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thrombotic microangiopathies

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https://www.readbyqxmd.com/read/28092354/use-of-defibrotide-to-treat-transplant-associated-thrombotic-microangiopathy-a-retrospective-study-of-the-paediatric-diseases-and-inborn-errors-working-parties-of-the-european-society-of-blood-and-marrow-transplantation
#1
L Yeates, M A Slatter, S Bonanomi, F L W I Lim, S Y Ong, A Dalissier, W Barberi, A Shulz, M Duval, C Heilmann, A Willekens, W H Y Hwang, C Uderzo, P Bader, A R Gennery
No abstract text is available yet for this article.
January 16, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28089409/the-italian-registry-of-therapeutic-apheresis-2015
#2
REVIEW
Giustina De Silvestro
Data collection on apheresis activities in Italy throughout 2015 including techniques, clinical indications and adverse effects was performed by means of a SidEM model questionnaire (aggregate data). These data provided by 67 Apheresis Units from 17 Italian regions, albeit rough, are sufficiently informative, mainly in comparison with previous surveys on these statistics. PEx has been the most frequent technique for plasma removal/treatment (11787 procedures) followed by LDL-apheresis (927 procedures). ECP/photopheresis has been the most frequent cytapheresis procedure: 6,606 session, mainlyby using the off-line technique...
December 29, 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28078430/blood-urea-nitrogen-to-serum-creatinine-ratio-is-an-accurate-predictor-of-outcome-in-diarrhea-associated-hemolytic-uremic-syndrome-a-preliminary-study
#3
Werner Keenswijk, Jill Vanmassenhove, Ann Raes, Evelyn Dhont, Johan Vande Walle
: Diarrhea-associated hemolytic uremic syndrome (D+HUS) is a common thrombotic microangiopathy during childhood and early identification of parameters predicting poor outcome could enable timely intervention. This study aims to establish the accuracy of BUN-to-serum creatinine ratio at admission, in addition to other parameters in predicting the clinical course and outcome. Records were searched for children between 1 January 2008 and 1 January 2015 admitted with D+HUS. A complicated course was defined as developing one or more of the following: neurological dysfunction, pancreatitis, cardiac or pulmonary involvement, hemodynamic instability, and hematologic complications while poor outcome was defined by death or development of chronic kidney disease...
January 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28071761/plasma-heme-induced-renal-toxicity-is-related-to-a-capillary-rarefaction
#4
Nahid Tabibzadeh, Céline Estournet, Sandrine Placier, Joëlle Perez, Héloïse Bilbault, Alexis Girshovich, Sophie Vandermeersch, Chantal Jouanneau, Emmanuel Letavernier, Nadjib Hammoudi, François Lionnet, Jean-Philippe Haymann
Severe hypertension can lead to malignant hypertension (MH) with renal thrombotic microangiopathy and hemolysis. The role of plasma heme release in this setting is unknown. We aimed at evaluating the effect of a mild plasma heme increase by hemin administration in angiotensin II (AngII)-mediated hypertensive rats. Prevalence of MH and blood pressure values were similar in AngII and AngII + hemin groups. MH rats displayed a decreased renal blood flow (RBF), increased renal vascular resistances (RVR), and increased aorta and interlobar arteries remodeling with a severe renal microcirculation assessed by peritubular capillaries (PTC) rarefaction...
January 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28060108/diagnostic-utility-of-complement-immunohistochemical-studies-in-post-stem-cell-transplant-intestinal-thrombotic-microangiopathy-case-report
#5
Jenna E Rossoff, Jennifer Schneiderman, Sonali Chaudhury, Nicoleta C Arva
Thrombotic complications are a significant source of morbidity and mortality following hematopoietic stem cell transplants. Among them, transplant-associated thrombotic microangiopathy (TA-TMA) is a well-recognized syndrome that can affect various organ systems. Its etiology is related to endothelial injury accompanied by complement activation. As many of the signs and symptoms of the disease are also encountered in other complications following hematopoietic stem cell transplant, it can often be difficult to establish the diagnosis based on clinical data alone...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28057640/murine-systemic-thrombophilia-and-hemolytic-uremic-syndrome-from-a-factor-h-point-mutation
#6
Yoshiyasu Ueda, Imran Mohammed, Delu Song, Damodar Gullipalli, Lin Zhou, Sayaka Sato, Yuan Wang, Shuchi Gupta, Zhongjian Cheng, Hong Wang, Jialing Bao, Yingying Mao, Lawrence Brass, X Long Zheng, Takashi Miwa, Matthew Palmer, Joshua Dunaief, Wen-Chao Song
Complement plays a key role in host defense but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure in patients. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis...
January 5, 2017: Blood
https://www.readbyqxmd.com/read/28050002/pulmonary-tumor-thrombotic-microangiopathy-caused-by-a-parotid-tumor-early-antemortem-diagnosis-and-long-term-survival
#7
Haruka Toyonaga, Michiko Tsuchiya, Chikara Sakaguchi, Hitomi Ajimizu, Yosuke Nakanishi, Seiya Nishiyama, Noboru Morikawa, Yasuyuki Hayashi, Yukio Nagasaka, Hiroshi Yasui
Pulmonary tumor thrombotic microangiopathy (PTTM) is a high-mortality disease that is difficult to diagnose clinically. Our patient was an 80-year-old woman who came to us due to symptoms of increasing dyspnea. A clinical evaluation showed that she had hypoxemia and pulmonary arterial hypertension without any abnormalities in the major pulmonary arteries, bronchi, or alveoli. A lung perfusion scan showed multiple wedge-shaped perfusion defects. Further examination revealed adenocarcinoma in her right parotid gland with metastasis to the submandibular lymph nodes...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28035470/the-clinical-and-laboratory-features-of-chinese-han-anti-factor-h-autoantibody-associated-hemolytic-uremic-syndrome
#8
Di Song, Xiao-Rong Liu, Zhi Chen, Hui-Jie Xiao, Jie Ding, Shu-Zhen Sun, Hong-Yan Liu, Wei-Yi Guo, Su-Xia Wang, Feng Yu, Ming-Hui Zhao
BACKGROUND: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS. METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study. RESULTS: All of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics...
December 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28033278/delayed-onset-of-posterior-reversible-encephalopathy-syndrome-in-a-case-of-scleroderma-renal-crisis-with-maintenance-hemodialysis-case-report-and-literature-review
#9
Ching-Yang Chen, Shin-Yuan Hung, Yi-Jer Lee, Yi-Chan Lin, Chu-Cheng Pai
INTRODUCTION: In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28029267/the-pathological-spectrum-associated-with-the-ultrastructural-finding-of-thin-glomerular-basement-membrane-a-tertiary-medical-city-experience-and-review-of-the-literature
#10
Hala Kfoury, Maria Arafah
BACKGROUND: Thin glomerular basement membrane (GBM) has been noted in several glomerular diseases including IgA nephropathy, focal segmental glomerulosclerosis (FSGS), Fabry's disease, and Alport's syndrome. We conducted this study to investigate the pathological ultrastructural spectrum of thin GBMs, to identify associated diseases, and to measure the GBM thickness in thin GBMs in our adult population. MATERIALS AND METHODS: All renal biopsies with thin GBM, diagnosed between 2010 and 2016, were retrieved and reviewed...
December 28, 2016: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28028259/-an-autopsy-case-of-pulmonary-tumor-thrombotic-microangiopathy-that-progressed-slowly-during-chemotherapy-for-advanced-gastric-cancer
#11
Nobuyasu Suzuki, Ayaka Azami, Yukitoshi Todate, Takeshi Tada, Mitsuru Waragai, Michitaka Honda, Atai Sato, Yoshinao Takano, Tsuyoshi Abe, Yasushi Teranishi
A man in his 50s presented to our hospital for anemia and was diagnosed with advanced gastric cancer with para-aortic lymph node metastases. He underwent gastrojejunostomy and received postoperative chemotherapy. Despite stable disease with chemotherapy, he complained of cough and respiratory discomfort and was subsequently admitted with progressive respiratory distress. Pulmonary hypertension and right-sided heart failure developed, and he died of sudden cardiopulmonary arrest 9 days after admission. An autopsy revealed widespread tumor metastasis, and he was diagnosed with pulmonary tumor thrombotic microangiopathy(PTTM)associated with gastric cancer...
December 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28025630/adjustment-of-eculizumab-dosage-pattern-in-patients-with-atypical-hemolytic-uremic-syndrome-with-suboptimal-response-to-standard-treatment-pattern
#12
Camino García Monteavaro, Carmen Peralta Roselló, Borja Quiroga, José María Baltar Martín, Lorena Castillo Eraso, Fernando de Álvaro Moreno, Alberto Martínez Vea, María Teresa Visus-Fernández de Manzanos
In patients with atypical hemolytic uremic syndrome (aHUS), complement blocking by eculizumab rapidly halts the process of thrombotic microangiopathy and it is associated with clear long-term hematologic and renal improvements. Eculizumab treatment consists of a 4-week initial phase with weekly IV administration of 900 mg doses, followed by a maintenance phase with a 1,200 mg dose in the fifth week and every 14 ± 2 days thereafter. We present three patients with aHUS and suboptimal response to eculizumab treatment at the usual administration dosage who showed hematologic and renal improvements after an adjustment in the eculizumab treatment protocol...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27999774/hyal-2-wwox-smad4-signaling-in-cell-death-and-anticancer-response
#13
REVIEW
Li-Jin Hsu, Ming-Fu Chiang, Chun-I Sze, Wan-Pei Su, Ye Vone Yap, I-Ting Lee, Hsiang-Ling Kuo, Nan-Shan Chang
Hyaluronidase HYAL-2 is a membrane-anchored protein and also localizes, in part, in the lysosome. Recent study from animal models revealed that both HYAL-1 and HYAL-2 are essential for the metabolism of hyaluronan (HA). Hyal-2 deficiency is associated with chronic thrombotic microangiopathy with hemolytic anemia in mice due to over accumulation of high molecular size HA. HYAL-2 is essential for platelet generation. Membrane HYAL-2 degrades HA bound by co-receptor CD44. Also, in a non-canonical signal pathway, HYAL-2 serves as a receptor for transforming growth factor beta (TGF-β) to signal with downstream tumor suppressors WWOX and SMAD4 to control gene transcription...
2016: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/27998645/thrombotic-microangiopathy-associated-with-monoclonal-gammopathy
#14
Aishwarya Ravindran, Ronald S Go, Fernando C Fervenza, Sanjeev Sethi
Thrombotic microangiopathy (TMA) is a rare disease comprising of a diverse set of disorders linked by a common histologic finding of endothelial injury. Monoclonal immunoglobulins may act as a potential trigger in the pathogenesis of TMA. To determine the prevalence of monoclonal gammopathy and clinicopathological features of TMA associated with monoclonal immunoglobulin, we performed a retrospective study in adults (18 and older) with a clinical diagnosis of TMA. Of 146 patients with TMA, we detected monoclonal immunoglobulin in 20 patients (13...
December 17, 2016: Kidney International
https://www.readbyqxmd.com/read/27990016/thrombotic-microangiopathies-vegf-complement-interactions
#15
Susan J Allison
No abstract text is available yet for this article.
December 19, 2016: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/27982687/angiotensinogen-and-interleukin-18-as-markers-of-chronic-kidney-damage-in-children-with-a-history-of-hemolytic-uremic-syndrome
#16
K Lipiec, P Adamczyk, E Świętochowska, K Ziora, M Szczepańska
Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). It is clinically important to investigate the markers of a poor prognosis. The levels of angiotensinogen (AGT) and interleukin-18 (IL-18) in serum and urine were evaluated. Study was conducted in 29 children with a history of HUS. Serum and urine AGT concentration was significantly higher in children after HUS as compared to the control group. No differences depending on the type of HUS and gender were noted...
December 16, 2016: Physiological Research
https://www.readbyqxmd.com/read/27979867/interferon-induced-thrombotic-microangiopathy
#17
James N George
No abstract text is available yet for this article.
December 15, 2016: Blood
https://www.readbyqxmd.com/read/27977428/breaking-down-the-complement-system-a-review-and-update-on-novel-therapies
#18
Yuvaram N V Reddy, Andrew M Siedlecki, Jean M Francis
PURPOSE OF REVIEW: The complement system represents one of the more primitive forms of innate immunity. It has increasingly been found to contribute to pathologies in the native and transplanted kidney. We provide a concise review of the physiology of the complement cascade, and discuss current and upcoming complement-based therapies. RECENT FINDINGS: Current agents in clinical use either bind to complement components directly or prevent complement from binding to antibodies affixed to the endothelial surface...
December 12, 2016: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/27976568/an-unexpected-diagnostic-course-of-systemic-lupus-erythematosus
#19
Aysel Taktak, Adem Köksoy, Özge Başaran, Saba Kiremitçi, Banu Acar, Nermin Uncu, Nilgün Çakar
Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27974406/thrombotic-microangiopathy-in-inverted-formin-2-mediated-renal-disease
#20
Rachel C Challis, Troels Ring, Yaobo Xu, Edwin K S Wong, Oliver Flossmann, Ian S D Roberts, Saeed Ahmed, Michael Wetherall, Giedrius Salkus, Vicky Brocklebank, Julian Fester, Lisa Strain, Valerie Wilson, Katrina M Wood, Kevin J Marchbank, Mauro Santibanez-Koref, Timothy H J Goodship, David Kavanagh
The demonstration of impaired C regulation in the thrombotic microangiopathy (TMA) atypical hemolytic uremic syndrome (aHUS) resulted in the successful introduction of the C inhibitor eculizumab into clinical practice. C abnormalities account for approximately 50% of aHUS cases; however, mutations in the non-C gene diacylglycerol kinase-ε have been described recently in individuals not responsive to eculizumab. We report here a family in which the proposita presented with aHUS but did not respond to eculizumab...
December 14, 2016: Journal of the American Society of Nephrology: JASN
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