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Br J Haematol 2019 Feb 15. Epub 2019 Feb 15.

Hannover Medical School, Hannover, Germany.

Eculizumab is the first and only medication approved for paroxysmal nocturnal haemoglobinuria (PNH) and atypical haemolytic uraemic syndrome (aHUS) treatment. However, eculizumab safety based on long-term pharmacovigilance is unknown. This analysis summarises safety data collected from spontaneous and solicited sources from 16 March 2007 through 1 October 2016. Read More

Drugs 2019 Feb 14. Epub 2019 Feb 14.

Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Ravulizumab (ravulizumab-cwvz; ULTOMIRIS™), a humanized monoclonal antibody, is a complement C5 inhibitor developed by Alexion Pharmaceuticals for the treatment of paroxysmal nocturnal haemoglobinuria (PNH) and atypical haemolytic uraemic syndrome (aHUS). Like the first-generation C5 inhibitor, eculizumab, ravulizumab binds specifically and with high affinity to the complement protein C5, thereby preventing formation of the terminal complement complex C5b-9, which mediates cell lysis. In December 2018, intravenous ravulizumab received its first global approval in the USA for the treatment of adults with PNH, and is under regulatory review in the European Union and Japan in this indication. Read More

Clin Pharmacokinet 2019 Feb 13. Epub 2019 Feb 13.

Department of Pharmacy, Radboud University Medical Center, Nijmegen, The Netherlands.

Eculizumab is the first drug approved for the treatment of complement-mediated diseases, and current dosage schedules result in large interindividual drug concentrations. This review provides insight into the pharmacokinetic and pharmacodynamic properties of eculizumab, both for reported on-label (paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, generalized myasthenia gravis) and off-label (hematopoietic stem cell transplantation-associated thrombotic microangiopathy) indications. Furthermore, we discuss the potential of therapeutic drug monitoring to individualize treatment and reduce costs. Read More

G Ital Nefrol 2019 Feb;36(1)

Professore Ordinario di Nefrologia, Università degli Studi della Campania, Luigi Vanvitelli.

Atypical hemolytic-uremic syndrome (aHUS) is a rare, potentially lethal (1-4) systemic disorder, capable of affecting both adults and children, causing thrombotic microangiopathy (TMA) (5) that leads to the formation of thrombus within small blood vessels with multiple organ failure. The pathogenesis of the aHUS is part of a sort of chronic and uncontrolled activation of the complement system by genetic mutation of some proteins usually responsible for its self-regulation (6,7). Today, the rapid diagnosis of the disease and the timely start of treatment with eculizumab, improve outcomes of renal failure, stroke and heart attack (8-10). Read More

Clin Kidney J 2019 Feb 20;12(1):106-109. Epub 2018 Mar 20.

Department of Nephrology and Kidney Transplantation, Centre Hospitalier Universitaire de Montpellier, Montpellier, France.

Haemolytic uraemic syndrome (HUS) is a rare complication of invasive infection by (SP-HUS), especially in adults. Here we report an unusual case of a 53-year-old man presenting SP-HUS with severe multivisceral involvement. After failure of supportive care and plasma exchanges, eculizumab (anti-C5 antibody) resulted in a favourable outcome. Read More

Case Rep Womens Health 2019 Jan 23;21:e00097. Epub 2019 Jan 23.

University of South Alabama, Children's and Women's Hospital, Department of Obstetrics and Gynecology, 251 Cox Street, Mobile, AL 36604, United States.

Cerebrovascular disease is not uncommon during pregnancy as a result of either venous or arterial occlusion, or a hemorrhagic event, resulting in ischemia. Pregnancy may alter the prognosis of these neurologic disorders, with increased risks of morbidity and mortality for the mother and the developing fetus. Etiologies of stroke during pregnancy and the postpartum period include preeclampsia, eclampsia, HELLP syndrome, posterior reversible encephalopathy syndrome (PRES), amniotic fluid embolism, postpartum angiopathy, postpartum cardiomyopathy, thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), cerebral venous thrombosis, CNS infections, and maternal thrombophilia. Read More

J Biomed Sci 2019 Feb 7;26(1):16. Epub 2019 Feb 7.

Universidad de Buenos Aires, Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Instituto de Fisiología y Biofísica ''Houssay" (IFIBIO), Laboratorio de Neurofisiopatología, Facultad de Medicina, Paraguay 2155 piso 7, 1121, Buenos Aires, Argentina.

Background: Shiga toxin 2 from enterohemorrhagic Escherichia coli is the etiologic agent of bloody diarrhea, hemolytic uremic syndrome and derived encephalopathies that may result to death in patients. Being a Gram negative bacterium, lipopolysaccharide is also released. Particularly, the hippocampus has been found affected in patients intoxicated with Shiga toxin 2. Read More

Liver Int 2019 Feb 4. Epub 2019 Feb 4.

Hannover Medical School, Department of Gastroenterology, Hepatology and Endocrinology, Hannover, Germany.

Background & Aims: The prognosis of biliary tract cancer (BTC) is poor. Standard treatment for advanced BTC is a chemotherapy (CT) with gemcitabine and cisplatin. Phase-III evidence for a second line (2L) CT is lacking. Read More

CEN Case Rep 2019 Feb 4. Epub 2019 Feb 4.

Department of Medicine, Dr.Soliman Fakeeh Hospital, Jeddah, Saudi Arabia.

Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition. It is characterized by very high maternal mortality and morbidity. Most cases of P-aHUS (79%) manifest in the postpartum period; this is probably due to the complement's involvement in aHUS pathogenesis. Read More

J Clin Invest 2019 Feb 4. Epub 2019 Feb 4.

Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.

Atypical hemolytic uremic syndrome (aHUS) is frequently associated in humans with loss-of-function mutations in complement-regulating proteins or gain-of-function mutations in complement-activating proteins. Thus, aHUS provides an archetypal complement-mediated disease with which to model new therapeutic strategies and treatments. Herein, we show that, when transferred to mice, an aHUS-associated gain-of-function change (D1115N) to the complement-activation protein C3 results in aHUS. Read More

J Paediatr Child Health 2019 Feb 3. Epub 2019 Feb 3.

Division of Pediatric Nephrology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates.

Aim: Atypical haemolytic uraemic syndrome (aHUS) is a debilitating condition that can cause significant morbidity and mortality in children if not adequately and promptly treated. This report shares real-world data on the use of eculizumab in children with aHUS.

Methods: We report our experience with the use of eculizumab in 14 children with aHUS. Read More

Am J Pathol 2019 Jan 31. Epub 2019 Jan 31.

Department of Systems Pharmacology and Translational Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Electronic address:

Single nucleotide polymorphisms and rare mutations in Factor H (FH; official name CFH) are associated with age-related macular degeneration and atypical hemolytic uremic syndrome, a form of thrombotic microangiopathy. Mice with the FH W1206R mutation (FH) share features with human atypical hemolytic uremic syndrome. Here, we report that FH mice exhibited retinal vascular occlusion and ischemia. Read More

Wilderness Environ Med 2019 Jan 30. Epub 2019 Jan 30.

Faculty of Medicine, University of Peradeniya, Peredeniya, Sri Lanka.

Thrombotic microangiopathy (TMA), which includes the spectrum of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, is an uncommon complication of hump-nosed pit viper envenomation. We describe 4 cases of TMA following hump-nosed pit viper (Hypnale spp) bites in Sri Lanka. The first case is a typical TMA that spontaneously resolved with supportive treatments. Read More

Ter Arkh 2018 Jun;90(6):28-34

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Aim: Analysis of clinical manifestations, course and outcomes of obstetric aHUS.

Materials And Methods: 45 patients with aHUS development during pregnancy or immediately after childbirth were observed between 2011 and 2017, age from 16 to 42 years.

Results: All patients had AKI (serum creatinine 521,5±388,0 µmol/l, oliguria or anuria that required initiation of hemodialysis). Read More

Pediatr Nephrol 2019 Jan 28. Epub 2019 Jan 28.

Pediatric Nephrology, Hospital Universitari Vall d' Hebron, Universitat Autonoma de Barcelona, Barcelona, Spain.

Klin Lab Diagn 2018 ;63(4):249-253

The Federal Budget Institution of Science "The State Scientific Center of Applied Microbiology and Biotechnology" of Rospotrebnadzor, 142279, village Obolensk of the Moscow Oblast, Russia.

The diarrheagenic bacteria coli take a significant place among agents of acute intestinal infections in children aged under 5 years. The main danger among these pathogens is represented by both enterotoxigenic E. coli causing enteritis and enterocolitis accompanied by acute dehydration diarrhea and Escherichia producing shiga-toxin being agents of hemorrhagic colitis and hemolytic uremic syndrome. Read More

Blood Coagul Fibrinolysis 2019 Mar;30(2):68-70

Department of Clinical Haematology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

: We bring the case of a 38-year-old man who was presented to the emergency department with nausea, fever, and choluria, 4 days after the ingestion of raw oysters. Analytical study revealed thrombocytopenia and acute kidney injury that were associated to a possible thrombotic microangiopathy. Therapeutic plasma exchange was started and resolution of the manifestations was obtained. Read More

Clin J Am Soc Nephrol 2019 01 23. Epub 2019 Jan 23.

Pediatric Nephrology Department, Hôpital Robert Debré, University Paris Diderot, Assistance Publique-Hôpitaux de Paris, Paris, France

Background And Objectives: Inherited complement hyperactivation is critical for the pathogenesis of atypical hemolytic uremic syndrome (HUS) but undetermined in postdiarrheal HUS. Our aim was to investigate complement activation and variants of complement genes, and their association with disease severity in children with Shiga toxin-associated HUS.

Design, Setting, Participants, & Measurements: Determination of complement biomarkers levels and next-generation sequencing for the six susceptibility genes for atypical HUS were performed in 108 children with a clinical diagnosis of post-diarrheal HUS (75 Shiga toxin-positive, and 33 Shiga toxin-negative) and 80 French controls. Read More

Infect Immun 2019 Jan 22. Epub 2019 Jan 22.

Department of Microbiology and Immunology, Uniformed Services University of the Health Sciences, Bethesda, MD, USA

Shiga toxin (Stx)-producing (STEC) cause food-borne outbreaks of bloody diarrhea. There are two major types of immunologically distinct Stxs, Stx1a and Stx2a. Stx1a is more cytotoxic to Vero cells than Stx2a, but Stx2a has a lower lethal dose 50% (LD) in mice. Read More

Case Rep Nephrol 2018 18;2018:3810249. Epub 2018 Dec 18.

Research Laboratory, Nordland Hospital, Bodø, Norway.

We report a pediatric patient with atypical hemolytic uremic syndrome due to a C3 gain-of-function mutation diagnosed in infancy. She was treated from the start with a constant dose of 300 mg eculizumab every second week from the onset and followed by routine complement analyses for six years. Her complement system was completely inhibited and the dose interval was prolonged from 2 to 3 weeks without alteration of the dose and the complement activity continued to be completely inhibited. Read More

Clin Case Rep 2019 Jan 22;7(1):131-134. Epub 2018 Nov 22.

Department of Respiratory Medicine Saitama Cardiovascular and Respiratory Center Saitama Japan.

Development of hemophagocytic syndrome and thrombotic microangiopathy due to community-acquired pneumonia is rare, but delayed management of these complications can lead to a poor prognosis. Infection by both and influenza virus can cause these complications; thus, physicians should pay attention to them when treating influenza-associated pneumococcal pneumonia. Read More

Clin Med (Lond) 2019 Jan;19(1):72-74

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

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

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

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.

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

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

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

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

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

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

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

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

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

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

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

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

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

Int J Food Microbiol 2019 Mar 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

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

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

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

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

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

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

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

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

Pediatr Nephrol 2019 Mar 17;34(3):533-537. 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

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

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

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

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