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Thrombotic microangiopathy

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https://www.readbyqxmd.com/read/28226413/thrombalexin-use-of-a-cytotopic-anticoagulant-to-reduce-thrombotic-microangiopathy-in-a-highly-sensitized-model-of-kidney-transplantation
#1
Miriam Manook, Jean Kwun, Christian Burghuber, Kannan Samy, Michael Mulvihill, Janghoon Yoon, He Xu, Andrea L MacDonald, Kyle Freischlag, Verna Curfman, Evelyn Branum, David Howell, Alton Brad Farris, Richard A Smith, Stephen Sacks, Anthony Dorling, Nizam Mamode, Stuart Knechtle
Early activation of coagulation is an important factor in the initiation of innate immunity, as characterized by thrombotic microangiopathy (TMA). In transplantation, systemic anti-coagulation is difficult due to bleeding. A novel 'cytotopic' agent, 'Thrombalexin', (TLN) combines a cell-membrane bound (mirystoyl tail) anti-thrombin (HLL peptide) which can be perfused directly to the donor organ or cells.. Thromboelastography (TEG) was used to measure time to clot formation (r-time) in both rhesus and human blood, comparing TLN vs...
February 22, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28217316/subacute-right-heart-failure-revealing-three-simultaneous-causes-of-post-embolic-pulmonary-hypertension-in-metastatic-dissemination-of-breast-cancer
#2
Flavien Vincent, Nicolas Lamblin, Marion Classe, Guillaume Schurtz, Antoine Rauch, Marie Fertin, Pascal De Groote
A 72-year-old woman with history of breast cancer only treated surgically was referred to our department for pulmonary hypertension (PH) suspicion. Echocardiogram revealed elevated right ventricular systolic pressure. Computed tomography (CT) angiogram showed no pulmonary embolism (PE), but lung scan revealed two ventilation-perfusion mismatch areas. Right cardiac catheterization established precapillary PH. Despite treatment with PH specific therapy (sildenafil, ambrisentan, and epoprostenol), her condition worsened rapidly with acute right heart failure (RHF)...
February 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#3
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28209752/opana-er-induced-thrombotic-microangiopathy
#4
Bernhard Lämmle
No abstract text is available yet for this article.
February 16, 2017: Blood
https://www.readbyqxmd.com/read/28207978/glomerular-c4d-deposits-can-mark-structural-capillary-wall-remodeling-in-thrombotic-microangiopathy-and-transplant-glomerulopathy-c4d-beyond-active-antibody-mediated-injury
#5
Adil H Gasim, Jamie S Chua, Ron Wolterbeek, John Schmitz, Eric Weimer, Harsharan K Singh, Volker Nickeleit
Peritubular capillary C4d (ptc-C4d) usually marks active antibody mediated rejection, while pseudolinear glomerular capillary C4d (GBM-C4d) is of undetermined diagnostic significance, especially when seen in isolation without concurrent ptc-C4d. We correlated GBM-C4d with structural GBM abnormalities and active antibody mediated rejection in 319 renal transplant and 35 control native kidney biopsies. In kidney transplants ptc-C4d was associated with GBM-C4d in 97% by immunofluorescence microscopy (IF) and 61% by immunohistochemistry (IHC; p<0...
February 16, 2017: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/28202168/paraprotein-associated-thrombotic-microangiopathy-expanding-the-spectrum-of-renal-disease-related-to-plasma-cell-dyscrasias
#6
Mercury Lin, Mark Haas
Plasma cell dyscrasias are associated with a variety of renal diseases, most resulting from the deposition of intact or altered monoclonal paraproteins within the renal parenchyma. Much less commonly, renal disease resulting from monoclonal gammopathies has been reported without actual accumulation of the paraprotein or a derivative of it within the kidney. One such instance involves thrombotic microangiopathy (TMA), which is a consequence of endothelial cell injury. New data from the Mayo Clinic indicate that the association of TMA with monoclonal gammopathies is far more frequent than previously appreciated...
March 2017: Kidney International
https://www.readbyqxmd.com/read/28198690/thrombotic-microangiopathies-similar-presentations-different-therapies
#7
REVIEW
Gerald B Appel
Thrombotic thrombocytopenic purpura, Shiga toxin hemolytic uremic syndrome, atypical hemolytic uremic syndrome, and antiphospholipid syndrome are thrombotic microangiopathies that present similarly but arise from different causes. Management depends on distinguishing them promptly and providing targeted therapy.
February 2017: Cleveland Clinic Journal of Medicine
https://www.readbyqxmd.com/read/28195256/two-cases-of-sudden-death-due-to-pulmonary-tumor-thrombotic-microangiopathy-caused-by-occult-gastric-carcinoma
#8
M Ben Khelil, Y Chkirbene, H Azzouz, S Haouet, M Hamdoun
We present two cases of occult gastric carcinoma associated to a large pulmonary tumors thrombosis microangiopathy (PTTM). The first case is a 28 years-old man. He was dead due to a respiratory failure. Autopsy showed a whitish indurated mass invading the stomach wall. Histological findings showed a primary "signet ring" gastric adenocarcinoma with pulmonary carcinomatosis and multiple PTTM and a heart metastasis. The second case is a 24 years-old pregnant woman. The main symptoms were nausea and stomach discomfort and they were seen as pregnancy signs...
December 2016: Pathologica
https://www.readbyqxmd.com/read/28187980/patients-with-hypertension-associated-thrombotic%C3%A2-microangiopathy-may-present-with%C3%A2-complement-abnormalities
#9
Sjoerd A M E G Timmermans, Myrurgia A Abdul-Hamid, Joris Vanderlocht, Jan G M C Damoiseaux, Chris P Reutelingsperger, Pieter van Paassen
Thrombotic microangiopathy (TMA) is a pattern of endothelial damage that can be found in association with diverse clinical conditions such as malignant hypertension. Although the pathophysiological mechanisms differ, accumulating evidence links complement dysregulation to various TMA syndromes and in particular the atypical hemolytic uremic syndrome. Here, we evaluated the role of complement in nine consecutive patients with biopsy-proven renal TMA attributed to severe hypertension. Profound hematologic symptoms of TMA were uncommon...
February 7, 2017: Kidney International
https://www.readbyqxmd.com/read/28183278/a-rare-case-of-renal-thrombotic-microangiopathy-associated-with-castleman-s-disease
#10
Anubha Mutneja, L Nicholas Cossey, Helen Liapis, Ying Maggie Chen
BACKGROUND: Castleman's disease (CD) is an uncommon, heterogeneous lympho-proliferative disorder leading to high circulating levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). Renal involvement has been only described in a limited number of small studies. Herein, we report a rare case of renal thrombotic microangiopathy (TMA) associated with CD and investigate the podocyte expression of VEGF in the renal biopsy prior to initiation of treatment. CASE PRESENTATION: An 18-year-old male presented with fever, diarrhea, diffuse lymphadenopathy, ascites and acute kidney injury...
February 10, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28182045/thrombotic-microangiopathy-an-unusual-cause-of-renal-failure-in-rheumatoid-arthritis
#11
R Sakthirajan, J Dhanapriya, T Dineshkumar, N Gopalakrishnan, S Murugan, T Balasubramaniyan
Rheumatoid arthritis (RA) is one of the commonest rheumatological diseases. Renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. Only a few cases of TMA in patients with RA were reported to date. We describe a 45-year-old female patient with RA who presented with oliguria and edema. Renal biopsy showed TMA with patchy cortical necrosis...
January 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28178879/prognostic-significance-of-renal-vascular-pathology-in-lupus-nephritis
#12
J M Mejía-Vilet, B M Córdova-Sánchez, N O Uribe-Uribe, R Correa-Rotter, L E Morales-Buenrostro
We performed a retrospective cohort analysis to define the prognostic significance of vascular lesions documented in renal biopsies of lupus nephritis patients. A total of 429 patients were segregated into five groups: (1) no vascular lesions (NVL), (2) arterial sclerosis (AS), (3) non-inflammatory necrotizing vasculitis (NNV), (4) thrombotic microangiopathy (TMA), and (5) true renal vasculitis (TRV). Renal outcomes were analyzed by Cox regression models, and correlations between vascular lesions and activity/chronicity scores were determined by Spearman's coefficients...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28178713/thrombotic-microangiopathy-in-a-patient-treated-with-gemcitabine
#13
Sowmya Nanjappa, Vivek Singh, Shyam Uttamchandani, Smitha Pabbathi
Thrombotic microangiopathy syndromes consist of a collection of disorders with a varied etiology that share common clinical and pathological features. Although thrombotic microangiopathy is rare, it is associated with significant morbidity and mortality. Without early recognition and intervention, the prognosis of the disease is poor. This report illustrates the case of a 56-year-old man with advanced-stage metastatic pancreatic cancer who presented with hemolytic uremic syndrome associated with gemcitabine use...
January 2017: Cancer Control: Journal of the Moffitt Cancer Center
https://www.readbyqxmd.com/read/28178155/complement-mediated-thrombotic-microangiopathy-secondary-to-sepsis-induced-disseminated-intravascular-coagulation-successfully-treated-with-eculizumab-a-case-report
#14
Tomohiro Abe, Akira Sasaki, Taichiro Ueda, Yoshitaka Miyakawa, Hidenobu Ochiai
Secondary thrombotic microangiopathies (TMAs) are induced by several underlying conditions; most are resolved by treating background disease. Eculizumab is a human monoclonal antibody that blocks the final stage of the complement system and effectively treats atypical hemolytic uremic syndrome (aHUS). In this report, we present a patient with TMA secondary to sepsis- induced coagulopathy, who was successfully treated with eculizumab.A 44-year-old woman, who had no special medical history or familial history of TMAs, was admitted on suspicion of septic shock...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28176480/de-novo-thrombotic-microangiopathy-following-simultaneous-pancreas-and-kidney-transplantation-managed-with-eculizumab
#15
REVIEW
Lani Shochet, John Kanellis, Ian Simpson, Joseph Ta, William Mulley
Thrombotic microangiopathy (TMA) is a well-recognised complication following transplantation, often due to an underlying genetic predisposition, medications or rejection. The use of eculizumab in these settings has been previously described, but its role still remains to be clarified. A 45-year-old man, with a history of type 1 diabetes mellitus and subsequent end-stage kidney failure, presented for a simultaneous pancreas-kidney transplant. Immunologically, he was well matched with the donor, and he received standard induction immunosuppression including tacrolimus...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28176476/recurrent-atypical-haemolytic-uraemic-syndrome-post-kidney-transplant-due-to-a-cd46-mutation-in-the-setting-of-smarcal1-mediated-inherited-kidney-disease
#16
REVIEW
Samuel Chan, Andrew J Mallett, Chirag Patel, Ross S Francis, David W Johnson, David W Mudge, Nicole M Isbel
Disorders in the regulation of the alternate complement pathway often result in complement-mediated damage to the microvascular endothelium and can be associated with both glomerulonephritis and atypical haemolytic uraemic syndrome. Inherited defects in complement regulatory genes or autoantibodies against complement regulatory proteins are predictive of the severity of the disease and the risk of recurrence post kidney transplantation. Heterozygous mutations in CD46, which codes for a transmembrane cofactor glycoprotein membrane cofactor protein, usually have a lower incidence of end-stage kidney disease and decreased risk of recurrent disease post transplant, as wild-type membrane cofactor protein is present in the transplanted kidney...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28176474/monoclonal-gammopathy-of-renal-significance-triggering-atypical-haemolytic-uraemic-syndrome
#17
REVIEW
Usman Mahmood, Nicole Isbel, Peter Mollee, Andrew Mallett, Sridevi Govindarajulu, Ross Francis
Haemolytic uraemic syndrome is a rare condition with an overall incidence of one to two cases in a population of 100 000 and approximately 10% of these cases are classified as atypical. Atypical haemolytic uraemic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and acute kidney injury. aHUS can be genetic, acquired or idiopathic (negative genetic screening and no environmental triggers). We describe a case of aHUS triggered by monoclonal gammopathy of renal significance (MGRS) successfully treated with plasmapheresis and a bortezomib-based chemotherapy regimen, resulting in marked improvement in renal function and other markers of haemolysis...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28164557/the-detection-of-urinary-podocytes-from-drug-induced-glomerular-thrombotic-microangiopathy-in-advanced-cancer-patients
#18
Joichi Usui, Chie Yokoyama, Masahiro Hagiwara, Kai Hirayasu, Takahiro Kojima, Takayuki Yoshino, Hiroyuki Nishiyama, Masanori Hara, Kunihiro Yamagata
BACKGROUND: Focusing on glomerular thrombotic microangiopathy (TMA), we detected urinary podocytes to evaluate podocyte damage following glomerular endothelial cell injury. METHODS: We analyzed the relationship between urinary podocytes as biomarkers for podocyte injuries and clinical manifestations in five patients of anti-cancer-drug-induced glomerular TMA. RESULTS: Cancer in all five patients was advanced, including 4 cases of renal cell carcinoma treated with tyrosine kinase inhibitor, and one with intrahepatic bile duct carcinoma treated with gemcitabine...
December 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28150184/thrombotic-thrombocytopenic-purpura-in-a-new-onset-lupus-patient
#19
Nery Sablón González, Noel Lorenzo, Yanet Parodis, Maria Belen Alonso Ortiz, Melek Kechida, Jose Carlos Rodriguez Perez
We are presenting a case of TTP with undetectable levels of ADAMTS 13 in a 39-year-old woman. Diagnosis of systemic lupus was evoked in the setting of thrombotic microangiopathy. The patient presented normal renal function but important neurological impairment. Treatment with daily plasmapheresis as well as Rituximab, cyclophosphamide as steroids was required to achieve clinical improvement.
February 2, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28139439/neutrophil-protease-cleavage-of-von-willebrand-factor-in-glomeruli-an-anti-thrombotic-mechanism-in-the-kidney
#20
Ramesh Tati, Ann-Charlotte Kristoffersson, Minola Manea Hedström, Matthias Mörgelin, Jörgen Wieslander, Cees van Kooten, Diana Karpman
Adequate cleavage of von Willebrand factor (VWF) prevents formation of thrombi. ADAMTS13 is the main VWF-cleaving protease and its deficiency results in development of thrombotic microangiopathy. Besides ADAMTS13 other proteases may also possess VWF-cleaving activity, but their physiological importance in preventing thrombus formation is unknown. This study investigated if, and which, proteases could cleave VWF in the glomerulus. The content of the glomerular basement membrane (GBM) was studied as a reflection of processes occurring in the subendothelial glomerular space...
January 24, 2017: EBioMedicine
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