, Selami Kocak Toprak, Taner Demirer
Thrombotic episodes are far less common than bleeding complications after hematopoietic stem cell transplantation (HSCT). However, they lead to significant morbidity and mortality. These complications are classified into four groups, including venous thromboembolic events (VTE), catheter-induced thrombosis (CIT), transplant-associated thrombotic microangiopathy (TA-TMA) and sinusoidal obstruction syndrome (SOS) or veno-occlusive disease (VOD). The frequency of VTE is increased among patients undergoing HSCT due to some acquired conditions including underlying malignancy, infections, administration of myeloablative conditioning regimens and/or total body irradiation, prolonged hospitalizations leading to immobility and presence of central venous catheters...
November 2016: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/27800206/immune-complex-mediated-glomerulonephritis-with-acute-thrombotic-microangiopathy-following-newly-detected-hepatitis-b-virus-infection-in-a-kidney-transplant-recipient
#11
Tracey Salter, Hannah Burton, Sam Douthwaite, William Newsholme, Catherine Horsfield, Rachel Hilton
Hepatitis B virus (HBV) presents a risk to patients and staff in renal units. To minimise viral transmission, there are international and UK guidelines recommending HBV immunisation for patients commencing renal replacement therapy (RRT) and HBV surveillance in kidney transplant recipients. We report the case of a 56-year-old male who was immunised against HBV before starting haemodialysis. He received a deceased donor kidney transplant three years later, at which time there was no evidence of HBV infection...
2016: Case Reports in Transplantation
https://www.readbyqxmd.com/read/27796825/complete-response-to-post-transplant-lymphoproliferative-disorder-by-surgical-resection-and-rituximab-after-living-donor-liver-re-transplantation-for-recurrent-primary-sclerosing-cholangitis
#12
Koichiro Haruki, Hiroaki Shiba, Junichi Shimada, Norimitsu Okui, Tomonori Iida, Katsuhiko Yanaga
Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication of solid organ transplantation. We herein report a case of PTLD after living-donor liver re-transplantation (reLDLT) for recurrent primary sclerosing cholangitis (PSC), for which complete response was achieved by surgical resection and rituximab. A 47-year-old man, who had undergone living-donor liver transplantation (LDLT) twice at age of 43 and 45 years for end-stage liver disease firstly for PSC and secondary for recurrent PSC, suffered liver dysfunction due to an acute cellular rejection (ACR) 17 months after reLDLT...
October 31, 2016: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/27775697/is-complement-blockade-an-acceptable-therapeutic-strategy-for-hematopoietic-cell-transplant-associated-thrombotic-microangiopathy
#13
P Dhakal, V R Bhatt
Diagnosis and management of hematopoietic cell transplant-associated thrombotic microangiopathy (TA-TMA) are very complex and controversial, given multiple ongoing issues and comorbidities in sick transplant recipients. Complement activation via classic and alternative pathways is emerging as a potential pathogenetic mechanism in the development of TA-TMA. Complement-centric diagnostic strategy using functional and genetic tests may possibly support diagnosis, enhance molecular understanding and direct drug development...
October 24, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27771173/complement-activation-inflammation-and-relative-adamts13-deficiency-in-secondary-thrombotic-microangiopathies
#14
Péter Farkas, Dorottya Csuka, Bálint Mikes, György Sinkovits, Marienn Réti, Endre Németh, Kristóf Rácz, Krisztina Madách, Mihály Gergely, Judit Demeter, Zoltán Prohászka
BACKGROUND: The secondary forms of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (secondary TMA) emerge as complications of coexisting diseases. OBJECTIVES: We hypothesized that secondary TMA could be characterized by the presence of relative ADAMTS13 deficiency and complement activation, and this relationship may have a prognostic value for outcome. PATIENTS AND METHODS: Fifty-three patients with thrombotic microangiopathy (TMA) and coexisting disease (such as malignancies, sepsis, heart surgery with extracorporeal circulation, solid organ transplantation, systemic autoimmune disorders), 41 patient controls, and 34 healthy controls were enrolled in our case-control study with 30days follow-up...
October 18, 2016: Immunobiology
https://www.readbyqxmd.com/read/27768015/hemolytic-uremic-syndrome-in-children
#15
Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
https://www.readbyqxmd.com/read/27766045/clinical-evaluation-of-thrombotic-microangiopathy-identification-of-patients-with-suspected-atypical-hemolytic-uremic-syndrome
#16
Yu-Min Shen
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/27739559/bilateral-cavernous-sinus-thrombosis-in-a-patient-with-tacrolimus-associated-posttransplant-thrombotic-microangiopathy
#17
Anton M Kolomeyer, Ken K Nischal, Ellen Mitchell
PURPOSE: To report a case of bilateral cavernous sinus thrombosis (CST) in a patient with tacrolimus-associated posttransplant thrombotic microangiopathy. METHODS: Case report. RESULTS: An 8-year-old boy with a medical history of orthotopic heart transplant, posttransplant lymphoproliferative disease, and recurrent infections was hospitalized for nausea, vomiting, and diarrhea. His ocular history included accommodative esotropia, hyperopia with astigmatism, Molluscum contagiosum lid lesions, and idiopathic intracranial hypertension...
October 13, 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/27723152/haemolytic-uraemic-syndrome
#18
Diana Karpman, Sebastian Loos, Ramesh Tati, Ida Arvidsson
Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains...
October 10, 2016: Journal of Internal Medicine
https://www.readbyqxmd.com/read/27696719/bk-polyomavirus-encephalitis-in-a-patient-with-thrombotic-microangiopathy-after-an-allogeneic-hematopoietic-stem-cell-transplant
#19
Jae-Bum Jun, Yunsuk Choi, Hawk Kim, Sun Ho Lee, Joseph Jeong, Jiwon Jung
To date, only 1 case of BK polyomavirus (BKPyV) encephalitis combined with transplant-associated thrombotic microangiopathy has been reported in a hematopoietic stem cell transplantation (HCT) recipient. We report the case of a HCT recipient who developed thrombotic microangiopathy and subsequent BKPyV encephalitis. She died despite treatment with cidofovir, ciprofloxacin, and intravenous immunoglobulin without improvement in mental status. Early suspicion of BKPyV encephalitis in a HCT recipient presenting with altered mentality and hemorrhagic cystitis is important...
October 1, 2016: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/27641135/scleroderma-renal-crisis-and-renal-involvement-in-systemic-sclerosis
#20
Thasia G Woodworth, Yossra A Suliman, Daniel E Furst, Philip Clements
Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients...
November 2016: Nature Reviews. Nephrology
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