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https://www.readbyqxmd.com/read/29352455/clinical-features-in-a-series-of-258-japanese-pediatric-patients-with-thrombotic-microangiopathy
#1
Akira Ashida, Hideki Matsumura, Toshihiro Sawai, Rika Fujimaru, Yuko Fujii, Akihiko Shirasu, Hyogo Nakakura, Kazumoto Iijima
BACKGROUND: Thrombotic microangiopathy (TMA) includes hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). This study examined the epidemiological characteristics of pediatric patients with TMA classified according to etiology. METHODS: The survey evaluated 258 Japanese pediatric patients diagnosed with TMA between 2012 and 2015. RESULTS: The primary diseases responsible for TMA were categorized as TTP (15 cases), Shiga toxin-producing Escherichia coli-associated HUS (STEC-HUS) (166 cases), atypical HUS (aHUS) (40 cases), and secondary TMA (27 cases)...
January 19, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29344072/bilateral-compressive-optic-neuropathy-from-renal-osteodystrophy-caused-by-branchio-oto-renal-syndrome-stabilised-after-parathyroidectomy
#2
Arun N E Sundaram, Archana Abhayambika, Sunil Kumar
Renal osteodystrophy can cause calvarial hypertrophy and narrowing of the neural canals and foramina. Compressive optic neuropathy is extremely rare in renal osteodystrophy and was reported once only. The authors report bilateral, simultaneous compressive optic neuropathy secondary to renal osteodystrophy with features of uremic leontiasis ossea in chronic renal failure caused by branchio-oto-renal syndrome. Because of the extensive calvarial hypertrophy and the surgical difficulties envisaged with optic canal decompression, conservative approach was pursued...
December 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/29336911/-detection-and-characterization-of-shiga-toxin-producing-escherichia-coli-in-children-treated-at-an-inter-zonal-pediatric-hospital-in-the-city-of-la-plata
#3
Sebastián Oderiz, Gerardo A Leotta, Lucía Galli
Shiga toxin-producing Escherichia coli (STEC) is a foodborne pathogen that can cause watery diarrhea, bloody diarrhea (BD), and hemolytic uremic syndrome (HUS). The objective of this study was to determine the phenotypic and genotypic profiles of STEC strains isolated from children with BD and HUS treated at a pediatric hospital in the city of La Plata in the period 2006-2012, and to establish the clonal relationship of O157:H7 isolates by pulsed field electrophoresis. The percentage of positive samples was 4...
January 11, 2018: Revista Argentina de Microbiología
https://www.readbyqxmd.com/read/29335241/the-mfhr1-fusion-protein-is-a-novel-synthetic-multitarget-complement-inhibitor-with-therapeutic-potential
#4
Stefan Michelfelder, Friedericke Fischer, Astrid Wäldin, Kim V Hörle, Martin Pohl, Juliana Parsons, Ralf Reski, Eva L Decker, Peter F Zipfel, Christine Skerka, Karsten Häffner
The complement system is essential for host defense, but uncontrolled complement system activation leads to severe, mostly renal pathologies, such as atypical hemolytic uremic syndrome or C3 glomerulopathy. Here, we investigated a novel combinational approach to modulate complement activation by targeting C3 and the terminal pathway simultaneously. The synthetic fusion protein MFHR1 links the regulatory domains of complement factor H (FH) with the C5 convertase/C5b-9 inhibitory fragment of the FH-related protein 1...
January 15, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29331477/atypical-presentation-of-pregnancy-related-hemolytic-uremic-syndrome
#5
Salim Baghli, Catherine Abendroth, Umar Farooq, Jennifer A Schaub
The cause of acute kidney injury during pregnancy and in the postpartum period can be particularly challenging to diagnose, especially when it is necessary to differentiate among preeclampsia; eclampsia; hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome; and thrombotic microangiopathies (TMAs). All these disease entities can present with kidney failure, microangiopathic hemolytic anemia, and thrombocytopenia. We present a teaching case of atypical hemolytic uremic syndrome in the postpartum period in a young woman who was found to have mutations of uncertain clinical significance in the complement cascade, including in C3, CFH, and CFI...
January 10, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29328045/assessment-of-the-level-of-knowledge-of-medical-personnel-in-diarrhea-and-hemolytic-uremic-syndrome
#6
O Chokoshvili, N Vepkhvadze, T Tsertsvadze, T Gabrichidze, M Khorbaladze
Survey have been conducted among medical professionals to test knowledge level of HUS and diarrheal diseases and to identify predictor variables for better knowledge. Cross-sectional survey have been conducted among medical personnel at different clinics in Tbilisi and in regions of Georgia. Participants were selected from different clinics in Tbilisi and in three biggest regional cities (Zugdidi, Batumi and Kutaisi) of Georgia. A total of 12 clinics were selected from them 6 were in Tbilisi and 2 at each regional cities...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#7
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29326853/purtscher-like-retinopathy-associated-with-atypical-hemolytic-uremic-syndrome
#8
Melih Ustaoğlu, Feyza Önder, Nilgün Solmaz, Savaş Öztürk, Mesut Ayer
A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure)...
December 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/29322327/combined-and-sequential-liver-kidney-transplantation-in-children
#9
Ryszard Grenda, Piotr Kaliciński
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms...
January 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29316724/biochemical-and-clinical-impact-of-organic-uremic-retention-solutes-a-comprehensive-update
#10
REVIEW
Raymond Vanholder, Anneleen Pletinck, Eva Schepers, Griet Glorieux
In this narrative review, the biological/biochemical impact (toxicity) of a large array of known individual uremic retention solutes and groups of solutes is summarized. We classified these compounds along their physico-chemical characteristics as small water-soluble compounds or groups, protein bound compounds and middle molecules. All but one solute (glomerulopressin) affected at least one mechanism with the potential to contribute to the uremic syndrome. In general, several mechanisms were influenced for each individual solute or group of solutes, with some impacting up to 7 different biological systems of the 11 considered...
January 8, 2018: Toxins
https://www.readbyqxmd.com/read/29301877/draft-genome-sequences-of-enterohemorrhagic-and-enteropathogenic-escherichia-coli-strains-isolated-from-alpacas-in-peru
#11
Lenin Maturrano, Marjorie Aleman, Dennis Carhuaricra, Jorge Maximiliano, Juan Siuce, Luis Luna, Raul Rosadio
The draft genome sequences of two strains of Escherichia coli, isolated from alpacas in Peru, are reported here. ECA1 has been determined to be a strain of enterohemorrhagic E. coli and ECB1 a strain of enteropathogenic E. coli These pathogens are responsible for hemolytic-uremic syndrome in humans and diarrhea in different mammals, respectively.
January 4, 2018: Genome Announcements
https://www.readbyqxmd.com/read/29288280/is-eculizumab-efficacious-in-shigatoxin-associated-hemolytic-uremic-syndrome-a-narrative-review-of-current-evidence
#12
REVIEW
Werner Keenswijk, Ann Raes, Johan Vande Walle
Severe complications due to Shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) currently present a serious challenge since no specific treatment for this condition is available. Eculizumab, a terminal complement inhibitor, has been used especially in STEC-HUS patients with severe neurological involvement, but the efficacy remains undetermined. In order to determine its efficacy, we searched the databases Pubmed, Web of Science, Embase, and LiLACS for reports describing outcomes of eculizumab administration in STEC-HUS...
December 29, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29282226/maternal-and-fetal-outcomes-of-pregnancies-in-women-with-atypical-hemolytic-uremic-syndrome
#13
Martina Gaggl, Christof Aigner, Dorottya Csuka, Ágnes Szilágyi, Zoltán Prohászka, Renate Kain, Natalja Haninger, Maarten Knechtelsdorfer, Raute Sunder-Plassmann, Gere Sunder-Plassmann, Alice Schmidt
Atypical HUS (aHUS) is a disorder most commonly caused by inherited defects of the alternative pathway of complement, or the proteins that regulate this pathway, and life-threatening episodes of aHUS can be provoked by pregnancy. We retrospectively and prospectively investigated 27 maternal and fetal pregnancy outcomes in 14 women with aHUS from the Vienna Thrombotic Microangiopathy Cohort. Seven pregnancies (26%) were complicated by pregnancy-associated aHUS (p-aHUS), of which three appeared to be provoked by infection, bleeding, and curettage, and three individuals were considered to have preeclampsia/HELLP syndrome before the definitive diagnosis of p-aHUS was made...
December 27, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29281993/vitamin-e-protects-against-extraskeletal-calcification-in-uremic-rats-fed-high-fat-diets
#14
Rafael Rios, Ana I Raya, Carmen Pineda, Mariano Rodriguez, Ignacio Lopez, Escolastico Aguilera-Tejero
BACKGROUND: High fat diets are implicated in the pathogenesis of metabolic syndrome, obesity and renal disease. Previous studies have revealed that high fat diets promote vascular calcification in uremic rats. Moreover, vitamin E has been shown to prevent uremic calcifications in genetically obese Zucker rats fed standard diet. The objective of this study was to investigate the influence of vitamin E supplementation on the development of extraskeletal calcifications in non-obese (wild type) uremic rats fed high fat diets...
December 28, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29280264/anti-shigatoxin-2-antibodies-in-ehec-o104-h4-infected-patients-may-predict-hemolytic-uremic-syndrome
#15
Werner Dammermann, Valentin Mihajlov, Barbara Middendorf, Alexander Mellmann, Helge Karch, Stefan Lüth, Sebastian Ullrich
BACKGROUND AND AIM: An outbreak of Shigatoxin 2 (Stx2) producing enterohemorrhagic and enteroaggregative E.coli (EHEC) O104:H4 infection in May 2011 in Germany caused enterocolitis and an unprecedented high 22% rate of hemolytic uremic syndrome (HUS). We hypothesized that anti-Stx2 IgM or IgG titers might predict HUS development. METHODS: 32 patients infected with EHEC O104:H4 (HUS: n=23;non-HUS: n=9) were retrospectively screened for anti-Stx2 IgM/IgG and matched with clinical data regarding HUS development, fever, superinfection, dialysis, neurological symptoms, intensive care, antibiotic treatment, and plasmapheresis...
December 27, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29279276/acquired-partial-lipodystrophy-and-c3-glomerulopathy-dysregulation-of-the-complement-system-as-a-common-pathogenic-mechanism
#16
Fernando Corvillo, Margarita López-Trascasa
The activation of the alternative pathway of the complement is involved in the development of several renal diseases, such as atypical haemolytic uremic syndrome and C3 glomerulopathy. In C3 glomerulopathy, a high percentage of patients have circulating levels of the autoantibody called C3NeF, which causes systemic dysregulation of the complement system. In some cases, the presence of this antibody has been related with abnormalities of adipose tissue, causing acquired partial lipodystrophy (Barraquer-Simons syndrome)...
December 23, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29273508/prevalence-quantification-and-isolation-of-pathogenic-shiga-toxin-escherichia-coli-o157-h7-along-the-production-and-supply-chain-of-pork-around-hubei-province-of-china
#17
Sher Bahadar Khan, Geng Zou, Ran Xiao, Yuting Cheng, Zia Ur Rehman, Sher Ali, Atta Muhammad Memon, Shah Fahad, Irshad Ahmad, Rui Zhou
Shiga toxin Escherichia coli (STEC) O157:H7 is an important zoonotic food borne pathogen causing gastroenteritis that may lead to life threatening hemorragic colitis (HC) and hemorrhagic uremic syndrome (HUS). 325 meat and tissue samples were tested for enumeration of O157:H7 strains using most probable number (MPN)-PCR targeting their specific genes flicH7 and rfbO157 followed by isolation, sereotyping and pathogenicity testing. The overall prevalence of O157:H7 was 41.3% (134/325) along the production and supply chain of pork (PSCP), being higher in supply chain (59%, 118/200) as compared to pig farms (12...
December 19, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/29260749/-difficulties-in-the-diagnosis-of-diarrhea-associated-hemolytic-uremic-syndrome-in-adults
#18
V A Malov, V V Maleev, N L Kozlovskaya, N A Tsvetkova, S V Smetanina, A N Gorobchenko, V V Serova, V B Chentsov, A G Volkov, A P Faller
Hemolytic uremic syndrome (HUS) is a rare, but menacing condition registered mainly in children. The paper gives a detailed description and analysis of a clinical case of HUS with a favorable outcome in an adult woman who developed the syndrome in the presence of bloody diarrhea. It considers an update on the etiology, pathogenesis, and clinical features of HUS associated with diarrheal syndrome and discusses differential diagnostic features, diagnostic problems, and characteristics of management tactics for patients...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/29250893/atypical-hemolytic-uremic-syndrome-a-meta-analysis-of-case-reports-confirms-the-prevalence-of-genetic-mutations-and-the-shift-of-treatment-regimens
#19
Vinod Krishnappa, Mohit Gupta, Mohamed Elrifai, Bahar Moftakhar, Michael J Ensley, Tushar J Vachharajani, Sidharth Kumar Sethi, Rupesh Raina
Atypical hemolytic uremic syndrome (aHUS) is a rare life-threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta-analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations...
December 17, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/29248444/excitotoxicity-in-encephalopathy-associated-with-stec-o-157-infection
#20
Shigenobu Ishida, Kumi Yasukawa, Mai Koizumi, Katsuhiro Abe, Nozomi Hirai, Takafumi Honda, Hiroshi Sakuma, Hiroko Tada, Jun-Ichi Takanashi
Cytokines play an important role in the pathogenesis of the severe complications of Shiga toxin-producing Escherichia coli (STEC) infection, such as hemolytic uremic syndrome (HUS) and acute encephalopathy. A 3-year-old boy with acute encephalopathy associated with STEC O-157 HUS showed increased levels of IL-6 and IL-10, which normalized after methylprednisolone pulse therapy, and additionally exhibited a transient increase of glutamine on MR spectroscopy. This finding suggests that excitotoxicity, in addition to hypercytokinemia, may play an important role in the pathogenesis of HUS encephalopathy...
December 13, 2017: Brain & Development
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