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thrombotic microangiopathies

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https://www.readbyqxmd.com/read/28303518/potentially-life-threatening-coagulopathy-associated-with-simultaneous-reduction-in-coagulation-and-fibrinolytic-function-in-pediatric-acute-leukemia-after-hematopoietic-stem-cell-transplantation
#1
Takashi Ishihara, Keiji Nogami, Tomoko Matsumoto, Akitaka Nomura, Yasufumi Takeshita, Satoshi Ochi, Midori Shima
The pathogenesis of sinusoidal obstruction syndrome (SOS) and thrombotic microangiopathy (TMA) after hematopoietic stem cell transplantation (HSCT) is poorly understood, and limited information is available on global hemostatic function in HSCT. We assessed changes in coagulation and fibrinolysis using a simultaneous thrombin and plasmin generation assay (T/P-GA) during HSCT. Measurements of endogenous thrombin potential (T-EP) and plasmin peak height (P-Peak) using T/P-GA in six pediatric acute leukemia patients treated with HSCT were compared to normal plasma...
March 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28292995/-a-case-of-stage-iv-breast-cancer-with-long-term-survival-by-chemotherapy-developing-pulmonary-tumor-thrombotic-microangiopathy-during-the-treatment-course
#2
Kana Kobayashi, Akari Murakami, Saaya Komatsu, Kanako Nishiyama, Michiko Yamashita, Wakana Sugimori, Yoshiaki Kamei, Hiroaki Asai, Akiyoshi Ogimoto, Kazuo Honda, Yasutsugu Takada
Pulmonary tumor thrombotic microangiopathy(PTTM)caused by pulmonary artery microscopic tumor emboli and fibrocellular and/or fibromuscular proliferation leads to progressive pulmonary hypertension and respiratory failure.The prognosis is extremely poor and most patients die shortly after onset.We report a patient with Stage IV breast cancer and long-term survival who developed PTTM during chemotherapy treatment.A 63-year-old woman with multiple metastases in her cerebellum, bone, lung, and lymph node after left breast conserving surgery started to experience dyspnea and malaise 7 years after the surgery...
March 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#3
Carole Philipponnet, Jean-Louis Kemeny, Cyril Garrouste, Martin Soubrier, Anne-Elisabeth Heng
Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits...
March 14, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28287636/transplant-associated-thrombotic-microangiopathy-opening-pandora-s-box
#4
REVIEW
E Gavriilaki, I Sakellari, A Anagnostopoulos, R A Brodsky
Transplant-associated thrombotic microangiopathy (TA-TMA) is an early complication of hematopoietic cell transplantation (HCT). A high mortality rate is documented in patients who are refractory to calcineurin inhibitor cessation. Estimates of TA-TMA prevalence vary significantly and are higher in allogeneic compared with autologous HCT. Furthermore, our understanding of the pathophysiology that is strongly related to diagnosis and treatment options is limited. Recent evidence has linked TA-TMA with atypical hemolytic uremic syndrome, a disease of excessive activation of the alternative pathway of complement, opening the Pandora's box in treatment options...
March 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28275964/reversal-of-threatening-blindness-after-initiation-of-eculizumab-in-purtscher-like-retinopathy-secondary-to-atypical-hemolytic-uremic-syndrome
#5
J E Ramos de Carvalho, R O Schlingemann, M Oranje, F J Bemelman, M J van Schooneveld
Purtscher-like retinopathy, a rare manifestation of systemic thrombotic microangiopathy, is a potentially visually debilitating condition with no effective proven treatment. Distinct pathogenic pathways have been proposed as etiological factors. We revisit the etiology of Purtscher-like retinopathy based on the rapid response and profound visual improvement after initiation of systemic intravenous eculizumab, an inhibitor of the complement cascade, in a patient with Purtscher-like retinopathy secondary to familial atypical hemolytic uremic syndrome (aHUS) due to a mutation in complement factor H...
March 8, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28271596/team-based-approach-to-identify-cardiac-toxicity-in-critically-ill-hematopoietic-stem-cell-transplant-recipients
#6
Christopher E Dandoy, Sonata Jodele, Zachary Paff, Russel Hirsch, Thomas D Ryan, John L Jefferies, Michelle Cash, Seth Rotz, Abigail Pate, Michael D Taylor, Javier El-Bietar, Kasiani C Myers, Gregory Wallace, Adam Nelson, Michael Grimley, Thomas Pfeiffer, Adam Lane, Stella M Davies, Ranjit S Chima
INTRODUCTION: We observed pulmonary hypertension (PH), pericardial effusions, and left ventricular systolic dysfunction (LVSD) in multiple critically ill hematopoietic stem cell transplant (HSCT) recipients. We implemented routine structured echocardiography screening for HSCT recipients admitted to the pediatric intensive care unit (PICU) using a standardized multidisciplinary process. METHODS: HSCT recipients admitted to the PICU with respiratory distress, hypoxia, shock, and complications related to transplant-associated thrombotic microangiopathy were screened on admission and every 1-2 weeks thereafter...
March 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28265287/pernicious-anemia-associated-cobalamin-deficiency-and-thrombotic-microangiopathy-case-report-and-review-of-the-literature
#7
Farhanah Yousaf, Bruce Spinowitz, Chaim Charytan, Marilyn Galler
A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28259520/derivation-and-external-validation-of-the-plasmic-score-for-rapid-assessment-of-adults-with-thrombotic-microangiopathies-a-cohort-study
#8
Pavan K Bendapudi, Shelley Hurwitz, Ashley Fry, Marisa B Marques, Stephen W Waldo, Ang Li, Lova Sun, Vivek Upadhyay, Ayad Hamdan, Andrew M Brunner, John M Gansner, Srinivas Viswanathan, Richard M Kaufman, Lynne Uhl, Christopher P Stowell, Walter H Dzik, Robert S Makar
BACKGROUND: Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed. METHODS: All consecutive adult patients presenting to three large academic medical centres in Boston, MA, USA, with thrombotic microangiopathy and a possible diagnosis of thrombotic thrombocytopenic purpura between Jan 8, 2004, and Dec 6, 2015, were included in an ongoing multi-institutional registry (the Harvard TMA Research Collaborative)...
March 1, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28258475/expanding-the-therapeutic-options-for-renal-involvement-in-lupus-eculizumab-available-evidence
#9
REVIEW
Savino Sciascia, Massimo Radin, Jinoos Yazdany, Maria Tektonidou, Irene Cecchi, Dario Roccatello, Maria Dall'Era
In this study, we aimed to systematically review available literature on the efficacy of eculizumab for the treatment of renal involvement in patients with systemic lupus erythematosus (SLE). We conducted a literature search developed a priori, to identify articles reporting clinical experience with the use of eculizumab in SLE patients, focusing on renal involvement. The search strategy was applied to Ovid MEDLINE, EMBASE, In-Process and Other Non-Indexed Citation, Cochrane Central Register of Controlled Trials and Scopus from 2006 to present...
March 3, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28248832/pathophysiology-of-pediatric-multiple-organ-dysfunction-syndrome
#10
Joseph A Carcillo, Bradley Podd, Rajesh Aneja, Scott L Weiss, Mark W Hall, Timothy T Cornell, Thomas P Shanley, Lesley A Doughty, Trung C Nguyen
OBJECTIVE: To describe the pathophysiology associated with multiple organ dysfunction syndrome in children. DATA SOURCES: Literature review, research data, and expert opinion. STUDY SELECTION: Not applicable. DATA EXTRACTION: Moderated by an experienced expert from the field, pathophysiologic processes associated with multiple organ dysfunction syndrome in children were described, discussed, and debated with a focus on identifying knowledge gaps and research priorities...
March 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28248187/fatal-proteinuric-kidney-disease-in-a-30-month-old-german-fleckvieh-heifer-caused-by-unilateral-focal-segmental-glomerulosclerosis-subsequent-to-a-non-functional-counterpart-kidney
#11
W Breuer, V Nickeleit, M Metzner, G Knubben-Schweizer, A Hafner-Marx
A case of secondary focal segmental glomerulosclerosis (FSGS) in a heifer is presented. A 30-month-old female German Fleckvieh heifer showed deterioration of the general condition, a poor nutritional status, proteinuria, hypoalbuminemia, and renal azotemia. Pathologically, it was diagnosed with unilateral hydronephrosis, and contralateral renal fibrosis with numerous cysts. Histologically, the fibrotic kidney showed FSGS, hyaline reabsorption droplets in proximal tubular epithelial cells, interstitial fibrosis, and tubulointerstitial inflammation...
March 2017: Schweizer Archiv Für Tierheilkunde
https://www.readbyqxmd.com/read/28242109/haemolytic-uraemic-syndrome
#12
REVIEW
Fadi Fakhouri, Julien Zuber, Véronique Frémeaux-Bacchi, Chantal Loirat
Haemolytic uraemic syndrome is a form of thrombotic microangiopathy affecting predominantly the kidney and characterised by a triad of thrombocytopenia, mechanical haemolytic anaemia, and acute kidney injury. The term encompasses several disorders: shiga toxin-induced and pneumococcus-induced haemolytic uraemic syndrome, haemolytic uraemic syndrome associated with complement dysregulation or mutation of diacylglycerol kinase ɛ, haemolytic uraemic syndrome related to cobalamin C defect, and haemolytic uraemic syndrome secondary to a heterogeneous group of causes (infections, drugs, cancer, and systemic diseases)...
February 23, 2017: Lancet
https://www.readbyqxmd.com/read/28226413/thrombalexin-use-of-a-cytotopic-anticoagulant-to-reduce-thrombotic-microangiopathy-in-a-highly-sensitized-model-of-kidney-transplantation
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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