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uremic hemolytic syndrom

Christina M Abrams, Diego R Hijano, Bindiya Bagga
No abstract text is available yet for this article.
2018: Global Pediatric Health
Chia Wei Teoh, Kathleen Mary Gorman, Bryan Lynch, Timothy H J Goodship, Niamh Marie Dolan, Mary Waldron, Michael Riordan, Atif Awan
Atypical hemolytic uremic syndrome (aHUS) is caused by dysregulation of the complement system. A humanised anti-C5 monoclonal antibody (eculizumab) is available for the treatment of aHUS. We present the first description of atypical HUS in a child with a coexistent diagnosis of a POL-III leukodystrophy. On standard eculizumab dosing regime, there was evidence of ongoing C5 cleavage and clinical relapses when immunologically challenged. Eculizumab is an effective therapy for aHUS, but the recommended doses may not be adequate for all patients, highlighting the need for ongoing efforts to develop a strategy for monitoring of treatment efficacy and potential individualisation of therapy...
2018: Case Reports in Nephrology
Nilgun Çakar, Z Birsin Ozcakar, Fatih Ozaltin, Mustafa Koyun, Banu Celikel Acar, Elif Bahat, Bora Gulhan, Emine Korkmaz, Ayşe Yurt, Songül Yılmaz, Oğuz Soylemezoglu, Fatoş Yalcinkaya
BACKGROUND: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS. MATERIALS AND METHODS: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry. RESULTS: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study...
March 13, 2018: Nephron
Kinnosuke Yahiro, Sayaka Nagasawa, Kimitoshi Ichimura, Hiroki Takeuchi, Kohei Ogura, Hiroyasu Tsutsuki, Takeshi Shimizu, Sunao Iyoda, Makoto Ohnishi, Hirotaro Iwase, Joel Moss, Masatoshi Noda
Shiga toxigenic Escherichia coli (STEC) are responsible for a worldwide foodborne disease, which is characterized by severe bloody diarrhea and hemolytic uremic syndrome (HUS). Subtilase cytotoxin (SubAB) is a novel AB5 toxin, which is produced by Locus for Enterocyte Effacement (LEE)-negative STEC. Cleavage of the BiP protein by SubAB induces endoplasmic reticulum (ER) stress, followed by induction of cytotoxicity in vitro or lethal severe hemorrhagic inflammation in mice. Here we found that steroids and diacylglycerol (DAG) analogues (e...
December 2018: Cell Death Discovery
Bin Yang, Shaomeng Wang, Jianxiao Huang, Zhiqiu Yin, Lingyan Jiang, Wenqi Hou, Xiaomin Li, Lu Feng
Enterohemorrhagic Escherichia coli O157:H7 is a major human enteric pathogen capable of causing large outbreaks of severe infections that induce bloody diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome. Its genome contains 177 unique O islands (OIs) including those carrying the main virulence elements, Shiga toxin-converting phages (OI-45 and OI-93) and locus for enterocyte effacement (OI-148). However, many of these islands harbor only genes of unknown function. Here, we demonstrate that OI-29 encodes a newly discovered transcriptional activator, Z0639 (named GmrA), that is required for motility and flagellar synthesis in O157:H7...
2018: Frontiers in Microbiology
M Kobrzynski, B Wile, S S Huang, G Filler
Eculizumab is the therapy of choice for patients with atypical hemolytic uremic syndrome (aHUS). Dosing recommendations stem from two trials: one retrospective trial (19 children and 5 infants) and one prospective trial (22 patients and 5 infants). This case report highlights the need for more precise dosing recommendations in children, particularly in infants, and for smaller vials of the medication to facilitate more precise dosing. Such changes would ensure that adverse events are minimized and that the children with aHUS who are treated with eculizumab experience an optimal clinical response...
January 2018: Indian Journal of Nephrology
Cheng-Ju Kuo, Sin-Tian Wang, Chia-Mei Lin, Hao-Chieh Chiu, Cheng-Rung Huang, Der-Yen Lee, Geen-Dong Chang, Ting-Chen Chou, Jenn-Wei Chen, Chang-Shi Chen
The enteric pathogen enterohemorrhagic Escherichia coli (EHEC) is responsible for outbreaks of bloody diarrhea and hemolytic uremic syndrome (HUS) worldwide. Several molecular mechanisms have been described for the pathogenicity of EHEC; however, the role of bacterial metabolism in the virulence of EHEC during infection in vivo remains unclear. Here we show that aerobic metabolism plays an important role in the regulation of EHEC virulence in Caenorhabditis elegans. Our functional genomic analyses showed that disruption of the genes encoding the succinate dehydrogenase complex (Sdh) of EHEC, including the sdhA gene, attenuated its toxicity toward C...
March 7, 2018: Cell Death & Disease
Chisa Fukasawa, Saori Ooishi, Takuma Kumagai, Megumi Koshiisi, Yuki Sueki, Kei Nakajima, Toru Mitsumori, Yoko Yoshida, Hideki Kato, Masaomi Nangaku, Toshiyuki Miyata, Keita Kirito
Herein, we present an elderly onset case of aHUS successfully treated with eculizumab. An 80-year-old woman with severe anemia, thrombocytopenia, and acute renal dysfunction was admitted to our hospital. A laboratory test revealed steep elevation in the LDH level, and the peripheral blood smear showed erythrocyte fragmentations. Accordingly, we diagnosed thrombotic microangiopathy, and treatment with plasma exchange was immediately initiated. In addition, she required hemodialysis because of rapid impairment of the renal function...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Motohiko Okano, Takeshi Matsumoto, Yoshiki Nakamori, Kazuko Ino, Kana Miyazaki, Atsushi Fujieda, Yuka Sugimoto, Isao Tawara, Motoko Yamaguchi, Kohshi Ohishi, Hiroshi Miwa, Masahiro Masuya, Hideo Wada, Naoyuki Katayama
A 23-year-old man from Mie Prefecture, Japan, with past and family history of hematuria was diagnosed with influenza A and admitted to our hospital on the following day because of hemoglobinuria. He was diagnosed with thrombotic microangiopathy and was suspected of having atypical hemolytic uremic syndrome (aHUS). C3 p.I1157T missense mutation, which we had previously reported in eight aHUS patients from six families in Mie Prefecture, was identified. The laboratory findings and symptoms of our patient promptly improved after administering eculizumab...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Christian Patry, Christian Betzen, Farnoosh Fathalizadeh, Alexander Fichtner, Jens H Westhoff, Thomas Fleming, Volker Eckstein, Tom Bruckner, Martina Bielaszewska, Helge Karch, Georg F Hoffmann, Burkhard Tönshoff, Neysan Rafat
Endothelial injury with consecutive microangiopathy and endothelial dysfunction plays a central role in the pathogenesis of the post-enteropathic hemolytic uremic syndrome (D+HUS). To identify new treatment strategies, we examined the regenerative potential of endothelial progenitor cells (EPC) in an in vitro model of Shiga toxin (Stx) 2a-induced glomerular endothelial injury present in D+HUS and the mechanisms of EPC-triggered endothelial regeneration. We simulated the pro-inflammatory milieu present in D+HUS by priming human renal glomerular endothelial cells (HRGEC) with Tumor Necrosis Factor (TNF)-α prior to stimulation with Stx2a...
March 7, 2018: American Journal of Physiology. Renal Physiology
Madoka Fujisawa, Hideki Kato, Yoko Yoshida, Tomoko Usui, Munenori Takata, Mika Fujimoto, Hideo Wada, Yumiko Uchida, Koichi Kokame, Masanori Matsumoto, Yoshihiro Fujimura, Toshiyuki Miyata, Masaomi Nangaku
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is caused by complement overactivation, and its presentation and prognosis differ according to the underlying molecular defects. The aim of this study was to characterize the genetic backgrounds of aHUS patients in Japan and to elucidate the associations between their genetic backgrounds, clinical findings, and outcomes. METHODS: We conducted a nationwide epidemiological survey of clinically diagnosed aHUS patients and examined 118 patients enrolled from 1998 to 2016 in Japan...
March 6, 2018: Clinical and Experimental Nephrology
Shuo Niu, John Paluszynski, Zhen Bian, Lei Shi, Koby Kidder, Yuan Liu
Shiga toxin (Stx)-induced hemolytic uremic syndrome (HUS) is a life-threatening complication associated with Stx-producing Escherichia coli infection. One critical barrier of understanding HUS is how Stx transports from infected intestine to kidney to cause HUS. Passive dissemination seems unlikely, while circulating blood cells have been debated to serve as the toxin carrier. Employing a murine model of Stx2-induced HUS with LPS priming (LPS-Stx2), we investigate how Stx causes HUS and identify possible toxin carrier...
March 5, 2018: Scientific Reports
(no author information available yet)
No abstract text is available yet for this article.
January 2018: Medicine (Baltimore)
Emre Tekgündüz, Mehmet Yılmaz, Mehmet Ali Erkurt, Ilhami Kiki, Ali Hakan Kaya, Leylagul Kaynar, Inci Alacacioglu, Guven Cetin, Ibrahim Ozarslan, Irfan Kuku, Gulden Sincan, Ozan Salim, Sinem Namdaroglu, Abdullah Karakus, Volkan Karakus, Fevzi Altuntas, Ismail Sari, Gulsum Ozet, Ismet Aydogdu, Vahap Okan, Emin Kaya, Rahsan Yildirim, Esra Yildizhan, Gokhan Ozgur, Osman Ilhami Ozcebe, Bahriye Payzin, Seval Akpinar, Fatih Demirkan
Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission...
February 20, 2018: Transfusion and Apheresis Science
J D Tanaro, L A Pianciola, B A D'Astek, M C Piaggio, M L Mazzeo, G Zolezzi, M Rivas
Shiga toxin-producing Escherichia coli (STEC) O157:H7 is a worldwide concern. Cattle are their main reservoir and may contaminate watercourses through manure. We characterized a collection of 38 STEC O157:H7 strains isolated from surface water in feedlots areas (puddles inside pens formed after the rainfall or by spill around drinking troughs, and small water courses and lagoons, formed by runoff). Nineteen (50.0%) strains harbored stx2a /stx2c genes, 18 (47.4%) stx2c and one stx1a /stx2c . All strains harbored eae, ehxA, rfbO157 , and fliCH 7 genes, and the putative virulence determinants ECSP_0242, ECSP_2687 and ECSP_3620...
March 3, 2018: Letters in Applied Microbiology
Amy J Osborne, Matteo Breno, Nicolo Ghiringhelli Borsa, Fengxiao Bu, Véronique Frémeaux-Bacchi, Daniel P Gale, Lambertus P van den Heuvel, David Kavanagh, Marina Noris, Sheila Pinto, Pavithra M Rallapalli, Giuseppe Remuzzi, Santiago Rodríguez de Cordoba, Angela Ruiz, Richard J H Smith, Paula Vieira-Martins, Elena Volokhina, Valerie Wilson, Timothy H J Goodship, Stephen J Perkins
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are associated with dysregulation and overactivation of the complement alternative pathway. Typically, gene analysis for aHUS and C3G is undertaken in small patient numbers, yet it is unclear which genes most frequently predispose to aHUS or C3G. Accordingly, we performed a six-center analysis of 610 rare genetic variants in 13 mostly complement genes ( CFH , CFI , CD46 , C3 , CFB , CFHR1 , CFHR3 , CFHR4 , CFHR5 , CFP , PLG , DGKE , and THBD ) from >3500 patients with aHUS and C3G...
March 2, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Lauren J Lee, Kristine J Roland, Gayatri M Sreenivasan, Leslie N Zypchen, Kimberley L S Ambler, Paul R Yenson
Solvent detergent-treated plasma (SDP) is a pathogen-inactivated blood plasma, which in comparison to frozen plasma is associated with lower rates of allergic reaction, transfusion-associated lung injury, and viral transmission. SDP has been available in Canada since 2012. Data on SDP use in Canada remains limited. We present a review of subjects receiving SDP at a large tertiary care centre primarily for thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome, demonstrating the tolerability and safety of SDP...
February 15, 2018: Transfusion and Apheresis Science
Robert Alvin Bernedo-Navarro, Ema Romão, Tomomasa Yano, Joar Pinto, Henri De Greve, Yann G-J Sterckx, Serge Muyldermans
BACKGROUND: Shiga toxin-producing Escherichia coli (STEC) are a subset of pathogens leading to illnesses such as diarrhea, hemolytic uremic syndrome and even death. The Shiga toxins are the main virulence factors and divided in two groups: Stx1 and Stx2, of which the latter is more frequently associated with severe pathologies in humans. RESULTS: An immune library of nanobodies (Nbs) was constructed after immunizing an alpaca with recombinant Shiga toxin-2a B subunit (rStx2aB), to retrieve multiple rStx2aB-specific Nbs...
March 1, 2018: Toxins
Colin D Robertson, Tracy H Hazen, James B Kaper, David A Rasko, Anne-Marie Hansen
Enteric pathogens with low infectious doses rely on the ability to orchestrate the expression of virulence and metabolism-associated genes in response to environmental cues for successful infection. Accordingly, the human pathogen enterohemorrhagic Escherichia coli (EHEC) employs a complex multifaceted regulatory network to link the expression of type III secretion system (T3SS) components to nutrient availability. While phosphorylation of histidine and aspartate residues on two-component system response regulators is recognized as an integral part of bacterial signaling, the involvement of phosphotyrosine-mediated control is minimally explored in Gram-negative pathogens...
February 27, 2018: MBio
Bahadur Singh Gurjar, T Manikanta Sriharsha, Angika Bhasym, Savit Prabhu, Mamta Puraswani, Priyanka Khandelwal, Himanshi Saini, Savita Saini, Anita Kamra Verma, Priyadarshini Chatterjee, Prasenjit Gucchait, Vineeta Bal, Anna George, Satyajit Rath, Arvind Sahu, Amita Sharma, Pankaj Hari, Aditi Sinha, Arvind Bagga
We previously reported that Indian pediatric patients of atypical hemolytic-uremic syndrome (aHUS) showed high frequencies of anti-complement factor H (FH) autoantibodies that are correlated with homozygous deletion of the genes for FH-related proteins 1 and 3 (FHR1 and FHR3) (FHR1/3-/-). We now report that Indian pediatric aHUS patients without anti-FH autoantibodies also showed modestly higher frequencies of the FHR1/3-/- genotype. Further, when we characterized epitope specificities and binding avidities of anti-FH autoantibodies in aHUS patients, most anti-FH autoantibodies were directed towards the FH cell-surface anchoring polyanionic binding site-containing C-terminal short conservative regions (SCRs) 17-20 with higher binding avidities than for native FH...
February 27, 2018: Immunology
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