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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 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

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

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

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

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

Hematology Am Soc Hematol Educ Program 2018 Nov;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

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

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

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

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

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

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

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

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

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

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

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

Inflamm Bowel Dis 2018 Nov 8. Epub 2018 Nov 8.

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

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

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

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

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

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

J Am Soc Nephrol 2018 Dec 30;29(12):2809-2819. Epub 2018 Oct 30.

Molecular Otolaryngology and Renal Research Laboratories,

Background: Genetic variation in complement genes is a predisposing factor for atypical hemolytic uremic syndrome (aHUS), a life-threatening thrombotic microangiopathy, however interpreting the effects of genetic variants is challenging and often ambiguous.

Methods: We analyzed 93 complement and coagulation genes in 400 patients with aHUS, using as controls 600 healthy individuals from Iowa and 63,345 non-Finnish European individuals from the Genome Aggregation Database. After adjusting for population stratification, we then applied the Fisher exact, modified Poisson exact, and optimal unified sequence kernel association tests to assess gene-based variant burden. Read More

World J Transplant 2018 Oct;8(6):203-219

Faculty of Health and Science, University of Liverpool, Institute of Learning and Teaching, School of Medicine, Liverpool L69 3GB, United Kingdom.

For decades, kidney diseases related to inappropriate complement activity, such as atypical hemolytic uremic syndrome and C3 glomerulopathy (a subtype of membranoproliferative glomerulonephritis), have mostly been complicated by worsened prognoses and rapid progression to end-stage renal failure. Alternative complement pathway dysregulation, whether congenital or acquired, is well-recognized as the main driver of the disease process in these patients. The list of triggers include: surgery, infection, immunologic factors, pregnancy and medications. Read More

Pediatr Nephrol 2018 Oct 27. Epub 2018 Oct 27.

Service de Pédiatrie, Centre de référence Maladies Rénales Rares du Sud-ouest (SoRare), CHU de Bordeaux, Bordeaux, France.

Background: Hemolytic uremic syndrome due to Shiga toxin-producing E. coli (STEC-HUS) is the main cause of acute kidney injury in young children. Most fully recover kidney function; however, some develop long-term sequelae. Read More

Am J Med 2018 Oct 24. Epub 2018 Oct 24.

Department of Medicine, College of Medicine, University of Arizona, Tucson; Arizona Kidney Disease and Hypertension Center, Tucson. Electronic address:

Patient 2018 Oct 26. Epub 2018 Oct 26.

Department of Clinical Epidemiology and Medical Technology Assessment, Care and Public Health Research Institute (CAPHRI), Maastricht University Medical Centre, Maastricht, The Netherlands.

Background: This study assessed the validity and reliability of the generic module of the recently developed Patient Experiences and Satisfaction with Medications (PESaM) questionnaire in a sample of patients in the Netherlands.

Methods: The generic module of the PESaM questionnaire consists of 18 items related to the domains effectiveness, side effects and ease of use of medications. It assesses patients' experiences regarding the impact of the medication on daily life, health and satisfaction. Read More

Pediatr Nephrol 2018 Oct 26. Epub 2018 Oct 26.

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.

Background: Providing proof of presence of Shiga toxin-producing E. coli (STEC) infection forms the basis for differentiating STEC-hemolytic uremic syndrome (HUS) and atypical HUS. As the gold standard to diagnose STEC-HUS has limitations, using ELISA to detect serum antibodies against STEC lipopolysaccharides (LPS) has proven additional value. Read More

Transplantation 2018 Oct 26. Epub 2018 Oct 26.

Center for HUS prevention, control and management at the Pediatric Nephrology, Dialysis and Transplantation Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Center for HUS prevention, control and management at the Nephrology, Dialysis and Kidney Transplantation Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Hematology and Bone Marrow Transplantation Unit, IRCCS S. Raffaele Scientific Institution, Milan Oncology and Hemato-Oncology dept. University of Milan, BTM Center, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Nephrology and Dialysis Unit, IRCSS Arcispedale S. Maria Nuova, Reggio Emilia Center for HUS prevention, control and management at the Pediatric Nephrology, Dialysis and transplantation Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Center for HUS prevention, control and management at the Kidney Transplant Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Dipartimento di Scienze Cliniche e di Comunità Università degli Studi di Milano Center for HUS prevention, control and management at the Pediatric Nephrology, Dialysis and transplantation Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan UOC Coordinamento trapianto (NITp), Dip. Servizi e Medicina Preventiva, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Center for HUS prevention, control and management at the Laboratory of Medical Genetics, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Center for HUS prevention, control and management at the Internal Medicine, University of Milan, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan Hematology and Bone Marrow Transplantation Unit, IRCCS S. Raffaele Scientific Institution, Milan.

Background: Atypical hemolytic uremic syndrome (aHUS) is life-threatening condition particularly when complicating allograft hematopoietic stem cell transplant (HSCT). In the past, the outcome was very poor with the majority of patients reaching end stage renal disease (ESRD) or dying with little or no chances of kidney transplant (KTx) due to the high risk of relapse. The availability of C5 inhibition has opened up significant therapeutic opportunities and has improved the outcome particularly if complement dysregulation (CD) is the underlying pathogenetic mechanism. Read More

Front Microbiol 2018 11;9:2373. Epub 2018 Oct 11.

Peripheral Unit of Basic and Clinical Research in Infectious Diseases, Bacterial Pathogenicity Laboratory, Hemato Oncology and Research Unit, Department of Public Health/Research Division Medicine Faculty, Children's Hospital of Mexico Federico Gómez, National Autonomous University of Mexico, Mexico City, Mexico.

Intestinal infections represent an important public health concern worldwide. is one of the main bacterial agents involved in the pathogenesis of different diseases. In 2011, an outbreak of hemorrhagic colitis (HC) and hemolytic uremic syndrome (HUS) in Germany was related to a non-O157 STEC strain of O104:H4 serotype. Read More

Pediatr Nephrol 2018 Oct 25. Epub 2018 Oct 25.

Pediatric Nephrology and Dialysis Unit, Pediatric Hospital "Giovanni XXIII", Bari, Italy.

Background: Thrombotic microangiopathy (TMA) is a clinical syndrome encompassing a large group of rare but severe disorders including thrombotic thrombocytopenic purpura (TTP) and both typical and atypical forms of hemolytic uremic syndrome (HUS). The key role of the complement system is well known in TTP and atypical HUS, but recent reports describe its involvement in the pathogenesis of HUS secondary to gastrointestinal infections due to Shiga toxin-producing Escherichia coli (STEC).

Methods: TMA mainly affects the kidney, but extra-renal complications are frequently described. Read More

Microorganisms 2018 Oct 23;6(4). Epub 2018 Oct 23.

Laboratorio de Fisiopatogenia, Instituto de Fisiología y Biofísica Bernardo Houssay (IFIBIO Houssay-CONICET), Departamento de Fisiología, Facultad de Medicina, Universidad de Buenos Aires, Paraguay 2155, Buenos Aires 1121, Argentina.

Gastrointestinal infection with Shiga toxin-producing (STEC) causes diarrhea, hemorrhagic colitis, and hemolytic uremic syndrome (HUS), characterized by hemolytic anemia, thrombocytopenia and acute renal failure. The main virulence factor of STEC is Shiga toxin (Stx), which is responsible for HUS development. STEC can produce Stx type 1 and/or 2 (Stx1, Stx2) and their variants, Stx2 being more frequently associated with severe cases of HUS. Read More

Surg Infect (Larchmt) 2018 Oct 25. Epub 2018 Oct 25.

Department of Surgery, Stanford University , Stanford, California.

Background: Intussusception is the process by which one segment of intestine "telescopes" into another segment. Escherichia coli O157:H7 is a rare cause of intussusception that uncommonly requires a surgical procedure.

Methods: Case report and literature review. Read More

Hematol Rep 2018 Sep 24;10(3):7553. Epub 2018 Sep 24.

Department of Nephrology, Bozyaka Teaching and Research Hospital, Izmir, Turkey.

Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulatıon of the complement activation results in thrombotic microangiopathy and multiorgan damage. A 29-yearold woman who was admitted with complaints of vomiting and headache was detected to have acute renal failure with microangiopathic hemolytic anemia (MAHA). Read More

J Nephrol 2018 Dec 17;31(6):919-924. Epub 2018 Oct 17.

Department of Internal Medicine, Ente Ospedaliero Cantonale, Mendrisio, Switzerland.

Background: Intestinal infections caused by a shigatoxin-positive Escherichia coli (mostly of the serogroups O26, O45, O103, O111, O121, O145 and especially O157) are a common cause of hemolytic-uremic syndrome. Hemolytic-uremic syndrome was first linked with an E. coli urinary tract infection 40 years ago. Read More

Ther Apher Dial 2018 Oct 15. Epub 2018 Oct 15.

Department of Pediatrics, Pediatric Nephrology, Ghent University Hospital, Ghent, Belgium.

Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS) is associated with significant mortality and morbidity. Case fatalities are often associated with severe neurological involvement in children and advanced age in adults but specific treatment is currently unavailable. Plasma exchange (PE) could theoretically enable removal of Shiga toxins, pro-inflammatory cytokines, and prothrombotic factors and has been used in deteriorating patients with STEC-HUS but the efficacy remains uncertain. Read More

Cureus 2018 Aug 2;10(8):e3088. Epub 2018 Aug 2.

Internal Medicine, Florida Hospital, Orlando, USA.

Hemolytic uremic syndrome (HUS) is the triad of nonimmune (Coombs negative) hemolytic anemia, low platelet count, and renal impairment. HUS has been associated with a variety of gastrointestinal malignancies and chemotherapeutic agents. We present a patient with pancreatic cancer treated with gemcitabine for palliation who developed gemcitabine-induced HUS (GiHUS) which responded to some extent to blood and platelet transfusions. Read More

Pediatr Nephrol 2018 Oct 12. Epub 2018 Oct 12.

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, People's Republic of China.

Objective: Atypical hemolytic uremic syndrome (aHUS) is associated with defective complement regulation. Anti-complement factor H (CFH) antibodies were thought to participate in the pathogenesis of aHUS. The aim of this study was to address the functions and properties of CFH autoantibodies in a Chinese Han cohort of aHUS patients. Read More

Int J Med Microbiol 2018 Dec 4;308(8):1036-1042. Epub 2018 Oct 4.

Laboratorio de Neurofisiopatología, Instituto de Fisiología y Biofisica "Houssay"-CONICET-UBA, Facultad de Medicina, Universidad de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina. Electronic address:

Infections by Enterohemorrhagic Escherichia coli may cause in addition to hemolytic uremic syndrome neurological disorders which may lead to fatal outcomes in patients. The brain striatum is usually affected during this outcome. The aim of this study was to determine in this area the role of the microglia in pro-inflammatory events that may occur during Shiga toxin 2 intoxication and consequently to this, whether oligodendrocytes were being affected. Read More

Foodborne Pathog Dis 2018 Oct 10. Epub 2018 Oct 10.

Eastern Regional Research Center , Agricultural Research Service, U.S. Department of Agriculture, Wyndmoor, Pennsylvania.

There are reports in the literature stating that Campylobacter infections can cause hemolytic uremic syndrome (HUS); however, a mechanism for how Campylobacter induces HUS has not been proposed by investigators. The most common bacterial inducer of HUS is the Shiga toxin-producing Escherichia coli (STEC), and a few cases of HUS are induced by an invasive Shigella dysenteriae or Streptococcus pneumoniae infection. Campylobacter spp. Read More

Nephrol Dial Transplant 2018 Mar 21. Epub 2018 Mar 21.

Department of Medicine, Division of Nephrology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Background: Alport syndrome (AS) and atypical hemolytic-uremic syndrome (aHUS) are rare forms of chronic kidney disease (CKD) that can lead to a severe decline of renal function. Steroid-resistant nephrotic syndrome (SRNS) is more common than AS and aHUS and causes 10% of childhood-onset CKD. In recent years, multiple monogenic causes of AS, aHUS and SRNS have been identified, but their relative prevalence has yet to be studied together in a typical pediatric cohort of children with proteinuria and hematuria. Read More

Ther Apher Dial 2018 Oct 8. Epub 2018 Oct 8.

Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. Read More

Mayo Clin Proc 2018 Oct;93(10):1351-1362

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN. Electronic address:

Objective: To investigate the clinical utility of a 9-analyte complement serology panel (COMS) covering complement function (CH50 and AH50), components (C3, C4), factor B (CFB), factor H, and activation markers (C4d, Bb, and soluble membrane attack complex) for the diagnosis of atypical hemolytic uremic syndrome (aHUS).

Methods: Physician orders for COMS from January 19, 2015, through November 4, 2016, were reviewed. Demographic characteristics, patient diagnosis, and laboratory parameters were recorded. Read More

Mayo Clin Proc 2018 Oct;93(10):1337-1339

Division of Nephrology and Hypertension, University of Colorado Denver, Aurora, CO. Electronic address: