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thrombotic microangiopathies

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https://www.readbyqxmd.com/read/28911789/a-retrospective-study-of-pregnancy-associated-atypical-hemolytic-uremic-syndrome
#1
Ana Huerta, Emilia Arjona, Jose Portoles, Paula Lopez-Sanchez, Cristina Rabasco, Mario Espinosa, Teresa Cavero, Miquel Blasco, Mercedes Cao, Joaquin Manrique, Virginia Cabello-Chavez, Marta Suñer, Manuel Heras, Xavier Fulladosa, Lara Belmar, Amparo Sempere, Carmen Peralta, Lorena Castillo, Alvaro Arnau, Manuel Praga, Santiago Rodriguez de Cordoba
Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy resulting from uncontrolled complement activation during pregnancy or the postpartum period. Pregnancy-associated aHUS is a devastating disease for which there is a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data of 22 cases of pregnancy-associated aHUS from the Spanish aHUS Registry under different treatments. Sixteen patients presented during the first pregnancy and as many as nine patients required hemodialysis at diagnosis...
September 11, 2017: Kidney International
https://www.readbyqxmd.com/read/28905254/use-of-eculizumab-in-a-systemic-lupus-erythemathosus-patient-presenting-thrombotic-microangiopathy-and-heterozygous-deletion-in-cfhr1-cfhr3-a-case-report-and-systematic-review
#2
REVIEW
Maria Izabel de Holanda, Luis Cristóvão Pôrto, Teresa Wagner, Luis Fernando Christiani, Lilian M P Palma
The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports...
September 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28890263/-secondary-thrombotic-microangiopathies
#3
P Coppo
Thrombotic microangiopathies (TMA) are termed secondary when associated to a specific context favouring their occurrence. They encompass mainly TMA associated with pregnancy, allogeneic hematopoietic stem cell transplantation, cancer, drugs, or HIV infection. Secondary TMA represent a heterogeneous group of diseases which clinical presentation largely depends on the associated context. It is therefore mandatory to recognize these conditions since they have a significant impact in TMA management and prognosis...
September 7, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28885079/a-review-of-renal-disease-in-children-with-hiv-infection
#4
Ankur Kumar Jindal, Karalanglin Tiewsoh, Rakesh Kumar Pilania
Human immunodeficiency virus (HIV) infection continues to be a leading cause of morbidity and mortality. HIV-infected individuals are now surviving for a relatively longer period and this is because of easy accessibility to antiretroviral therapy these days. As a result, chronic disease-related complications are now being recognized more often. Kidney disease in HIV-infected children can vary from glomerular to tubular-interstitial involvement. We searched the database to identify various kidney diseases seen in HIV-infected children...
September 8, 2017: Infectious Diseases
https://www.readbyqxmd.com/read/28884355/influenza-associated-thrombotic-microangiopathies
#5
REVIEW
Martin Bitzan, Jakub Zieg
Thrombotic microangiopathy (TMA) refers to phenotypically similar disorders, including hemolytic uremic syndromes (HUS) and thrombotic thrombocytopenic purpura (TTP). This review explores the role of the influenza virus as trigger of HUS or TTP. We conducted a literature survey in PubMed and Google Scholar using HUS, TTP, TMA, and influenza as keywords, and extracted and analyzed reported epidemiological and clinical data. We identified 25 cases of influenza-associated TMA. Five additional cases were linked to influenza vaccination and analyzed separately...
September 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28884034/snakebite-induced-thrombotic-microangiopathy-leading-to-renal-cortical-necrosis
#6
Ying Mao Gn, Arvind Ponnusamy, Vikram Thimma
Renal complications from snakebite result in high mortality and morbidity. Acute kidney injury (AKI) occurs in 5-30% of cases. Renal manifestation could include acute tubular necrosis, cortical necrosis, interstitial nephritis, glomerulonephritis, and vasculitis. We present a case of thrombotic microangiopathy (TMA) resulting in renal cortical necrosis. Renal biopsy showed fibrin thrombi in glomeruli and arterioles with cortical necrosis. Our patient progressed to end-stage renal disease.
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28882938/imatinib-dramatically-alleviates-pulmonary-tumour-thrombotic-microangiopathy-induced-by-gastric-cancer
#7
Kana Kubota, Taro Shinozaki, Yasushi Imai, Kazuomi Kario
Pulmonary tumour thrombotic microangiopathy (PTTM) is a rare complication of cancer, which can be lethal due to progressive pulmonary hypertension (PH). Several case reports have demonstrated that imatinib, a platelet-derived growth factor receptor-tyrosine kinase inhibitor, can improve severe PH in patients with PTTM.We describe the case of a 56-year-old woman. Her mean pulmonary arterial pressure (mPAP) was 47 mm Hg, and her dyspnoea worsened rapidly over several days. Although pulmonary embolism was not observed on CT, enlargement of the para-aortic lymph nodes was detected...
September 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28882367/defining-the-phenotype-of-antibody-mediated-rejection-in-kidney-transplantation-advances-in-diagnosis-of-antibody-injury
#8
REVIEW
Neetika Garg, Milagros D Samaniego, Dana Clark, Arjang Djamali
The diagnostic criteria for antibody-mediated rejection (ABMR) are constantly evolving in light of the evidence. Inclusion of C4d-negative ABMR has been one of the major advances in the Banff Classification in recent years. Currently Banff 2015 classification requires evidence of donor specific antibodies (DSA), interaction between DSA and the endothelium, and acute tissue injury (in the form of microvasculature injury (MVI); acute thrombotic microangiopathy; or acute tubular injury in the absence of other apparent cause)...
August 15, 2017: Transplantation Reviews
https://www.readbyqxmd.com/read/28877521/case-report-pulmonary-tumor-thrombotic-microangiopathy-in-a-cervical-cancer-patient
#9
Naoya Tsujimoto, Ai Miyoshi, Mayuko Mimura, Toshio Naka, Masaru Yamasaki, Takeshi Yokoi
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rapidly progressive and often fatal pulmonary disease induced by tumor emboli within the small pulmonary arteries. PTTM presents clinically as progressive hypoxia and pulmonary hypertension. Most cases of PTTM are caused by an adenocarcinoma of the stomach. We present the first case report of PTTM caused by cervical squamous cell carcinoma. An 82-year-old woman presented with vaginal bleeding and exertional dyspnea. A cervical mass biopsy showed squamous cell carcinoma...
September 7, 2017: Gynecologic and Obstetric Investigation
https://www.readbyqxmd.com/read/28868021/a-case-of-recurrent-breast-cancer-identified-by-pulmonary-tumor-thrombotic-microangiopathy
#10
Tomomi Abe, Ippei Fukada, Taro Shiga, Hidetomo Morizono, Koichi Ikebata, Tomoko Shibayama, Kokoro Kobayashi, Takuji Iwase, Shinji Ohno, Yoshinori Ito
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare, cancer-related, pulmonary complication that causes hypoxia and pulmonary hypertension. We report on a 42-year-old woman who was diagnosed with recurrent breast cancer that was detected due to the presence of PTTM. Eleven months after surgery for heterochronous bilateral cancer of the left breast, she developed progressive dyspnea but computerized tomography showed no pulmonary thromboembolism, and a transthoracic echocardiography revealed mild pulmonary hypertension...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28864711/meningococcal-b-vaccine-failure-with-a-penicillin-resistant-strain-in-a-young-adult-on-long-term-eculizumab
#11
Sydel R Parikh, Jay Lucidarme, Coralie Bingham, Paul Warwicker, Tim Goodship, Ray Borrow, Shamez N Ladhani
We describe a case of invasive meningococcal disease due to a vaccine-preventable and penicillin-resistant strain in a fully immunized young adult on long-term complement inhibitor therapy and daily penicillin chemoprophylaxis. Eculizumab is a humanized monoclonal antibody that binds human complement C5 protein and inhibits the terminal complement pathway. It is currently recommended for the treatment of complement-mediated thrombotic microangiopathies. An unwanted complication of inhibiting complement, however, is an increased risk of invasive meningococcal disease...
September 2017: Pediatrics
https://www.readbyqxmd.com/read/28864336/analysis-of-transfusion-related-acute-lung-injury-and-possible-transfusion-related-acute-lung-injury-reported-to-the-french-hemovigilance-network-from-2007-to-2013
#12
REVIEW
Georges Andreu, Karim Boudjedir, Jean-Yves Muller, Elodie Pouchol, Yves Ozier, Guillaume Fevre, Chantal Gautreau, Jean-François Quaranta, Christian Drouet, Claire Rieux, Paul-Michel Mertes, Benoit Clavier, Monique Carlier, Imad Sandid
Using the French Hemovigilance Network database from 2007 to 2013, we provide information on demographics, incidence, and risk factors of reported transfusion-related acute lung injury (TRALI) and possible TRALI, analyze TRALI mitigation efforts for fresh frozen plasma and platelet concentrates, and consider the impact of platelet additive solutions on TRALI incidence. We applied the Toronto consensus conference definitions for TRALI and possible TRALI. Two TRALI subgroups were considered: "antibody positive" when a donor has human leukocyte antigen (class I or II) and/or human neutrophil antigen antibodies and the recipient has cognate antigen, and "antibody negative" when immunological investigation is negative or not done...
July 15, 2017: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/28860002/thrombotic-microangiopathy-after-allogeneic-stem-cell-transplantation-a-comparison-of-eculizumab-therapy-and-conventional-therapy
#13
Stephan R Bohl, Florian Kuchenbauer, Stefanie von Harsdorf, Nadine Kloevekorn, Stefan S Schönsteiner, Arefeh Rouhi, Phyllis Schwarzwälder, Hartmut Döhner, Donald Bunjes, Martin Bommer
We report the results of a single center analysis of a cohort of 39 patients treated between 1997 and 2016 for transplantion-associated thrombotic microangiopathy. We evaluated two subgroups of patients: 24 patients treated between 1997 and 2014 who received conventional therapy and 15 patients who were treated with the complement-inhibiting monoclonal antibody eculizumab between 2014 and 2016. The conventional therapy group was predominantly treated with defibrotide alone or in combination with plasmapheresis or rituximab...
August 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28858176/midterm-outcomes-of-12-renal-transplant-recipients-treated-with-eculizumab-to-prevent-atypical-hemolytic-syndrome-recurrence
#14
Charlène Levi, Véronique Frémeaux-Bacchi, Julien Zuber, Marion Rabant, Magali Devriese, Renaud Snanoudj, Anne Scemla, Lucile Amrouche, Arnaud Mejean, Christophe Legendre, Rebecca Sberro-Soussan
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is an orphan disease with a high rate of recurrence after kidney transplantation. However, reports of successful prevention of posttransplant aHUS recurrence with eculizumab emerged a few years ago. To further delineate its optimal use, we describe the largest series of kidney transplant recipients treated with prophylactic eculizumab. METHODS: Twelve renal transplant recipients with aHUS-related end stage renal disease received eculizumab: 10 from day 0 and 2 at the time of recurrence (days 6 and 25)...
August 25, 2017: Transplantation
https://www.readbyqxmd.com/read/28856466/tma-secondary-to-sle-rituximab-improves-overall-but-not-renal-survival
#15
Fangfang Sun, Xiaodong Wang, Wanlong Wu, Kaiwen Wang, Zhiwei Chen, Ting Li, Shuang Ye
Thrombotic microangiopathy (TMA) includes a series of life-threatening disorders. Systemic lupus erythematosus (SLE) is one of the most common acquired causes. To identify predictors of prognosis in patients with TMA secondary to SLE, we conducted a single-center historical study. From January 2013 to June 2016, of 2182 SLE hospitalized patients in the Ren Ji Hospital, a total of 21 consecutive patients with TMA secondary to SLE were identified. The 90-day short-term mortality was 33.3%. The kidney involvement (66...
August 30, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28855497/a-49-year-old-man-with-subacute-respiratory-failure-and-interstitial-lung-opacities
#16
Anastasiia A Rudkovskaia, Ying-Chun Lo, Virginia Brady, Jose Costa, Wassim H Fares
BACKGROUND Pulmonary arterial hypertension (PAH) results from proliferative vasculopathy involving all layers of the blood vessel. Similar findings may be present in pulmonary hypertension (PH) associated with microscopic tumor embolism, which are thought to be related to the phenomenon of pulmonary tumor thrombotic microangiopathy (PTTM). PTTM is associated with the activation of the coagulation system at the surface of the tumor emboli, resulting in stenosis or occlusion of the vessel. CASE REPORT A 49-year-old man with stage IV gastro-esophageal junction adenocarcinoma presented with complaints of cough and shortness of breath...
August 31, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28836275/impact-of-treatment-and-outcomes-for-patients-with-posttransplant-drug-associated-thrombotic-microangiopathy
#17
Narendranath Epperla, Kathleen Hemauer, Mehdi Hamadani, Kenneth D Friedman, Lisa Baumann Kreuziger
BACKGROUND: Drug-induced transplant-associated thrombotic microangiopathy (DTA-TMA) is a rare but serious complication that can occur after hematopoietic cell transplantation (HCT) or solid organ transplantation (SOT) without guidelines for optimal management of this condition. STUDY DESIGN AND METHODS: Given the ambiguity surrounding the treatment for DTA-TMA, we conducted a retrospective review to evaluate the impact of different treatment strategies in DTA-TMA patients...
August 23, 2017: Transfusion
https://www.readbyqxmd.com/read/28835895/antiangiogenic-therapy-for-diabetic-nephropathy
#18
REVIEW
Katsuyuki Tanabe, Yohei Maeshima, Yasufumi Sato, Jun Wada
Angiogenesis has been shown to be a potential therapeutic target for early stages of diabetic nephropathy in a number of animal experiments. Vascular endothelial growth factor (VEGF) is the main mediator for abnormal angiogenesis in diabetic glomeruli. Although beneficial effects of anti-VEGF antibodies have previously been demonstrated in diabetic animal experiments, recent basic and clinical evidence has revealed that the blockade of VEGF signaling resulted in proteinuria and renal thrombotic microangiopathy, suggesting the importance of maintaining normal levels of VEGF in the kidneys...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28835862/late-onset-cobalamin-disorder-and-hemolytic-uremic-syndrome-a-rare-cause-of-nephrotic-syndrome
#19
Gianluigi Ardissino, Michela Perrone, Francesca Tel, Sara Testa, Amelia Morrone, Ilaria Possenti, Francesco Tagliaferri, Robertino Dilena, Francesca Menni
Hemolytic uremic syndrome (HUS) is an unrare and severe thrombotic microangiopathy (TMA) caused by several pathogenetic mechanisms among which Shiga toxin-producing Escherichia coli infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC) can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS) related to CblC disease which first presented in a previously healthy boy at age of 13...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28833243/a-novel-homozygous-frameshift-mutation-in-exon-7-of-the-adamts13-gene-in-a-patient-with-congenital-thrombotic-thrombocytopenic-purpura-from-india-a-case-report
#20
Sneha Yadav, Shrimati Shetty, Bipin Kulkarni
BACKGROUND: Thrombotic thrombocytopenia purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. It is caused by deficiency of ADAMTS13 metalloprotease, which cleaves ultra-large von Willebrand factor into smaller functional units. TTP may be congenital or acquired, and the congenital form is caused by inherited mutations in the ADAMTS13 gene, leading to deficiency of protein or reduced protein activity...
August 21, 2017: Transfusion
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