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https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#1
REVIEW
K Kottke-Marchant
Thrombotic micro-angiopathies (TMA) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMAs is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura (TTP) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin-related hemolytic-uremic syndrome (Stx-HUS) and complement-mediated TMA (also called atypical hemolytic-uremic syndrome), respectively...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28445600/caplacizumab-reduces-the-frequency-of-major-thromboembolic-events-exacerbations-and-death-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#2
F Peyvandi, M Scully, J A Kremer Hovinga, P Knöbl, S Cataland, K De Beuf, F Callewaert, H De Winter, R K Zeldin
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk for thrombotic complications, exacerbations and death. In the Phase II TITAN study, treatment with caplacizumab, an anti-vWF Nanobody(®) , was shown to reduce the time to confirmed platelet count normalization and exacerbations during treatment. OBJECTIVE: The clinical benefit of caplacizumab was further investigated in a post-hoc analysis of the incidence of major thromboembolic events and exacerbations during the study drug treatment period and TTP-related death during the study...
April 26, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#3
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28442312/expert-statements-on-the-standard-of-care-in-critically-ill-adult-patients-with-atypical-haemolytic-uraemic-syndrome
#4
REVIEW
Elie Azoulay, Paul Knoebl, José Garnacho-Montero, Katerina Rusinova, Gennadii Galstian, Philippe Eggimann, Fekri Abroug, Dominique Benoit, Michael von Bergwelt-Baildon, Julia Wendon, Marie Scully
BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU...
April 22, 2017: Chest
https://www.readbyqxmd.com/read/28440611/the-meningioma-1-mn1-gene-is-an-independent-poor-prognostic-factor-in-adult-egyptian-acute-myeloid-leukemia-patients
#5
Roxan E Shafik, Naglaa M Hassan, Yomna M El Meligui, Hanan E Shafik
Aim: To determine the prognostic importance of meningioma 1 (MN1) gene expression levels in the context of other predictive markers for acute myeloid leukemia (AML) cases. Methods: MN1 expression was measured in 85 newly diagnosed adults younger than 60 years by real-time reverse-transcriptase polymerase chain reaction. Results: At diagnosis 67.4% of cases had elevated MN1 expression, this being associated with a worse prognosis, higher incidence of lymphadenopathy and CD34 transcript expression (p=0.02 and <0...
March 1, 2017: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/28434898/bacteraemia-due-to-ampc-%C3%AE-lactamase-producing-escherichia-coli-in-hospitalized-cancer-patients-risk-factors-antibiotic-therapy-and-outcomes
#6
Qing Zhang, Wenfang Zhang, Zheng Li, Changsen Bai, Ding Li, Shan Zheng, Peng Zhang, Sihe Zhang
AmpC β-lactamase-producing Escherichia coli (AmpC-EC) is one of the main antimicrobial resistant pathogens in patients with cancer. A cohort study was performed to evaluate the risk factors, antibiotic therapy, and outcomes of AmpC-EC bacteraemia in hospitalized cancer patients from September 2012 through December 2015. Two hundred forty-eight cases of E. coli bacteraemia were documented in cancer patients, 51 (20.6%) were caused by AmpC-EC and 197 (79.4%) were caused with non-AmpC-EC. Prior exposure to cephalosporins (OR 2...
April 12, 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/28432084/topoisomerase2%C3%AE-and-thymidylate-synthase-expression-in-adrenocortical-cancer
#7
Elisa Roca, Alfredo Berruti, Silviu Sbiera, Ida Rapa, Ester Oneda, Paola Sperone, Cristina L Ronchi, Laura Ferrari, Salvatore Grisanti, Antonina Germano, Barbara Zaggia, Giorgio Scagliotti, Martin Fassnacht, Marco Volante, Massimo Terzolo, Mauro Papotti
Topoisomerase II alpha (TOP2A) and Thymidylate Synthase (TS) are known prognostic parameters in several tumors and also predictors of efficacy of anthracyclines, topoisomerase inhibitors and fluoropirimidines, respectively. Expression of TOP2A and TS mRNA was assessed in 98 patients with adrenocortical carcinoma (ACC) and protein expression was assessed by immunohistochemistry in a subset of 39 tumors. Ninety-two patients were radically resected for stage II-III disease and 38 of them received adjuvant mitotane...
April 21, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28429931/heterobimetallic-nitrido-complexes-of-group-8-metalloporphyrins
#8
Wai-Man Cheung, Wai-Hang Chiu, Matthew de Vere-Tucker, Herman H-Y Sung, Ian D Williams, Wa-Hung Leung
Heterobimetallic nitrido porphyrin complexes with the [(L)(por)M-N-M'(LOEt)Cl2] formula {por(2-) = 5,10,15,20-tetraphenylporphyrin (TPP(2-)) or 5,10,15,20-tetra(p-tolyl)porphyrin (TTP(2-)) dianion; LOEt(-) = [Co(η(5)-C5H5){P(O)(OEt)2}3](-); M = Fe, Ru, or Os; M' = Ru or Os; L = H2O or pyridine} have been synthesized, and their electrochemistry has been studied. Treatment of trans-[Fe(TPP)(py)2] (py = pyridine) with Ru(VI) nitride [Ru(LOEt)(N)Cl2] (1) afforded Fe/Ru μ-nitrido complex [(py)(TPP)Fe(μ-N)Ru(LOEt)Cl2] (2)...
April 21, 2017: Inorganic Chemistry
https://www.readbyqxmd.com/read/28424082/subventricular-zones-new-key-targets-for-glioblastoma-treatment
#9
J Khalifa, F Tensaouti, A Lusque, B Plas, J-A Lotterie, A Benouaich-Amiel, E Uro-Coste, V Lubrano, E Cohen-Jonathan Moyal
BACKGROUND: We aimed to identify subventricular zone (SVZ)-related prognostic factors of survival and patterns of recurrence among patients with glioblastoma. METHODS: Forty-three patients with primary diagnosed glioblastoma treated in our Cancer Center between 2006 and 2010 were identified. All patients received surgical resection, followed by temozolomide-based chemoradiation. Ipsilateral (iSVZ), contralateral (cSVZ) and bilateral (bSVZ) SVZs were retrospectively segmented and radiation dose-volume histograms were generated...
April 20, 2017: Radiation Oncology
https://www.readbyqxmd.com/read/28423691/predicting-clinical-benefit-from-everolimus-in-patients-with-advanced-solid-tumors-the-cpct-03-study
#10
Fleur Weeber, Geert A Cirkel, Marlous Hoogstraat, Sander Bins, Christa G M Gadellaa-van Hooijdonk, Salo Ooft, Erik van Werkhoven, Stefan M Willems, Marijn van Stralen, Wouter B Veldhuis, Nicolle J M Besselink, Hugo M Horlings, Neeltje Steeghs, Maja J de Jonge, Marlies H G Langenberg, Lodewyk F A Wessels, Edwin P J G Cuppen, J H Schellens, Stefan Sleijfer, Martijn P Lolkema, Emile E Voest
BACKGROUND: In this study, our aim was to identify molecular aberrations predictive for response to everolimus, an mTOR inhibitor, regardless of tumor type. METHODS: To generate hypotheses about potential markers for sensitivity to mTOR inhibition, drug sensitivity and genomic profiles of 835 cell lines were analyzed. Subsequently, a multicenter study was conducted. Patients with advanced solid tumors lacking standard of care treatment options were included and underwent a pre-treatment tumor biopsy to enable DNA sequencing of 1,977 genes, derive copy number profiles and determine activation status of pS6 and pERK...
March 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423370/transarterial-chemoembolization-combined-with-jie-du-granule-preparation-improves-the-survival-outcomes-of-patients-with-unresectable-hepatocellular-carcinoma
#11
Hetong Zhao, Xiaofeng Zhai, Zhe Chen, Xuying Wan, Lanyu Chen, Feng Shen, Changquan Ling
The aim of the present study was to compare the effectiveness of transarterial chemoembolization (TACE), TACE combined with Jie-du granules (JD), and TACE combined with sorafenib (SOR) for treating patients with unresectable hepatocellular carcinoma (HCC). For this purpose, we conducted a retrospective analysis of data from 266 consecutive patients with unresectable HCC who underwent TACE treatment at the Shanghai Hospital and Eastern Hepatic Surgery Hospital between Jan 2009 and Dec 2010. We prospectively analyzed patient survival and progression times as well as independent predictors, within a follow-up period of 86 months...
April 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28417064/adenocarcinoma-of-the-lung-presenting-as-thrombotic-thrombocytopenic-purpura
#12
Ahmed Dirweesh, Waqas Siddiqui, Muhammad Khan, Praneet Iyer, Marc Seelagy
Acquired thrombotic thrombocytopenic purpura (TTP) usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. It occurs in approximately three in one million adults and 1 in 10 million children annually. The incidence is increased in females and blacks. Diagnosing TTP and initiating therapy with plasma exchange is a must to avoid preventable complications. Acquired thromotic microangiopathy has been linked to collagen vascular diseases, use of certain medications, organ transplants, infections, pregnancy and cancer...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#13
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#14
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28413093/-thrombotic-thrombocytopenia-purpura-in-martinique-retrospective-study-between-2008%C3%A2-and-2015
#15
M Patient, P Fuseau, C Deligny
INTRODUCTION: Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique. METHODS: We retrospectively analysed all patients with TTP diagnosed at the Fort-de-France hospital between 2008, January 1st and 2015, December 31st...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28412316/differentiating-stroke-and-seizure-in-acute-setting-perfusion-computed-tomography
#16
Katarzyna Kubiak-Balcerewicz, Urszula Fiszer, Ewa Nagańska, Cezary Siemianowski, Aleksander Sobieszek, Agnieszka Witak-Grzybowska, Aldona Kosińska-Szot
BACKGROUND: Perfusion computed tomography (PCT) is part of acute stroke protocol in many hospitals; however, its clinical utility is still being disputed. Beyond its use in core and penumbra estimation, there is also a question about PCT role in stroke mimics diagnosis. Case series or small, retrospective studies showed equivocal results. This is the first published prospective, comparative study on PCT in differentiating stroke and seizure in acute setting. METHODS: Patients with acute focal neurologic deficits and without acute ischemic lesions on routine CT underwent PCT and electroencephalography (EEG) within 12 hours after symptom onset...
April 13, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28411877/combined-resection-and-radiofrequency-ablation-versus-transarterial-embolization-for-intermediate-stage-hepatocellular-carcinoma-a-propensity-score-matching-study
#17
Wendell Espinosa, Yueh-Wei Liu, Chih-Chi Wang, Chih-Che Lin, Jing-Houng Wang, Sheng-Nan Lu, Chao-Hung Hung
BACKGROUND/PURPOSE: This study aimed to compare the outcomes of combined hepatic resection (HR) plus intraoperative radiofrequency ablation (RFA) and transarterial embolization (TAE) for Barcelona Clinic Liver Cancer (BCLC) stage B hepatocellular carcinoma (HCC) in case-controlled patient groups using the propensity score. METHODS: A total of 179 patients with multifocal HCC treated with HR plus RFA (n = 26) or TAE (n = 153) were retrospectively studied. All patients were classified as BCLC stage B and Child-Pugh class A...
April 13, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28410592/study-of-sv40-large-t-antigen-nucleotide-specificity-for-dna-unwinding
#18
Damian Wang, Ana Lucia Álvarez-Cabrera, Xiaojiang S Chen
BACKGROUND: Simian Virus 40 (SV40) Large Tumor Antigen (LT) is an essential enzyme that plays a vital role in viral DNA replication in mammalian cells. As a replicative helicase and initiator, LT assembles as a double-hexamer at the SV40 origin to initiate genomic replication. In this process, LT converts the chemical energy from ATP binding and hydrolysis into the mechanical work required for unwinding replication forks. It has been demonstrated that even though LT primarily utilizes ATP to unwind DNA, other NTPs can also support low DNA helicase activity...
April 14, 2017: Virology Journal
https://www.readbyqxmd.com/read/28410208/tristetraprolin-inhibits-mitochondrial-function-through-suppression-of-%C3%AE-synuclein-expression-in-cancer-cells
#19
Mai-Tram Vo, Seong Hee Choi, Ji-Heon Lee, Chung Hwan Hong, Jong Soo Kim, Unn Hwa Lee, Hyung-Min Chung, Byung Ju Lee, Jeong Woo Park, Wha Ja Cho
Mitochondrial dynamics play critical roles in maintaining mitochondrial functions. Here, we report a novel mechanism for regulation of mitochondrial dynamics mediated by tristetraprolin (TTP), an AU-rich element (ARE)-binding protein. Overexpression of TTP resulted in elongated mitochondria, down-regulation of mitochondrial oxidative phosphorylation, reduced membrane potential, cytochrome c release, and increased apoptotic cell death in cancer cells. TTP overexpression inhibited the expression of α-Synuclein (α-Syn)...
March 30, 2017: Oncotarget
https://www.readbyqxmd.com/read/28409550/erk-and-p38-upregulation-versus-bcl-6-downregulation-in-rat-kidney-epithelial-cells-exposed-to-prolonged-hypoxia
#20
Fengbao Luo, Jian Shi, Qianqian Shi, Xiaozhou He, Ying Xia
Hypoxia is a common cause of kidney injury and a major issue in kidney transplantation. Mitogen-activated protein kinases (MAPKs) are involved in the cellular response to hypoxia, but the precise roles of MAPKs in renal cell reaction to hypoxic stress are not well known yet. This work was conducted to investigate the regulation of ERK1/2 and p38 and their signaling-relevant molecules in kidney epithelial cells exposed to prolonged hypoxia. Rat kidney epithelial cells NRK-52E were exposed to hypoxic conditions (1% O2) for 24-72 hrs...
April 12, 2017: Cell Transplantation
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