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J Intensive Care Soc 2020 May 7;21(2):191-193. Epub 2019 Mar 7.

Haematology Department, University College London Hospital, London, UK.

A 19-year-old female with no medical history presented with bloody diarrhoea. Investigations revealed an acute kidney injury, thrombocytopenia and microangiopathic haemolysis. A diagnosis of haemolytic uraemic syndrome secondary to Shiga toxin-producing 055 was confirmed and supportive therapy commenced in the intensive therapy unit. Read More

Blood 2020 Jun 2. Epub 2020 Jun 2.

Centro de Investigaciones Biológicas (CSIC), Madrid, Spain.

Syst Biol Reprod Med 2020 Jun;66(3):151-169

Laboratory of Vascular Biology and Histopathology, Institute of Health Sciences and Health, Federal University of Mato Grosso , Barra Do Garcas, Brazil.

The placenta works as a selective barrier, protecting the fetus from potential infections that may affect the maternal organism during pregnancy. In this review, we will discuss several challenging infections that are common within Latin American countries and that may affect the maternal-fetal interface and pose risks to fetal development. Specifically, we will focus on emerging infectious diseases including the arboviruses, malaria, leishmaniasis, and the bacterial foodborne disease caused by Shiga toxin-producing . Read More

Medicine (Baltimore) 2020 May;99(22):e19731

Department of Surgery, University of Florida Health Sciences Center.

Introduction: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by the sudden onset of hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). HUS is categorized as either typical, caused by Shiga toxin-producing Escherichia coli infection, or atypical HUS (aHUS), usually complement mediated or secondary to systemic disease. We describe a rare case of aHUS in an adult male patient with recurrent acute pancreatitis. Read More

BMC Nephrol 2020 May 29;21(1):204. Epub 2020 May 29.

Department of Nephrology, CHU Tenon, Assistance Publique-Hopitaux de Paris, Paris, France.

Background: Thrombotic microangiopathies (TMAs) occurring in the postpartum period may be difficult to manage. They present as the combination of mechanical hemolytic anemia and consumption thrombocytopenia due to endothelial dysfunction. The cause of this endothelial aggression can be multiple: thrombocytopenic thrombotic purpura (TTP), HELLP syndrome, antiphospholipid syndrome, atypical hemolytic and uremic syndrome or acute fatty liver of pregnancy. Read More

Arch Argent Pediatr 2020 Jun;118(3):e305-e308

Hospital Interzonal General Dr. José Penna, Bahía Blanca, Buenos Aires, Argentina.

Streptococcus pneumoniae associated hemolytic uremic syndrome (Sp-HUS) is defined as microangiopathic hemolytic anemia, thrombocytopenia and acute renal injury, in a patient with Streptococcus pneumoniae (Sp) invasive infection. A 2-year-old boy was admitted with pneumonia and empyema. Sp was isolated from blood and pleural fluid cultures. Read More

Pediatr Nephrol 2020 May 28. Epub 2020 May 28.

Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.

Int Immunopharmacol 2020 May 25;85:106617. Epub 2020 May 25.

Defence Food Research Laboratory, India.

Escherichia coli O157:H7 and Shigella flexneri are the predominant diarrhoeal pathogens and those strains producing Shiga toxins cause life-threatening sequelae including hemolytic uremic syndrome (HUS) upon their entry into the host. Intimate adherence of E. coli O157 and invasion of S. Read More

Emerg Microbes Infect 2020 Dec;9(1):1065-1076

Université Clermont Auvergne, INRAE, MEDiS, F-63000 Clermont-Ferrand, France.

Enterohaemorrhagic (EHEC) are bacterial pathogens responsible for life-threatening diseases in humans such as bloody diarrhoea and the hemolytic and uremic syndrome. To date, no specific therapy is available and treatments remain essentially symptomatic. In recent years, we demonstrated that nitric oxide (NO), a major mediator of the intestinal immune response, strongly represses the synthesis of the two cardinal virulence factors in EHEC, namely Shiga toxins (Stx) and the type III secretion system, suggesting NO has a great potential to protect against EHEC infection. Read More

Toxins (Basel) 2020 May 23;12(5). Epub 2020 May 23.

Friedrich-Loeffler-Institut/Federal Research Institute for Animal Health, Institute of Molecular Pathogenesis, Naumburger Str. 96a, D-07743 Jena, Germany.

Shiga toxins (Stxs), syn. Vero(cyto)toxins, are potent bacterial exotoxins and the principal virulence factor of enterohemorrhagic (EHEC), a subset of Shiga toxin-producing (STEC). EHEC strains, e. Read More

Placenta 2020 Apr 17;95:9-17. Epub 2020 Apr 17.

Mount Sinai Hospital, Department of Obstetrics and Gynaecology, Division of Maternal-Fetal Medicine, Toronto, Canada; University of Toronto, Department of Medicine, Toronto, Canada; Lunenfeld-Tanenbaum Research Institute, Mount Sinai Hospital, Toronto, Canada.

Introduction: Abnormal placental development is a unifying factor amongst many adverse pregnancy outcomes (APOs) in Sickle Cell Disease (SCD). Our aim was to describe placental histopathologic findings in women with SCD and their relationship with APOs, and to explore the association between antenatal sonographic findings and placental pathology.

Methods: Retrospective single-centre case series of all pregnant women with SCD (January 2000-December 2017), pregnancy beyond 20 weeks' gestation, and available placenta histopathology. Read More

Braz J Microbiol 2020 May 24. Epub 2020 May 24.

Laboratório de Bacteriologia, Instituto Butantan, São Paulo, Brazil.

Shiga toxin-producing Escherichia coli (STEC) pathotype secretes two types of AB cytotoxins (Stx1 and Stx2), responsible for complications such as hemorrhagic colitis (HC) and hemolytic uremic syndrome (HUS) in infected patients, which could lead to sequels and death. Currently, there is no effective treatment against the cytotoxic effect of these toxins. However, in order to approve any therapy molecule, an animal experiment is required in order to evaluate the efficacy and safety of therapeutic approaches. Read More

Kidney Int 2020 Jun;97(6):1106-1108

Department of Nephrology and Hypertension, Medical School Hannover, Hannover, Germany. Electronic address:

Ravulizumab, a new long-acting C5 inhibitor, recently received FDA approval for the treatment of aHUS. Rates of complete thrombotic microangiopathy response were similar to those observed in major eculizumab trials; however, fewer patients in the ravulizumab study were able to stop dialysis, probably due to differences in the study populations. Until additional data/analyses are available, eculizumab remains the drug of choice for an acute aHUS episode, whereas ravulizumab has several advantages in maintenance treatment. Read More

Front Cell Infect Microbiol 2020 6;10:169. Epub 2020 May 6.

Institute for Infectiology, University of Münster, Münster, Germany.

Infections with Shiga toxin-producing (STEC) cause outbreaks of severe diarrheal disease in children and the elderly around the world. The severe complications associated with toxin production and release range from bloody diarrhea and hemorrhagic colitis to hemolytic-uremic syndrome, kidney failure, and neurological issues. As the use of antibiotics for treatment of the infection has long been controversial due to reports that antibiotics may increase the production of Shiga toxin, the recommended therapy today is mainly supportive. Read More

BMC Nephrol 2020 May 20;21(1):189. Epub 2020 May 20.

Department of Medicine, Division of Nephrology, The Johns Hopkins University School of Medicine, 600 N Wolfe St, Carnegie 344B, Baltimore, Maryland, 21287, USA.

Background: Atypical hemolytic uremic syndrome (aHUS) can result in severe kidney dysfunction, secondary to thrombotic microangiopathy. Eculizumab has been used to treat this disorder, and has resulted in favourable outcomes in both, native and transplanted kidneys. There is limited long term follow up data in kidney transplant recipients (KTRs) who received prevention and treatment with Eculizumab. Read More

Shock 2020 May 18. Epub 2020 May 18.

Department of Anesthesiology and Intensive Care Medicine, Jena University Hospital, Jena, Germany.

The hemolytic-uremic syndrome (HUS) is a thrombotic microangiopathy which can occur as a severe systemic complication after an infection with Shiga-toxin-(Stx)-producing Escherichia coli (STEC). Elevated levels of proinflammatory cytokines associated with the classical NF-κB signaling pathway were detected in the urine of HUS patients. Thus, we hypothesize that the immune response of the infected organism triggered by Stx can affect the kidneys and contributes to acute kidney injury. Read More

Front Cell Infect Microbiol 2020 5;10:195. Epub 2020 May 5.

Department of Cell Biology, Centro de Investigación y de Estudios Avanzados del IPN (CINVESTAV-IPN), Mexico City, Mexico.

Shiga-toxin-producing (STEC) has become an important pathogen that can cause diarrhea, hemorrhagic colitis and hemolytic uremic syndrome (HUS) in humans. Recent reports show that the type VI secretion system (T6SS) from EHEC is required to produce infection in a murine model and its expression has been related to a higher prevalence of HUS. In this work, we use bioinformatics analyses to identify the core genes of the T6SS and compared the differences between these components among the two published genomes for EHEC O157:H7 strain EDL933. Read More

Eur J Pediatr 2020 May 18. Epub 2020 May 18.

Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague, and Motol University Hospital, Prague, Czech Republic.

Atypical haemolytic uraemic syndrome is an ultra-rare, life-threatening disease. Causative variants in genes that encode complement factors can be identified in 40-70% of cases. We performed genetic analysis of 21 Czech children with atypical haemolytic uraemic syndrome. Read More

Transplant Proc 2020 May 15. Epub 2020 May 15.

Department of Organ Transplantation, Koç University Hospital.

Clin Exp Nephrol 2020 May 15. Epub 2020 May 15.

Department of Nephrology and Hypertension, Kawasaki Medical School, Kurashiki, Japan.

Background: There have been only a few large-scale cohort studies that have reviewed accumulated cases of thrombotic microangiopathy (TMA). The aim of this study was to collect and analyze TMA cases based on the renal biopsy, as a nationwide survey in Japan.

Methods: In this cross-sectional study, large nationwide data from the Japan Renal Biopsy Registry (J-RBR) were used. Read More

Mol Genet Metab 2020 May 5. Epub 2020 May 5.

Department of Human Genetics, McGill University, Montreal, Quebec, Canada.; Division of Medical Genetics, Department of Specialized Medicine, McGill University Health Centre, Montreal, Quebec, Canada.; Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada.; Division of Medical Biochemistry, Department of Specialized Medicine, McGill University Health Centre, Montreal, Quebec, Canada.

MTHFD1 is a trifunctional protein containing 10-formyltetrahydrofolate synthetase, 5,10-methenyltetrahydrofolate cyclohydrolase and 5,10-methylenetetrahydrofolate dehydrogenase activities. It is encoded by MTHFD1 and functions in the cytoplasmic folate cycle where it is involved in de novo purine synthesis, synthesis of thymidylate and remethylation of homocysteine to methionine. Since the first reported case of severe combined immunodeficiency resulting from MTHFD1 mutations, seven additional patients ascertained through molecular analysis have been reported with variable phenotypes, including megaloblastic anemia, atypical hemolytic uremic syndrome, hyperhomocysteinemia, microangiopathy, infections and autoimmune diseases. Read More

Ceska Gynekol 2020 ;85(1):30-34

Objective: Case of a primigravid woman who suffered from severe PTMS (postpartum thrombotic microangiopathy syndrome) in the 26th week of pregnancy.

Design: Case report.

Setting: Department of Gynecology and Obstetrics, Hospital Nový Jičín; Department of Gynecology and Obstetrics, University Hospital Ostrava; Department of Hematooncology, University Hospital Ostrava; Department of Anaesthesiology and Resuscitation, University Hospital Ostrava. Read More

Ceska Gynekol 2020 ;85(1):18-28

Objective: The aim of this study is to draw attention to a nosological unit called thrombotic microangiopathy (TMA). This syndrome represents a serious pathological condition characterized by microangiopathic haemolytic anemia (MAHA), thrombocytopenia and various organ dysfunction. Patients are most often presented with symptoms of the HELLP syndrome but if the clinical picture is not restituted within 48-72 hours after delivery, other TMAs should be considered. Read More

Eur J Med Genet 2020 May 13:103953. Epub 2020 May 13.

Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Úvalu 84, 150 06, Prague 5, Czech Republic. Electronic address:

Atypical haemolytic uraemic syndrome and steroid-resistant nephrotic syndrome are highly rare kidney diseases that can occur in childhood. In some cases, genetic variants may trigger these conditions, although in atypical haemolytic uraemic syndrome they mostly confer only a predisposition to the disease. Most variants causing atypical haemolytic uraemic syndrome were identified in genes encoding proteins regulating the complement pathway; on the other hand, there are approximately 58 genes encoding distinct proteins primarily causing steroid-resistant nephrotic syndrome. Read More

Pediatr Dev Pathol 2020 May 14:1093526620925947. Epub 2020 May 14.

Department of Pediatric Nephrology, Gazi University School of Medicine, Ankara, Turkey.

Background: The aim of this study was to investigate the histopathological findings in kidney biopsies in children with atypical hemolytic uremic syndrome (aHUS) and to determine whether specific pathological findings in aHUS have a prognostic value.

Methods: Renal biopsy specimens of 29 patients who were recorded in the national Turkish aHUS registry database were available for review. Histopathological findings were compared with the clinical and laboratory features at the presentation and the final outcome. Read More

Saudi J Kidney Dis Transpl 2020 Mar-Apr;31(2):493-502

Department of Nephrology, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.

This study was initiated to look into the etiologies, prevalence, and outcome of pregnancy-related acute kidney injury (PRAKI) in a tertiary care hospital. Women admitted with PRAKI from January 2015 to December 2016 were included in the study. All patients were investigated and treated and followed up for the next six months. Read More

Kidney Int 2020 Jun 28;97(6):1260-1274. Epub 2020 Feb 28.

National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK. Electronic address:

Recessive mutations in diacylglycerol kinase epsilon (DGKE) display genetic pleiotropy, with pathological features reported as either thrombotic microangiopathy or membranoproliferative glomerulonephritis (MPGN), and clinical features of atypical hemolytic uremic syndrome (aHUS), nephrotic syndrome or both. Pathophysiological mechanisms and optimal management strategies have not yet been defined. In prospective and retrospective studies of aHUS referred to the United Kingdom National aHUS service and prospective studies of MPGN referred to the National Registry of Rare Kidney Diseases for MPGN we defined the incidence of DGKE aHUS as 0. Read More

Microorganisms 2020 May 6;8(5). Epub 2020 May 6.

Institute of Hygiene, University of Münster, 48149 Münster, Germany.

Enterohemorrhagic (EHEC) is a zoonotic pathogen responsible for life-threating diseases such as hemolytic uremic syndrome. While its major virulence factor, the Shiga toxin (Stx), is known to exert its cytotoxic effect on various endothelial and epithelial cells when in its free, soluble form, Stx was also recently found to be associated with EHEC outer membrane vesicles (OMVs). However, depending on the strain background, other toxins can also be associated with native OMVs (nOMVs), and nOMVs are also made up of immunomodulatory agents such as lipopolysaccharides and flagellin. Read More

Nephrology (Carlton) 2020 May 7. Epub 2020 May 7.

Department of Renal Medicine, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.

Aims: To describe the baseline characteristics and treatment of Australian patients diagnosed with atypical haemolytic uraemic syndrome (aHUS) reported to the Global aHUS Registry.

Methods: Descriptive analysis of the Australian cohort with aHUS (n = 106) was undertaken for demographics, disease characteristics and prior treatment with eculizumab; comparing with the global cohort (n = 1688) for certain pre-specified disease characteristics.

Results: In Australia, almost two-thirds of patients diagnosed with aHUS were female and over 80% of patients were Caucasians, with similar proportions reported in the global cohort. Read More

Am J Case Rep 2020 May 3;21:e922567. Epub 2020 May 3.

Department of Internal Medicine, Inje University Haeundae Paik Hospital, Busan, South Korea.

BACKGROUND Hemolytic uremic syndrome (HUS) can be categorized as primary (typical or atypical) or secondary (with a coexisting diseases). Typical HUS usually means shiga-toxin-medicated and thrombotic thrombocytopenic purpura. Secondary HUS is often initiated by coexisting diseases or conditions such as infections, transplantation, cancer, and autoimmune disease. Read More

Iran J Kidney Dis 2020 May;14(3):173-183

Aliasghar Clinical Research Development center, Aliasghar Children Hospital,Iran University of Medical Sciences, Tehran.

HUS is a leading cause of AKI in infants. Though new classification of HUS is based on underlying disease, it traditionally defines as diarrhea positive (typical) and negative (atypical). We have no figure of the incidence and prevalence of HUS, the underlying disease and the outcome in Iranian patients. Read More

Case Rep Nephrol Dial 2020 Jan-Apr;10(1):35-41. Epub 2020 Apr 14.

Pediatric Intensive Care Unit, Bachir Ben Nacer Hospital, Biskra, Algeria.

Insertion of a peritoneal dialysis (PD) catheter is frequently done by interventional nephrologists, but these procedures are typically only performed for adults. Almost all invasive procedures in children are performed by pediatric surgeons. If a pediatric surgeon is unavailable, the initiation of PD in acute situations may be delayed, thus increasing the risk of complications and chronic kidney disease. Read More

Eur Heart J Case Rep 2020 Apr 12;4(2):1-6. Epub 2020 Mar 12.

Division of Cardiovascular Medicine, Department of Internal Medicine, Davis Heart & Lung Research Institute, The Ohio State University Wexner Medical Center, 473 W 12th Ave, Suite 200, Columbus, OH 43210, USA.

Background: Atypical haemolytic uraemic syndrome (aHUS) is a life-threatening, genetic disease of complement-mediated thrombotic microangiopathy that typically presents as anaemia, thrombocytopenia, and renal failure. Cardiomyopathy is seen in up to 10% of aHUS cases, but the aetiology is not well-understood.

Case Summary: A 63-year-old man recently was diagnosed with a thrombotic microangiopathy most consistent with aHUS by renal biopsy after presentation with acute renal failure requiring haemodialysis. Read More

Br J Haematol 2020 Apr 27. Epub 2020 Apr 27.

Division of Maternal-Fetal Medicine, Department of Obstetrics & Gynaecology, Mount Sinai Hospital, Toronto, Canada.

Br J Haematol 2020 Apr 27. Epub 2020 Apr 27.

Department of Nephrology and Clinical Immunology, Maastricht University Medical Center, Maastricht, the Netherlands.

Pregnancy has been linked to various microangiopathies, including primary atypical haemolytic uraemic syndrome (aHUS). Complement dysregulation, often linked to rare variants in complement genes, is key for primary aHUS to manifest and may play a role in pregnancy complications of the mother and fetus. The burden of such complications is unknown, making counselling of women with primary aHUS and asymptomatic relatives difficult. Read More

J Matern Fetal Neonatal Med 2020 Apr 26:1-9. Epub 2020 Apr 26.

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

Complement is a part of the innate immune system with a critical role in host defense. Although essential for survival, when dysregulated or excessively triggered complement activation can cause tissue damage and drive inflammatory and immune disorders. The alternative pathway of complement (APC) is especially important for survival against infection and can be triggered by a variety of settings: infection, trauma, surgery, or pregnancy. Read More

Wilderness Environ Med 2020 Apr 23. Epub 2020 Apr 23.

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

Introduction: Hump-nosed pit vipers (Hypnale spp) cause the highest number of venomous snakebites in Sri Lanka. Bites commonly cause local envenoming leading to local pain, swelling, and necrosis of the site of the bite. Acute kidney injury is the most common systemic manifestation, and some patients develop venom-induced consumption coagulopathy (VICC). Read More

Pediatr Nephrol 2020 Apr 22. Epub 2020 Apr 22.

Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital|, P.O. Box 347, 00029 HUS, Helsinki, Finland.

Background: Hemolytic uremic syndrome (HUS) is a multisystemic disease. In a nationwide study, we characterized the incidence, clinical course, and prognosis of HUS caused by Shiga toxin (Stx)-producing Escherichia coli (STEC) strains with emphasis on risk factors, disease severity, and long-term outcome.

Methods: The data on pediatric HUS patients from 2000 to 2016 were collected from the medical records. Read More

Microb Pathog 2020 Apr 19;145:104199. Epub 2020 Apr 19.

Department of Bacteriology and Virology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Objective: Enterohemorrhagic Escherichia coli (E. coli O157: H7) is an enteric pathogen, transmitted through contaminated water and food. Pathogenic factors include bacterial adhesion, invasion of intestinal epithelial and epithelium cells. Read More

Clin Transplant 2020 Apr 20:e13885. Epub 2020 Apr 20.

Division of Nephrology, Oregon Health and Science University, Portland, OR, USA.

Thrombotic microangiopathy (TMA) is a recognized and serious complication of renal transplantation. Atypical hemolytic uremic syndrome (aHUS), a subset of TMA, occurs in the setting of dysregulation of the alternative complement pathway and can cause disease in native kidneys as well as recurrence in allografts. De novo TMA represents a classification of TMA post-transplant in the absence of clinical or histopathological evidence of TMA or aHUS in the native kidney. Read More

J Clin Invest 2020 May;130(5):2152-2163

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

The renaissance of complement diagnostics and therapeutics has introduced precision medicine into a widened field of complement-mediated diseases. In particular, complement-mediated diseases (or complementopathies) with ongoing or published clinical trials of complement inhibitors include paroxysmal nocturnal hemoglobinuria, cold agglutinin disease, hemolytic uremic syndrome, nephropathies, HELLP syndrome, transplant-associated thrombotic microangiopathy, antiphospholipid antibody syndrome, myasthenia gravis, and neuromyelitis optica. Recognizing that this field is rapidly expanding, we aim to provide a state-of-the-art review of (a) current understanding of complement biology for the clinician, (b) novel insights into complement with potential applicability to clinical practice, (c) complement in disease across various disciplines (hematology, nephrology, obstetrics, transplantation, rheumatology, and neurology), and (d) the potential future of precision medicine. Read More

BMC Pediatr 2020 Apr 17;20(1):169. Epub 2020 Apr 17.

Pediatric Nephrology Department, Shengjing Hospital of China Medical University, No.36 Sanhao Street Heping District, Shenyang City, 110004, Liaoning Province, China.

Background: Autoimmune thyroid disease (AITD) has a complex pathogenesis and is associated with the development of autoimmunity against the thyroid. Graves' disease and Hashimoto's thyroiditis are the two main types of AITD, and they are characterized by thyrotoxicosis and hypothyroidism, respectively. Atypical hemolytic uremic syndrome (aHUS) is a rare disease, presenting with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Read More

J Hematol 2019 Jun 30;8(2):64-67. Epub 2019 Jun 30.

Division of Hematology and Cellular Therapy, Western Pennsylvania Hospital, Pittsburgh, PA, USA.

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) which generally presents as a triad of thrombocytopenia, hemolytic anemia and renal failure. We present the case of a 69-year-old woman with ongoing fevers, arthralgias, diffuse rash and pharyngitis for 3 months. Investigation revealed an elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and ferritin; however, autoimmune and infectious studies were unremarkable, raising the suspicion for adult onset Still's disease (AOSD). Read More

Kidney Int 2020 Jun 6;97(6):1287-1296. Epub 2020 Mar 6.

Department of Nephrology and Hypertension, Hannover Medical School, Hannover, Germany.

Ravulizumab is a long-acting C5 inhibitor engineered from eculizumab with increased elimination half-life, allowing an extended dosing interval from two to eight weeks. Here we evaluate the efficacy and safety of ravulizumab in adults with atypical hemolytic uremic syndrome presenting with thrombotic microangiopathy. In this global, phase 3, single arm study in complement inhibitor-naïve adults (18 years and older) who fulfilled diagnostic criteria for atypical hemolytic uremic syndrome, enrolled patients received ravulizumab through a 26-week initial evaluation period. Read More

Ir Med J 2020 Jan 16;113(1). Epub 2020 Jan 16.

Public Health Laboratory, Health Service Executive Dublin Mid-Leinster, Cherry Orchard Hospital, Ballyfermot, Dublin 10, Ireland.

Aims To describe laboratory data on clinical human Verotoxigenic E. coli (VTEC) strains causing haemolytic uraemic syndrome (HUS) and to characterise the VTEC strains, thus contributing to risk mitigation to decrease HUS incidence in Ireland. Methods Laboratory characterisation was performed on isolates from 52 VTEC-associated HUS cases identified in the National clinical VTEC Reference Laboratory (NRL-VTEC) for the years 2012-2014. Read More

Front Cell Infect Microbiol 2020 31;10:123. Epub 2020 Mar 31.

Molecular Medicine, Research Institute, Hospital for Sick Children, Toronto, ON, Canada.

Verotoxin, VT (aka Shiga toxin,Stx) is produced by enterohemorrhagic (EHEC) and is the key pathogenic factor in EHEC-induced hemolytic uremic syndrome (eHUS-hemolytic anemia/thrombocytopenia/glomerular infarct) which can follow gastrointestinal EHEC infection, particularly in children. This AB5 subunit toxin family bind target cell globotriaosyl ceramide (Gb), a glycosphingolipid (GSL) (aka CD77, pk blood group antigen) of the globoseries of neutral GSLs, initiating lipid raft-dependent plasma membrane Gb clustering, membrane curvature, invagination, scission, endosomal trafficking, and retrograde traffic via the TGN to the Golgi, and ER. In the ER, A/B subunits separate and the A subunit hijacks the ER reverse translocon (dislocon-used to eliminate misfolded proteins-ER associated degradation-ERAD) for cytosolic access. Read More

Clin Kidney J 2020 Apr 18;13(2):208-216. Epub 2019 Jun 18.

Internal Medicine, Nephrology and Intensive Care Medicine, Phillips University of Marburg, Marburg, Germany.

Background: Data are lacking on the relative incidence of thrombotic thrombocytopenic purpura (TTP), haemolytic uraemic syndrome (HUS) caused by Shiga toxin-producing (STEC) and atypical HUS (aHUS) in patients presenting with thrombotic microangiopathies (TMAs).

Methods: This was a prospective, cross-sectional, multicentre and non-interventional epidemiological study. Patients fulfilling criteria for TMAs (platelet consumption, microangiopathic haemolytic anaemia and organ dysfunction) were included in the study. Read More

Int J Mol Sci 2020 Apr 12;21(8). Epub 2020 Apr 12.

Division of Bacterial Disease Research, Center for Infectious Disease Research, Korea National Institute of Health, Cheongju, Chungchungbuk-do 28160, Korea.

Enterohemorrhagic (EHEC) causes hemorrhagic colitis and hemolytic uremic syndrome. EHEC infection begins with bacterial adherence to the host intestine via lectin-like adhesins that bind to the intestinal wall. However, EHEC-related lectin-glycan interactions (LGIs) remain unknown. Read More

Circulation 2020 06 9;141(22):1739-1741. Epub 2020 Apr 9.

Division of Cardiovascular Medicine, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus.