8,025 results match your criteria Hemolytic-Uremic Syndrome


Correction to: Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use.

Pediatr Nephrol 2019 Jan 15. Epub 2019 Jan 15.

Radboud Institute for Molecular Life Sciences, Amalia Children's Hospital, Department of Pediatric Nephrology, Radboud University Medical Center, P.O. Box 9101, 6500, HB, Nijmegen, The Netherlands.

The original version of this article unfortunately contained two mistakes. The presentation of Table 1 and Fig. 1 was incorrect. Read More

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http://link.springer.com/10.1007/s00467-018-4186-x
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http://dx.doi.org/10.1007/s00467-018-4186-xDOI Listing
January 2019
1 Read

Complement Activation Contributes to the Pathophysiology of Shiga Toxin-Associated Hemolytic Uremic Syndrome.

Microorganisms 2019 Jan 10;7(1). Epub 2019 Jan 10.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, 24126 Bergamo, Italy.

Shiga toxin (Stx)-producing (STEC) infections have become a threat to public health globally because of the severe illnesses that they can trigger, such as hemorrhagic colitis and the post-diarrheal hemolytic uremic syndrome (HUS), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Glomerular endothelial cells are primary targets of Stx which, after binding to its specific receptor globotriaosylceramide, upregulates proinflammatory proteins involved both in the recruitment and adhesion of leukocytes and thrombus formation at the site of endothelial injury. In this review, we discuss the role of complement activation in promoting glomerular microvascular dysfunction, providing evidence from experimental models and patients with STEC-HUS. Read More

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http://dx.doi.org/10.3390/microorganisms7010015DOI Listing
January 2019

Lactobacillus casei suppresses hfq gene expression in Escherichia coli O157:H7.

Br J Biomed Sci 2019 Jan 11. Epub 2019 Jan 11.

b Immunology Research Center , Tabriz University of Medical Sciences , Tabriz , Iran.

Introduction Escherichia coli O157:H7 infection causes dysentery and hemolytic-uremic syndrome (HUS). It is often contracted by intake of contaminated water or food. This bacterium encodes the sRNA chaperone hfq, which is important in growth and virulence. Read More

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January 2019

Purtscher-Like Retinopathy in Hemolytic Uremic Syndrome.

JAMA Ophthalmol 2019 Jan 10;137(1):e183911. Epub 2019 Jan 10.

Department of Ophthalmology, Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

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http://dx.doi.org/10.1001/jamaophthalmol.2018.3911DOI Listing
January 2019

Heme Drives Susceptibility of Glomerular Endothelium to Complement Overactivation Due to Inefficient Upregulation of Heme Oxygenase-1.

Front Immunol 2018 20;9:3008. Epub 2018 Dec 20.

INSERM, UMR_S 1138, Centre de Recherche des Cordeliers, Paris, France.

Atypical hemolytic uremic syndrome (aHUS) is a severe disease characterized by microvascular endothelial cell (EC) lesions leading to thrombi formation, mechanical hemolysis and organ failure, predominantly renal. Complement system overactivation is a hallmark of aHUS. To investigate this selective susceptibility of the microvascular renal endothelium to complement attack and thrombotic microangiopathic lesions, we compared complement and cyto-protection markers on EC, from different vascular beds, in and models as well as in patients. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.03008
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http://dx.doi.org/10.3389/fimmu.2018.03008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306430PMC
December 2018
2 Reads

Relevance of Bacteriophage 933W in the Development of Hemolytic Uremic Syndrome (HUS).

Front Microbiol 2018 13;9:3104. Epub 2018 Dec 13.

Laboratorio de Ingeniería Genética y Biología Celular y Molecular, Universidad Nacional de Quilmes, Buenos Aires, Argentina.

Hemolytic uremic syndrome (HUS), principally caused by shiga toxins (Stxs), is associated with Shiga toxin-producing (STEC) infections. We previously reported Stx2 expression by host cells and . As the genes encoding the two Stx subunits are located in bacteriophage genomes, the aim of the current study was to evaluate the role of bacteriophage induction in HUS development in absence of an O157:H7 genomic background. Read More

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http://dx.doi.org/10.3389/fmicb.2018.03104DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300567PMC
December 2018

Targeting properdin in the treatment of atypical haemolytic uraemic syndrome: better than eculizumab?

Ann Transl Med 2018 Nov;6(Suppl 1):S62

NRCTC and Newcastle University, The medical school, Farmington place, Newcastle-upon-Tyne, UK.

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http://dx.doi.org/10.21037/atm.2018.10.35DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291531PMC
November 2018

Giant Negative T Waves and QT Prolongation in Non-cardiogenic Pulmonary Edema: A Case Report and Review of Literature.

Cureus 2018 Oct 8;10(10):e3423. Epub 2018 Oct 8.

Department of Internal Medicine, University of Missouri-Columbia, Columbia, USA.

Giant negative T-waves have been linked to several cardiac and non-cardiac conditions. However, the presence of giant negative T-waves with QT prolongation in the setting of non-cardiogenic pulmonary edema is a rarely reported, female predominant, and poorly understood electrocardiographic phenomenon. We report a case of a 28-year-old white female who presented with acute diarrhea and was admitted due to acute kidney injury caused by a hemolytic uremic syndrome (HUS). Read More

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http://dx.doi.org/10.7759/cureus.3423DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314535PMC
October 2018

A Case of Suspected Streptococcus Pneumoniae Hemolytic Uremic Syndrome (pHUS) with Utilization of Minor Crossmatching for Platelet Blood Products Lead to a Diagnosis of Atypical Hemolytic Uremic Syndrome (aHUS).

Ann Clin Lab Sci 2018 Nov;48(6):797-800

Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center McGovern Medical School, Houston, TX, USA.

Background: The action of bacterial neuraminidase of (SPN) results in exposure of the normally "hidden" Thomsen-Freidenreich antigen (T-antigen) found on erythrocytes and other tissues. This may lead to SPN-induced hemolytic uremic syndrome (pHUS) with subsequent hemolysis and end organ damage. pHUS can be identified by minor crossmatch incompatibility. Read More

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November 2018
5 Reads

Quantification of enterohemorrhagic Escherichia coli O157:H7 protein abundance by high-throughput proteome.

PLoS One 2018 31;13(12):e0208520. Epub 2018 Dec 31.

Institute of Biotechnology, CICVyA, National Institute of Agricultural Technology, Hurlingham, Buenos Aires, Argentina.

Enterohemorrhagic Escherichia coli (EHEC) O157:H7 is a human pathogen responsible for diarrhea, hemorrhagic colitis and hemolytic uremic syndrome (HUS). To promote a comprehensive insight into the molecular basis of EHEC O157:H7 physiology and pathogenesis, the combined proteome of EHEC O157:H7 strains, Clade 8 and Clade 6 isolated from cattle in Argentina, and the standard EDL933 (clade 3) strain has been analyzed. From shotgun proteomic analysis a total of 2,644 non-redundant proteins of EHEC O157:H7 were identified, which correspond approximately 47% of the predicted proteome of this pathogen. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0208520PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312284PMC
December 2018
3.234 Impact Factor

C3 Glomerulonephritis With Multiple Mutations in Complement Factor H.

Iran J Kidney Dis 2018 Nov;12(6):376-381

Chronic Kidney Disease Research Center, Department of Nephrology, Labbafinejad Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Complement C3 glomerulopathy refers to a disease process in which abnormal control of complement activation or degradation results in predominant C3 fragment deposition within the glomerulus and causes glomerular damage. Abnormal control of the complement alternative pathway is a well-established risk factor for the occurrence of C3 glomerulonephritis. It is the first reported case in Iran with multiple mutations in complement factor H, with one of these mutations we have expected in hemolytic uremic syndrome rather than C3 glomerulopathy Genetic analysis showed that the molecular abnormalities of factor H led to complement factor H malfunction that were polymorphous and not restricted to the C-terminal domains of the protein. Read More

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November 2018
1 Read
0.979 Impact Factor

Biochemical characterization of in-vitro reconstituted biologically active recombinant Shiga toxin.

Protein Pept Lett 2018 Dec 28. Epub 2018 Dec 28.

Biotechnology Division, Defence Research & Development Establishment, Jhansi Road, Gwalior-474002, MP. India.

The Shiga toxins comprise a family of related protein toxins produced by bacteria Shigella dysenteriae and some strains of Escherichia coli that cause severe clinical manifestations. Severe Shiga toxin intoxication results in hemolytic-uremic syndrome (HUS), up to 50% of HUS patients manifest some degree of renal failure and ∼10% of such cases develop permanent renal failure or death. Purification of Shiga toxin from bacteria is a multistep time consuming process resulting in low yield. Read More

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http://dx.doi.org/10.2174/0929866526666181228161834DOI Listing
December 2018
2 Reads

Norovirus-associated hemolytic uremic syndrome in a renal transplant recipient.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1519-1522

Department of Nephrology, Sir Gangaram Hospital, New Delhi, India.

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http://dx.doi.org/10.4103/1319-2442.248289DOI Listing
December 2018

A single-center 4-year experience with 47 pediatric renal transplants: Evolving trends.

Saudi J Kidney Dis Transpl 2018 Nov-Dec;29(6):1303-1310

Department of Surgery, Section of Transplantation, Renal Transplant Unit, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.

Outcome of pediatric kidney transplantation (KT) has improved over the last several decades. We retrospectively reviewed the outcomes pediatric KT in King Faisal Specialist Hospital and Research Center-Jeddah, Saudi Arabia. Between May 2013 and November 2016, we performed renal transplantation in 47 children, 30 (64%) males, and 17 (36%) females. Read More

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http://www.sjkdt.org/text.asp?2018/29/6/1303/248297
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http://dx.doi.org/10.4103/1319-2442.248297DOI Listing
December 2018
5 Reads

Hazard Identification and Characterization: Criteria for Categorizing Shiga Toxin-Producing Escherichia coli on a Risk Basis.

Authors:

J Food Prot 2019 Jan;82(1):7-21

The Joint FAO/WHO Expert Meetings on Microbiological Risk Assessment (JEMRA) Secretariat, * Food Safety and Quality Unit, Agriculture and Consumer Protection Department, Food and Agriculture Organization of the United Nations, Viale delle Terme di Caracalla, 00153 Rome, Italy.

Shiga toxin-producing Escherichia coli (STEC) comprise a large, highly diverse group of strains. Since the emergence of STEC serotype O157:H7 as an important foodborne pathogen, serotype data have been used for identifying STEC strains, and this use continued as other serotypes were implicated in human infections. An estimated 470 STEC serotypes have been identified, which can produce one or more of the 12 known Shiga toxin (Stx) subtypes. Read More

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http://dx.doi.org/10.4315/0362-028X.JFP-18-291DOI Listing
January 2019
2 Reads

Improving hazard characterization in microbial risk assessment using next generation sequencing data and machine learning: Predicting clinical outcomes in shigatoxigenic Escherichia coli.

Int J Food Microbiol 2018 Dec 4;292:72-82. Epub 2018 Dec 4.

Research Group for Genomic Epidemiology, National Food Institute, Technical University of Denmark, Kemitorvet, Building 204, 2800 Kgs. Lyngby, Denmark.

The ever decreasing cost and increase in throughput of next generation sequencing (NGS) techniques have resulted in a rapid increase in availability of NGS data. Such data have the potential for rapid, reproducible and highly discriminative characterization of pathogens. This provides an opportunity in microbial risk assessment to account for variations in survivability and virulence among strains. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01681605183089
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http://dx.doi.org/10.1016/j.ijfoodmicro.2018.11.016DOI Listing
December 2018
4 Reads

The structure of the Shiga toxin 2a A-subunit dictates the interactions of the toxin with blood components.

Cell Microbiol 2018 Dec 22:e13000. Epub 2018 Dec 22.

Division of Hygiene and Medical Microbiology, Medical University of Innsbruck, Innsbruck, Austria.

Hemolytic uremic syndrome (eHUS) is a severe complication of human infections with Shiga toxins (Stxs)-producing Escherichia coli. A key step in the pathogenesis of eHUS is the interaction of Stxs with blood components before the targeting of renal endothelial cells. Here we show that a single proteolytic cleavage in the Stx2a A-subunit, resulting into two fragments (A1 and A2) linked by a disulfide bridge (cleaved Stx2a), dictates different binding abilities. Read More

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http://doi.wiley.com/10.1111/cmi.13000
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http://dx.doi.org/10.1111/cmi.13000DOI Listing
December 2018
5 Reads

Therapeutic Plasma Exchange in Pediatric Renal Transplantation Experience of One Decade and 389 Sessions.

Transplant Proc 2018 Dec 10;50(10):3483-3486. Epub 2018 Jul 10.

The Children's Memorial Health Institute, Warsaw, Poland. Electronic address:

Objective: There are no specific recommendations for therapeutic plasma exchange (TPE) in children after renal transplantation. The purpose of this study was to report the experience with TPE in a pediatric transplant setting.

Materials And Methods: 59 patients (mean age 12. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00411345183094
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http://dx.doi.org/10.1016/j.transproceed.2018.07.015DOI Listing
December 2018
6 Reads

Corrigendum: Insights into pregnancy associated and atypical hemolytic uremic syndrome.

Authors:

Obstet Med 2018 Dec 13;11(4):198. Epub 2018 Dec 13.

[This corrects the article DOI: 10.1177/1753495X18780099.]. Read More

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http://dx.doi.org/10.1177/1753495X18815566DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295768PMC
December 2018

Progressive Retinal Findings in Hemolytic Uremic Syndrome.

J Pediatr Ophthalmol Strabismus 2018 Dec 19;55:e49-e51. Epub 2018 Dec 19.

Ocular involvement in hemolytic uremic syndrome is rare and in most cases presents with retinal ischemia, hemorrhages, and neovascularization. The authors describe the progression of retinal involvement as a rare complication of typical hemolytic uremic syndrome in a 2-year-old boy. Progression of retinal findings were demonstrated with serial fundus photographs. Read More

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http://dx.doi.org/10.3928/01913913-20181017-03DOI Listing
December 2018
1 Read

The Relationship Between Census Tract Poverty and Shiga Toxin-Producing Risk, Analysis of FoodNet Data, 2010-2014.

Open Forum Infect Dis 2018 Jul 3;5(7):ofy148. Epub 2018 Jul 3.

Emerging Infections Program, Yale School of Public Health, New Haven, Connecticut.

Background: The relationship between socioeconomic status and Shiga toxin-producing (STEC) is not well understood. However, recent studies in Connecticut and New York City found that as census tract poverty (CTP) decreased, rates of STEC increased. To explore this nationally, we analyzed surveillance data from laboratory-confirmed cases of STEC from 2010-2014 for all Foodborne Disease Active Surveillance Network (FoodNet) sites, population 47. Read More

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http://dx.doi.org/10.1093/ofid/ofy148DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6041751PMC
July 2018
1 Read

CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease.

Authors:
Ahmet Ozen

Immunol Rev 2019 Jan;287(1):20-32

Division of Allergy and Immunology, Marmara University School of Medicine, Istanbul, Turkey.

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease was described in 1961 as an important cause of protein-losing enteropathy (PLE). PIL can be the sole finding in rare individuals or occur as part of a multisystemic genetic syndrome. Although genetic etiologies of many lymphatic dysplasia syndromes associated with PIL have been identified, the pathogenesis of isolated PIL (with no associated syndromic features) remains unknown. Read More

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http://dx.doi.org/10.1111/imr.12715DOI Listing
January 2019

Hemolytic uremic syndrome: differential diagnosis with the onset of inflammatory bowel diseases.

Acta Biomed 2018 Dec 17;89(9-S):153-157. Epub 2018 Dec 17.

Pediatric Emergency Unit, University Hospital of Parma, Maternal and Infant Department, Parma, Italy.

Background: Shiga-toxin Escherichia coli productor (STEC) provokes frequently an important intestinal damage that may be considered in differential diagnosis with the onset of Inflammatory Bowel Disease (IBD). The aim of this work is to review in the current literature about Hemolytic Uremic Syndrome (HUS) and IBD symptoms at the onset, comparing the clinical presentation and symptoms, as the timing of diagnosis and of the correct treatment of both these conditions is a fundamental prognostic factor. A focus is made about the association between typical or atypical HUS and IBD and a possible renal involvement in patient with IBD (IgA-nephropathy). Read More

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http://dx.doi.org/10.23750/abm.v89i9-S.7911DOI Listing
December 2018
10 Reads

Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H.

Pediatr Nephrol 2018 Dec 17. Epub 2018 Dec 17.

Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France.

Background: Hemolytic uremic syndrome (HUS) has been associated with a number of infectious agents. We report here the case of an infant with severe Bordetella pertussis infection who developed HUS.

Case Diagnosis/treatment: A 2-month-old preterm male was admitted for severe Bordetella pertussis infection. Read More

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http://dx.doi.org/10.1007/s00467-018-4174-1DOI Listing
December 2018
1 Read

Hemolytic Uremic Syndrome Associated With O157 Infection in an Allogenic Stem Cell Transplant Recipient.

Mayo Clin Proc Innov Qual Outcomes 2018 Dec 15;2(4):387-391. Epub 2018 Sep 15.

Department of Immunology, Mayo Clinic, Rochester, MN.

We report the development of a Shiga toxin-producing O157 gastrointestinal infection associated with hemolytic uremic syndrome in an allogenic stem cell transplant recipient with a history of gastrointestinal graft-vs-host disease receiving long-term immunosuppression. Read More

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http://dx.doi.org/10.1016/j.mayocpiqo.2018.07.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260488PMC
December 2018
1 Read

Treatment of hemolytic uremic syndrome related to Bordetella pertussis infection -is plasma exchange or eculizumab use necessary?

BMC Nephrol 2018 Dec 17;19(1):365. Epub 2018 Dec 17.

Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Bordetella pertussis infection is a known trigger of atypical hemolytic uremic syndrome (HUS). For patients suspected of having atypical HUS, prompt plasma exchange/infusion (PE/PI) or eculizumab (ECZ) treatment is recommended.

Case Presentation: We report a 1-month-old female infant who was admitted with a severe cough and a B. Read More

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http://dx.doi.org/10.1186/s12882-018-1168-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297948PMC
December 2018
1 Read

Risk Factors for Short- and Long-Term Outcomes in Children With STEC-HUS/D HUS: A Single-Center Experience.

Glob Pediatr Health 2018 4;5:2333794X18816920. Epub 2018 Dec 4.

The Children's Mercy Hospitals and Clinics, Kansas City, MO, USA.

. Hemolytic uremic syndrome (HUS) is one of the common causes for acute kidney injury in childhood. . Read More

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http://dx.doi.org/10.1177/2333794X18816920DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287298PMC
December 2018

A rare case of Alport syndrome, atypical hemolytic uremic syndrome and Pauci-immune crescentic glomerulonephritis.

BMC Nephrol 2018 Dec 12;19(1):355. Epub 2018 Dec 12.

Department of Pathology, Stanford University, Stanford, CA, USA.

Background: Renal thrombotic microangiopathy (TMA) is occasionally seen in biopsies with pauci-immune necrotizing crescentic glomerulonephritis (PCGN). Recent study indicated that the complement activation is more prominent in the ANCA-negative glomerulonephritis.

Case Presentation: We report a case of concurrent TMA and PCGN without ANCA positivity. Read More

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http://dx.doi.org/10.1186/s12882-018-1170-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6291978PMC
December 2018

Deficiency of complement factor H-related proteins and autoantibody-positive hemolytic uremic syndrome in an infant with combined partial deficiencies and autoantibodies to complement factor H and ADAMTS13.

Clin Kidney J 2018 Dec 7;11(6):791-796. Epub 2018 Mar 7.

Baylor College of Medicine, Houston, TX, USA.

A 3-month-old male infant developed an extremely severe episode of atypical hemolytic uremic syndrome (aHUS) associated with partial deficiencies of full-length complement factor H (FH; ∼15% of infant normal) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) (39% of normal) and autoantibodies reactive with both proteins. His FH and ADAMTS13 genes were normal, indicating that the partial deficiencies were acquired, probably as the result of autoantibodies against full-length FH and ADAMTS13. The child also had a homozygous deletion of the complement factor H-related (CFHR)3-CFHR1 portion in the complement factor H () gene cluster. Read More

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http://dx.doi.org/10.1093/ckj/sfy010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275444PMC
December 2018
1 Read

Detection of Autoantibodies to Complement Components by Surface Plasmon Resonance-Based Technology.

Methods Mol Biol 2019 ;1901:271-280

INSERM, UMR_S 1138, Centre de Recherche des Cordeliers, Paris, France.

The innate immune complement system is a powerful defense cascade against pathogens, but can induce host tissue damage when overactivated. In pathological conditions, mainly but not restricted to renal diseases, such as lupus nephritis, atypical hemolytic uremic syndrome, and C3 glomerulopathies, complement is overactivated or dysregulated by autoantibodies directed against its components and regulators. Among the key autoantibody targets are the initiator of the classical complement pathway C1q, the alternative pathway regulator Factor H, the components of the alternative pathway C3 convertase complex C3 and Factor B and the convertase complex itself. Read More

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http://dx.doi.org/10.1007/978-1-4939-8949-2_24DOI Listing
January 2019

Anti-factor H Autoantibodies Assay by ELISA.

Methods Mol Biol 2019 ;1901:191-196

Laboratoire d'Immunologie, Hôpital Européen Georges Pompidou, Paris, France.

The complement system is a part of the immune system implicated in host defense against pathogens and damaged cells and Factor H is the main regulatory protein of this powerful enzymatic cascade. Autoantibodies directed against Factor H (anti-FH antibodies) are implicated in different pathologies mainly atypical hemolytic and uremic syndrome and C3 glomerulopathies. The detection and quantification of these autoantibodies are crucial for the clinical management of the patients. Read More

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http://link.springer.com/10.1007/978-1-4939-8949-2_15
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http://dx.doi.org/10.1007/978-1-4939-8949-2_15DOI Listing
January 2019
6 Reads

Complement-driven anemia: more than just paroxysmal nocturnal hemoglobinuria.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):371-376

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD.

Atypical hemolytic uremic syndrome (aHUS); hemolysis, elevated liver function tests, and low platelets syndrome; and transplant-associated thrombotic microangiopathy are related conditions, in that many patients harbor germline heterozygous mutations in genes that regulate the alternative pathway of complement (APC). Penetrance is variable because development of clinically significant disease appears to require supervention of a process such as inflammation. Complement activation on the endothelial surfaces leads to endothelial damage, platelet consumption, microthrombi, and a mechanical hemolytic anemia with schistocytes. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.371DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245985PMC
November 2018
1 Read

CHARACTERISTICS OF DIARRHEAL DISEASE COMPLICATED WITH HEMOLYTIC UREMIC SYNDROME AMONG CHILDREN IN GEORGIA, 2009-2016.

Georgian Med News 2018 Oct(283):123-129

Infectious Diseases, AIDS and Clinical Immunology Research Center; Tbilisi State Medical University; M. Iashvili Children's Central Hospital; 4National Center for Disease Control and Public Health, Tbilisi, Georgia.

Shiga toxin-producing Escherichia coli (STEC) causes illness ranging from mild diarrhea to bloody diarrhea, to the hemolytic uremic syndrome (HUS), which manifests with a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Surveillance of HUS and bloody diarrhea is not performed in Georgia. The primary objective of our study was to determine the annual incidence of diarrheal diseases. Read More

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October 2018
2 Reads

Human Recombinant Fab Fragment Neutralizes Shiga Toxin Type 2 Cytotoxic Effects and .

Toxins (Basel) 2018 Dec 2;10(12). Epub 2018 Dec 2.

Laboratório de Bacteriologia, Instituto Butantan, São Paulo 05503900, Brasil.

Shiga toxin (Stx) producing (STEC) is responsible for causing hemolytic uremic syndrome (HUS), a life-threatening thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and acute renal failure after bacterially induced hemorrhagic diarrhea. Until now, there has been neither an effective treatment nor method of prevention for the deleterious effects caused by Stx intoxication. Antibodies are well recognized as affinity components of therapeutic drugs; thus, a previously obtained recombinant human FabC11:Stx2 fragment was used to neutralize Stx2 in vitro in a Vero cell viability assay. Read More

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http://dx.doi.org/10.3390/toxins10120508DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6315604PMC
December 2018
2 Reads

Atypical Hemolytic Uremic Syndrome Presenting as Pre-eclampsia in a 24-year-old Woman with Chronic Kidney Disease: Pathogenesis and Genetics.

Cureus 2018 Sep 25;10(9):e3358. Epub 2018 Sep 25.

Internal Medicine, Brookdale University Hospital, New York, USA.

Atypical hemolytic uremic syndrome (aHUS) is a kidney disorder that is frequently unrecognized during its progression, and misdiagnosed with more common etiologies of microangiopathic hemolytic anemia (MAHA): hemolytic uremic syndrome, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura (TTP). During pregnancy, the diagnosis of aHUS is furthermore challenging. The clinical presentation of aHUS may mimic pre-eclampsia as it occurred to the patient described in the case report. Read More

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http://dx.doi.org/10.7759/cureus.3358DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6257492PMC
September 2018
3 Reads

[Ocular thrombotic microangiopathy in atypical hemolytic-uremic syndrome (a clinical case study)].

Vestn Oftalmol 2018 ;134(5. Vyp. 2):215-226

I.M. Sechenov First Moscow State Medical University, 8-2 Trubetskaya St., Moscow, Russian Federation, 119991.

The article presents a clinical observation of ocular thrombotic microangiopathy in atypical hemolytic-uremic syndrome - a rare genetic disease characterized by systemic thrombosis caused by uncontrolled activation of alternative complement pathway. A typical manifestation of this ocular lesion in this disease is bilateral Purtscher-like retinopathy. Timely diagnostics of atypical hemolytic-uremic syndrome, including ophthalmologic examination, determines the early start of a highly effective pathogenetic therapy with complement inhibitor eculizumab. Read More

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http://dx.doi.org/10.17116/oftalma2018134051215DOI Listing
January 2018
5 Reads

Interleukin-33/ST2 signaling contributes to the severity of hemolytic uremic syndrome induced by enterohemorrhagic Escherichia coli.

Clin Exp Nephrol 2018 Nov 22. Epub 2018 Nov 22.

Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takara-machi, Kanazawa, 920-8641, Japan.

Objectives: Interleukin (IL)-33 plays an important role in host defense, immune regulation, and inflammation. This study assessed IL-33's role in the pathogenesis of severe hemolytic uremic syndrome (HUS) induced by enterohemorrhagic Escherichia coli (EHEC). We also investigated the clinical significance of IL-33 and soluble ST2 (soluble form of IL-33 receptor) serum levels in patients with EHEC-induced HUS. Read More

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http://dx.doi.org/10.1007/s10157-018-1675-yDOI Listing
November 2018
7 Reads

Case definitions of hemolytic uremic syndrome following Escherichia coli O157:H7 infection vary in validity.

Int J Med Microbiol 2018 Dec 10;308(8):1121-1127. Epub 2018 Oct 10.

Department of Pediatrics, Washington University in St. Louis School of Medicine, One Children's Place, St. Louis, MO, 63110, USA. Electronic address:

Background: Multiple case definitions for post-diarrheal hemolytic uremic syndrome (D+ HUS) associated with Shiga toxin-producing Escherichia coli (STEC) are used across public health, research, and clinical practice.

Methods: To identify a single definition of D+ HUS for standardized use, we evaluated the comparability and validity of four common, heterogeneous definitions: the Council of State and Territorial Epidemiologists (CSTE) definition, hematology-focused and age-focused definitions from the literature, and hospital diagnosis. We reviewed medical records from 471 hospitalized E. Read More

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http://dx.doi.org/10.1016/j.ijmm.2018.10.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260930PMC
December 2018
9 Reads

Emerging Multidrug-Resistant Hybrid Pathotype Shiga Toxin-Producing Escherichia coli O80 and Related Strains of Clonal Complex 165, Europe.

Emerg Infect Dis 2018 Dec;24(12):2262-2269

Enterohemorrhagic Escherichia coli serogroup O80, involved in hemolytic uremic syndrome associated with extraintestinal infections, has emerged in France. We obtained circularized sequences of the O80 strain RDEx444, responsible for hemolytic uremic syndrome with bacteremia, and noncircularized sequences of 35 O80 E. coli isolated from humans and animals in Europe with or without Shiga toxin genes. Read More

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http://dx.doi.org/10.3201/eid2412.180272DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6256387PMC
December 2018
8 Reads

Hemolytic Uremic Syndrome.

Pediatr Clin North Am 2019 Feb;66(1):235-246

Departments of Pediatrics & Medicine, University of Colorado School of Medicine, 12631 E. 17th Avenue, Aurora, CO 80045, USA. Electronic address:

Hemolytic uremic syndrome (HUS) is the clinical triad of thrombocytopenia, anemia, and acute kidney injury. Classically associated with enterocolitis from Shiga toxin-producing Escherichia coli, HUS is also associated with Streptococcus pneumoniae infections; genetic dysregulation of the alternative complement pathway or coagulation cascade; and, rarely, a hereditary disorder of cobalamin C metabolism. These share a common final pathway of a prothrombotic and proinflammatory state on the endothelial cell surface, with fibrin and platelet deposition. Read More

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http://dx.doi.org/10.1016/j.pcl.2018.09.011DOI Listing
February 2019
10 Reads

Thrombotic microangiopathy following Bothrops jararaca snakebite: case report.

Clin Toxicol (Phila) 2018 Nov 16:1-6. Epub 2018 Nov 16.

c Department of Pharmacology, School of Medical Sciences , State University of Campinas (UNICAMP) , Campinas , Brazil.

Context: Thrombotic microangiopathy (TMA) is an uncommon and severe complication of snakebites, and is similar, in general, to hemolytic-uremic syndrome (HUS). We describe a case of TMA following envenomation by Bothrops jararaca.

Case Details: A 56-y-old-woman with controlled hypertension was transferred from a primary hospital to our ER ∼7 h after being bitten by B. Read More

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http://dx.doi.org/10.1080/15563650.2018.1514621DOI Listing
November 2018
8 Reads

A Case of Typical Hemolytic Uremic Syndrome in an Adult.

Cureus 2018 Sep 11;10(9):e3289. Epub 2018 Sep 11.

Hematology & Oncology, American University of Beirut Medical, Beirut, LBN.

Typical hemolytic uremic syndrome (HUS) in adults is an uncommon clinical occurrence and has been rarely reported in the literature. Typical HUS is mainly caused by Shiga toxin-producing (STEC) and is typically a pediatric disease. Worldwide outbreaks have been reported, one of the largest and most recent being in Germany. Read More

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https://www.cureus.com/articles/14645-a-case-of-typical-hemo
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http://dx.doi.org/10.7759/cureus.3289DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6235642PMC
September 2018
12 Reads

Emerging Shiga-toxin-producing Escherichia coli serogroup O80 associated hemolytic and uremic syndrome in France, 2013-2016: Differences with other serogroups.

PLoS One 2018 12;13(11):e0207492. Epub 2018 Nov 12.

Santé Publique France, Saint-Maurice, France.

To generate hypotheses on possible sources of Shiga toxin-producing Escherichia coli (STEC) serogroup O80 associated hemolytic-uremic syndrome (HUS), we explored differences in factors associated with STEC O80 associated HUS, compared with STEC O157 or STEC of other serogroups, in France during 2013-16. STEC was isolated from 153/521 (30%) reported HUS cases: 45 serogroup O80, 46 O157 and 62 other serogroups. Median ages were 1. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0207492PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6231688PMC
November 2018
1 Read
3.234 Impact Factor

Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use.

Pediatr Nephrol 2018 Nov 6. Epub 2018 Nov 6.

Radboud Institute for Molecular Life Sciences, Amalia Children's Hospital, Department of Pediatric Nephrology, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.

With the introduction of the complement C5-inhibitor eculizumab, a new era was entered for patients with atypical hemolytic uremic syndrome (aHUS). Eculizumab therapy very effectively reversed thrombotic microangiopathy and reduced mortality and morbidity. Initial guidelines suggested lifelong treatment and recommended prophylactic use of eculizumab in aHUS patients receiving a kidney transplant. Read More

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http://dx.doi.org/10.1007/s00467-018-4091-3DOI Listing
November 2018
1 Read

Pathogenesis of Atypical Hemolytic Uremic Syndrome.

J Atheroscler Thromb 2018 Nov 2. Epub 2018 Nov 2.

Division of Nephrology and Endocrinology, the University of Tokyo Hospital.

Atypical hemolytic uremic syndrome (aHUS) is a type of thrombotic microangiopathy (TMA) defined by thrombocytopenia, microangiopathic hemolytic anemia, and renal failure. aHUS is caused by uncontrolled complement activation in the alternative pathway (AP). A variety of genetic defects in complement-related factors or acquired autoantibodies to the complement regulators have been found in 50 to 60% of all cases. Read More

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https://www.jstage.jst.go.jp/article/jat/advpub/0/advpub_RV1
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http://dx.doi.org/10.5551/jat.RV17026DOI Listing
November 2018
6 Reads

Impact of a multidisciplinary team for the management of thrombotic microangiopathy.

PLoS One 2018 2;13(11):e0206558. Epub 2018 Nov 2.

Department of Gynecology and Obstetrics. Son Espases University Hospital, Palma de Mallorca, Spain.

Background: Thrombotic microangiopathy (TMA) is an important complication associated with several diseases that are rare and life-threatening. TMA is common to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). TTP is defined by a severe deficiency of ADAMTS13, and early treatment is associated with good prognosis. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0206558PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214549PMC
November 2018
19 Reads

Extrarenal manifestations of the hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC HUS).

Pediatr Nephrol 2018 Nov 1. Epub 2018 Nov 1.

Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN, USA.

Hemolytic uremic syndrome is commonly caused by Shiga toxin-producing Escherichia coli (STEC). Up to 15% of individuals with STEC-associated hemorrhagic diarrhea develop hemolytic uremic syndrome (STEC HUS). Hemolytic uremic syndrome (HUS) is a disorder comprising of thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. Read More

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http://dx.doi.org/10.1007/s00467-018-4105-1DOI Listing
November 2018
18 Reads

Bilateral renal cortical necrosis following acute pancreatitis - A rare complication of a common disease.

Saudi J Kidney Dis Transpl 2018 Sep-Oct;29(5):1211-1215

Department of Nephrology, Government T. D. Medical College, Alappuzha, Kerala, India.

Acute renal cortical necrosis is a rare cause of intrinsic acute kidney injury (AKI) which is commonly associated with obstetric complications such as placental abruption and some serious systemic disorders such as hemolytic-uremic syndrome, sepsis, severe burns, and snake bite. Acute pancreatitis is an extremely rare cause of renal cortical necrosis, and only less than 10 cases are reported in the literature. Here, we present a 24-year-old male presented with features of acute pancreatitis and oliguric AKI. Read More

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http://www.sjkdt.org/text.asp?2018/29/5/1211/243968
Publisher Site
http://dx.doi.org/10.4103/1319-2442.243968DOI Listing
November 2018
8 Reads

Virulence genes in Escherichia coli isolates from commercialized saltwater mussels Mytella guyanensis (Lamarck, 1819).

Braz J Biol 2018 Oct 25. Epub 2018 Oct 25.

Centro de Ciências da Saúde, Complexo Multidisciplinar de Estudos e Pesquisas em Saúde - COMEPS, Universidade Federal do Recôncavo da Bahia - UFRB, Avenida Carlos Amaral, 1015, Cajueiro, CEP 44574-490, Santo Antônio de Jesus, BA, Brasil.

The isolation of Escherichia coli from food is a major concern. Pathogenic strains of these bacteria cause diseases which range from diarrhea to hemolytic-uremic syndrome. Therefore the virulence genes in E. Read More

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http://dx.doi.org/10.1590/1519-6984.185930DOI Listing
October 2018
2 Reads