Síndrome hemolítico-urêmica (português brasileiro) ou síndrome hemolítica-urémica (português europeu) (SHU) é uma síndrome caracterizada por insuficiência renal progressiva, anemia hemolítica e lesão das paredes dos vasos sanguíneos. É causada pela verotoxina produzida por uma infecção bacteriana.
Introdução
O que você precisa saber de cara
Doença rara caracterizada por anemia hemolítica microangiopática, trombocitopenia e lesão renal aguda. Frequentemente associada a anormalidades no sistema complemento e metabolismo, com manifestações como proteinúria e hematúria.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 15 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 43 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
8 genes identificados com associação a esta condição.
Trypsin-like serine protease that plays an essential role in regulating the immune response by controlling all complement pathways. Inhibits these pathways by cleaving three peptide bonds in the alpha-chain of C3b and two bonds in the alpha-chain of C4b thereby inactivating these proteins (PubMed:17320177, PubMed:7360115). Essential cofactors for these reactions include factor H and C4BP in the fluid phase and membrane cofactor protein/CD46 and CR1 on cell surfaces (PubMed:12055245, PubMed:21418
Secreted, extracellular spaceSecreted
Hemolytic uremic syndrome, atypical, 3
An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.
Probable chaperone required for the generation of 1 O-glycan Gal-beta1-3GalNAc-alpha1-Ser/Thr (T antigen), which is a precursor for many extended O-glycans in glycoproteins. Probably acts as a specific molecular chaperone assisting the folding/stability of core 1 beta-3-galactosyltransferase (C1GALT1)
Membrane
Tn polyagglutination syndrome
A clonal disorder characterized by the polyagglutination of red blood cells by naturally occurring anti-Tn antibodies following exposure of the Tn antigen on the surface of erythrocytes. Only a subset of red cells express the antigen, which can also be expressed on platelets and leukocytes. This condition may occur in healthy individuals who manifest asymptomatic anemia, leukopenia, or thrombocytopenia. However, there is also an association between the Tn antigen and leukemia or myelodysplastic disorders.
Endothelial cell receptor that plays a critical role in regulating several physiological processes including hemostasis, coagulation, fibrinolysis, inflammation, and angiogenesis (PubMed:10761923). Acts as a cofactor for thrombin activation of protein C/PROC on the surface of vascular endothelial cells leading to initiation of the activated protein C anticoagulant pathway (PubMed:29323190, PubMed:33836597, PubMed:9395524). Also accelerates the activation of the plasma carboxypeptidase B2/CPB2, w
Membrane
Thrombophilia due to thrombomodulin defect
A hemostatic disorder characterized by a tendency to thrombosis.
Precursor of non-enzymatic components of the classical, alternative, lectin and GZMK complement pathways, which consist in a cascade of proteins that leads to phagocytosis and breakdown of pathogens and signaling that strengthens the adaptive immune system Non-enzymatic component of C5 convertase (PubMed:28264884, PubMed:31507604, PubMed:3653927, PubMed:3897448). Generated following cleavage by C3 convertase, it covalently attaches to the surface of pathogens, where it acts as an opsonin that ma
SecretedCell surface
Complement component 3 deficiency
A rare defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis.
Acts as a cofactor for complement factor I, a serine protease which protects autologous cells against complement-mediated injury by cleaving C3b and C4b deposited on host tissue. May be involved in the fusion of the spermatozoa with the oocyte during fertilization. Also acts as a costimulatory factor for T-cells which induces the differentiation of CD4+ into T-regulatory 1 cells. T-regulatory 1 cells suppress immune responses by secreting interleukin-10, and therefore are thought to prevent auto
Cytoplasmic vesicle, secretory vesicle, acrosome inner membrane
Hemolytic uremic syndrome, atypical, 2
An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.
Membrane-bound diacylglycerol kinase that converts diacylglycerol/DAG into phosphatidic acid/phosphatidate/PA and regulates the respective levels of these two bioactive lipids (PubMed:15544348, PubMed:19744926, PubMed:21477596, PubMed:22108654, PubMed:23949095). Thereby, acts as a central switch between the signaling pathways activated by these second messengers with different cellular targets and opposite effects in numerous biological processes (PubMed:15544348, PubMed:8626589). Also plays an
MembraneCytoplasm
Nephrotic syndrome 7
A form of nephrotic syndrome, a renal disease clinically characterized by severe proteinuria, resulting in complications such as hypoalbuminemia, hyperlipidemia and edema. NPHS7 is an autosomal recessive form characterized by onset of proteinuria usually in the first decade of life. The disorder is progressive, and some patients develop end-stage renal disease within several years. Renal biopsy typically shows membranoproliferative glomerulonephritis.
Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces (PubMed:21285368, PubMed:21317894, PubMed:25402769). Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of
Secreted
Basal laminar drusen
Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.
Precursor of the catalytic component of the C3 and C5 convertase complexes of the alternative pathway of the complement system, a cascade of proteins that leads to phagocytosis and breakdown of pathogens and signaling that strengthens the adaptive immune system (PubMed:3638964, PubMed:624565, PubMed:6554279, PubMed:6919543, PubMed:9748277). The alternative complement pathway acts as an amplification loop that enhances other complement pathways (classical, lectin and GZMK) by promoting formation
SecretedCell surface
Macular degeneration, age-related, 14
A form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
Medicamentos e terapias
Mecanismo: Complement C5 inhibitor
Mecanismo: Mannan-binding lectin serine protease 2 inhibitor
Mecanismo: Complement factor B inhibitor
Mecanismo: Complement C5 inhibitor
Mecanismo: Complement C5 inhibitor
Mecanismo: Complement C5 mRNA RNAi inhibitor
Mecanismo: C5a anaphylatoxin chemotactic receptor antagonist
Variantes genéticas (ClinVar)
447 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
15 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome hemolítico urêmico genético
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
[Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].
Objective: To evaluate the efficacy and safety of eculizumab (ECZ) in pediatric atypical hemolytic uremic syndrome (aHUS). Methods: A single-center retrospective study was conducted on 11 children with aHUS who received ECZ therapy in the Department of Nephrology, Children's Hospital of Fudan University, between December 2022 and February 2025. The clinical data, including genetic testing results, were collected for analyzing hematological and renal remission, prognosis and adverse events. Results: Among the 11 patients, 6 were male and 5 were female, with age at onset of 2.83 (0.55, 5.75) years. Eight patients had newly diagnosed aHUS, and 3 had relapsed disease. The pathogenic genetic variants were identified in 3 patients. Before ECZ treatment, 8 children required dialysis, including 2 with chronic dialysis. The age at first ECZ administration was 5.03 (0.72, 11.39) years, and the follow-up duration was (15±9) months. All 11 patients achieved complete thrombotic microangiopathy remission, with a time to remission of (36±23) d. In the 11 patients, the estimated glomerular filtration rate at the final follow-up increased by 46 (31, 62) ml/(min·1.73 m2) compared with baseline. The time to achieve normalization of serum creatinine or a ≥25% reduction was 10.0 (5.5, 31.5) d. Plasma exchange or plasma infusion was discontinued in all patients. The 8 patients who required dialysis at baseline successfully discontinued dialysis, and no new dialysis cases occurred. Six patients with negative genetic testing discontinued ECZ without relapse during follow-up. ECZ was well tolerated, with no meningococcal infections reported. Two patients with elevated liver enzymes recovered fully. Conclusions: ECZ demonstrates favorable efficacy and tolerability in pediatric aHUS. The application of ECZ may contribute to improve the prognosis of aHUS. 目的： 探讨依库珠单抗（ECZ）治疗儿童非典型溶血尿毒综合征（aHUS）的疗效和安全性。 方法： 病例系列研究，收集2022年12月至2025年2月在复旦大学附属儿科医院肾内科应用ECZ治疗的11例aHUS患儿临床资料包括基因诊断，分析其血液学、肾脏缓解情况以及预后和不良反应。 结果： 11例患儿中，男6例、女5例，起病年龄2.83（0.55，5.75）岁；8例为新发病例，3例为复发病例；3例检出aHUS相关基因变异。ECZ治疗前有8例患儿需行透析治疗，其中2例为长期透析患儿。首剂ECZ治疗年龄为5.03（0.72，11.39）岁，随访时间（15±9）个月。ECZ治疗后，11例患儿均达到血栓性微血管病完全缓解，达到时间（36±23）d。11例患儿末次随访估算肾小球滤过率较基线上升了46（31，62）ml/（min·1.73 m2）；达到血肌酐正常或下降≥25%的时间为10.0（5.5，31.5）d。所有患儿均停止血浆置换或血浆输注；基线时需要透析的8例患儿均成功停止透析，无新增透析患儿。6例基因检测阴性患儿已停用ECZ，无复发病例。ECZ耐受性良好，没有发生脑膜炎球菌感染；2例肝酶升高患儿均恢复正常。 结论： ECZ对儿童aHUS具有良好的疗效和耐受性。应用ECZ有助于改善aHUS预后。.
Cellular signaling pathway of Shiga toxin-induced ATP release.
Shiga toxin (Stx) is the main virulence factor of enterohemorrhagic Escherichia coli, a food-borne pathogen that colonizes the intestine causing gastroenteritis and, in severe cases, hemolytic uremic syndrome. Stx was shown to induce ATP release in vivo and in vitro and blockade of purinergic P2X receptors inhibited its cytotoxicity. Here we investigated the intracellular signaling events preceding ATP release. Inhibitors included pertussis toxin, wortmannin, manoalide, 2-aminoethoxydiphenylborate (2-APB), BAPTA-AM and Ca2+-free medium. The inositol 1,4,5-triphosphate receptor (IP3R) was silenced. Stx-induced apoptosis was detected by caspase 3/7 activation. BALB/c mice were injected with Stx2 i.p. Certain mice were pretreated with alpelisib (1 h before and 24 h after Stx2). Kidneys collected after 4 days were stained for phosphatidylinositol 4,5-bisphosphate (PIP2). Stx1-mediated ATP release in HeLa cells was blocked by pertussis toxin affecting the Gi/o family of G-protein coupled receptors. ATP release was also abrogated by wortmannin, an inhibitor of phosphoinositide 3-kinase (PI3K), by manoalide, inhibiting phospholipase C, by 2-APB inhibiting IP3R, and by reduction of intracellular calcium (BAPTA-AM) and extracellular calcium (Ca2+-free medium). Blocking or silencing the IP3R protected HeLa cells from Stx1-induced apoptosis. Likewise, blocking the IP3R reduced Stx2-induced apoptosis. Stx2-challenged mice had distinct PIP2 glomerular staining that decreased in the presence of the PI3K inhibitor alpelisib. Stx interaction with HeLa cells initiates a signaling pathway involving membrane G protein, PI3K, phospholipase C and IP3R, ultimately leading to ATP release and promoting cytotoxic effects. The PI3K inhibitor alpelisib altered PIP2 expression in Stx2-challenged mice.
Engineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.
Enterohemorrhagic Escherichia coli (EHEC) is a severe foodborne pathogen that can lead to hemolytic uremic syndrome. However, antibiotics are contraindicated for EHEC treatment due to toxin release and gut microbiota disruption. Here we report a dual‑mechanism therapeutic strategy combining an engineered Escherichia coli Nissle 1917 strain (EcN3) with 2'‑fucosyllactose (2‑FL) delivered via multicompartment microspheres (MCMs). EcN3 expresses α‑L‑fucosidase to hydrolyze 2‑FL into lactose and fucose. Lactose enhances glucuronic acid utilization, limiting a preferred nutrient of EHEC, whereas fucose activates FusKR signaling to suppress virulence gene expression. MCMs confer gastric protection and enable targeted colonic release, ensuring coordinated activity. In female mouse models and infant rabbit models of Citrobacter rodentium and EHEC infection, this system reduces intestinal colonization, virulence gene expression and epithelial damage without inducing Shiga toxin production. Moreover, MCMs-based strategy preserves the relative abundance of Lactobacillus, and promotes intestinal integrity. This targeted strategy presents a viable alternative to antibiotics, addressing EHEC pathogenesis and antibiotic resistance.
Developing a simple, cost-effective proof-of-concept vaccine candidate against EHEC for cattle.
Enterohemorrhagic Escherichia coli (EHEC) is a zoonotic pathogen responsible for severe human diseases, including hemorrhagic diarrhea and hemolytic uremic syndrome (HUS), primarily mediated by Shiga toxins (Stx). Intestinal colonization depends on the type III secretion system (T3SS), which induces attaching and effacing (A/E) lesions. Cattle are the main reservoir, and preharvest vaccines are key to reducing human exposure. In this work, we engineered a chimeric antigen EIT (EspA36-192-Intimin653-935-Tir258-361) to be expressed in the periplasm of E. coli BL21, enabling simple extraction by thermal shock. To favor scalable production, the process avoids the use of antibiotics, chemical inducers, and mechanical disruption, while remaining compatible with standard infrastructure and low-cost adjuvants. Immunization of mice with an EIT-enriched periplasmic fraction (EIT-PF) induced strong antibody responses with enhanced functional activity against A/E pedestal formation in vitro, and accelerated clearance of experimental E. coli O157 infection in mice. A three-dose EIT-PF immunization was tested in cattle. Notably, a single EIT-PF dose elicited antibodies that recognized multiple EHEC serotypes and EPEC, as well as each chimera component, effectively interfering with T3SS-dependent pedestal formation in vitro. Our findings support EIT-PF as a broadly reactive, proof-of-concept vaccine candidate for cattle; further studies are required to assess efficacy under field conditions.
Treatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are diseases driven by dysregulation of the alternative pathway (AP) complement. Current treatments of these diseases involve the use of frequent and often large doses of monoclonal antibodies, peptide inhibitors, or chemical inhibitors of complement proteins, with various limitations. In this study, we describe ectopic expression of factor D (FD) in the liver as a potential novel therapy for these indications. Unlike most plasma complement proteins, which are synthesized in the liver, FD, a serine protease in the AP, is synthesized primarily in fat tissues. Newly synthesized FD is released largely as an inactive proenzyme that requires activation by mannose-binding lectin-associated serine protease 3 (MASP3) to form the mature enzyme. We found serendipitously that ectopic expression of mature FD, but not pro-FD, in the mouse liver led to C3-dependent depletion of plasma factor B (FB) and abolished AP complement activity. Coexpression of C3, FB, and mature FD in cultured Hepa1-6 cells caused intracellular FB activation and consumption, probably within the secretory vesicles where all 3 proteins may colocalize. Hepatic FD-mediated local FB activation and depletion effectively prevented disease development in murine models of aHUS and C3G. Our results suggest that segregated tissue production of FD, FB, and C3, as well as FD regulation by MASP3, is necessary to prevent in situ FB activation and depletion. Ectopic expression of mature FD in the liver is an effective way to inhibit FB expression and AP complement activity, with potential therapeutic applications in diseases such as aHUS and C3G.
Publicações recentes
Clinical Characteristics of 30 Cases of Childhood Haemolytic Uremic Syndrome in a Single Centre.
Nationwide outbreak of Shiga toxin-producing Escherichia coli infections associated with frozen pizzas, France, 2022.
Pathogenic potential and phylogenomic analysis of stx2-carrying O26:H11 Shiga toxin-producing Escherichia coli isolated from dairy products in France (2014-2024).
Successful living kidney transplantation in a T-cell flow cytometry crossmatch-positive patient with atypical hemolytic uremic syndrome treated with an anti-C5 antibody: a case report.
Complement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.
📚 EuropePMCmostrando 199
Hemoglobinuria-associated acute kidney injury in hemolytic uremic syndrome without renal thrombotic microangiopathy.
BMC nephrologyComprehensive gene profiling by Next-Generation sequencing in a cohort of Egyptian pediatric Atypical HUS.
Journal, genetic engineering & biotechnologyRecurrent Atypical Hemolytic-Uremic Syndrome (aHUS) Associated With CD46 Genetic Mutation: A Report of a Rare Case.
Cureus[Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].
Zhonghua er ke za zhi = Chinese journal of pediatricsThrombotic microangiopathy after kidney transplantation: diagnosis and management strategies.
Clinical kidney journalThe C3 p.Ile1157Thr mutation associated with atypical hemolytic uremic syndrome, particularly in Japan, does not lead to disease development in several mouse models.
Journal of thrombosis and haemostasis : JTHCellular signaling pathway of Shiga toxin-induced ATP release.
Frontiers in cellular and infection microbiologyClinical Characteristics of 30 Cases of Childhood Haemolytic Uremic Syndrome in a Single Centre.
Nephrology (Carlton, Vic.)From post-infectious glomerulonephritis to complement-mediated aHUS: a diagnostic challenge.
Pediatric nephrology (Berlin, Germany)Nationwide outbreak of Shiga toxin-producing Escherichia coli infections associated with frozen pizzas, France, 2022.
Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletinPathogenic potential and phylogenomic analysis of stx2-carrying O26:H11 Shiga toxin-producing Escherichia coli isolated from dairy products in France (2014-2024).
Microbial genomicsAn unusual trilogy: a case of comorbid aHUS, Fabry disease, and hypertrophic cardiomyopathy.
Frontiers in medicineSuccessful living kidney transplantation in a T-cell flow cytometry crossmatch-positive patient with atypical hemolytic uremic syndrome treated with an anti-C5 antibody: a case report.
CEN case reportsC3 mutation-associated atypical hemolytic uremic syndrome with severe renal dysfunction and hypertensive emergency successfully treated with ravulizumab and sacubitril/valsartan: a case report.
BMC nephrologyC3 molecular structural and histopathological analyses in a pediatric case of atypical hemolytic uremic syndrome with life-threatening gastrointestinal bleeding-a case report.
Frontiers in pediatricsPractical Considerations for Infection Prevention with the Clinical Use of Complement Inhibitors.
Clinical journal of the American Society of Nephrology : CJASNEngineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.
Nature communicationsComplement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.
Frontiers in immunologySuccessful term pregnancy after renal transplant in end-stage renal disease with complement factor H-related mutation: A case report.
World journal of transplantationDeveloping a simple, cost-effective proof-of-concept vaccine candidate against EHEC for cattle.
PloS oneCFHR3*B Haplotype, Complement Activation, and Risk of IgA Nephropathy.
Journal of the American Society of Nephrology : JASNCase Report: Possible C3 nephritic factor-driven complement-mediated severe hemolytic anemia and acute kidney injury in a child with Bordetella parapertussis infection.
Frontiers in immunologyTreatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
Blood advancesRedefining Shiga toxin-induced human cell death as NLRP1- and gasdermin E-mediated pyroptosis.
Proceedings of the National Academy of Sciences of the United States of AmericaTherapeutic Antibodies in Hematology: Advances in Malignant and Non-Malignant Disorders.
CellsDiagnostic Challenges in Atypical Hemolytic Uremic Syndrome: A Case of Artificial Mitral Valve Dysfunction as a Possible Trigger for Severe Thrombotic Microangiopathy.
The American journal of case reportsCrovalimab Rescue Therapy in a Case With Genetic Complement Mediated Thrombotic Microangiopathy.
Kidney medicineAn outbreak of Shiga toxin-producing Escherichia coli O26:H11 associated with dried fruit, UK 2023.
Epidemiology and infectionIntegrated computational analysis for Escherichia coli prevalence, genetic evolution, and antimicrobial resistance evolution: Implications for public health and environmental sustainability in Asia.
Journal of infection and public healthO‑Island 28 encodes a type I secretion and RTX adhesion system regulated by RstA and required for early EHEC O157:H7 adherence.
Gut microbesClick Detect: A Rapid and Sensitive Assay for Shiga Toxin 2 Detection.
BiosensorsPost-miscarriage Complement-Mediated Thrombotic Microangiopathy in a 27-Year-Old Woman: A Case Highlighting Diagnostic and Therapeutic Gaps in Brazil.
CureusHypertriglyceridaemic pancreatitis associated with atypical haemolytic uraemic syndrome and secondary haemophagocytic lymphohistiocytosis: an immune system gone rogue.
BMJ case reportsCommentary: Complement genetic variants and FH desialylation in S. pneumoniae-haemolytic uraemic syndrome.
Frontiers in immunologyClinical and Radiographic Evaluation of Molar Root-Incisor Malformation (MRIM): A Case Series.
Case reports in dentistryLong-term outcome and management of complement-mediated thrombotic microangiopathy/aHUS.
Hematology. American Society of Hematology. Education ProgramUpdate in the diagnosis of complement-mediated thrombotic microangiopathy/atypical hemolytic uremic syndrome.
Hematology. American Society of Hematology. Education ProgramRise in the number of notifications of Shiga toxin-producing Escherichia coli (STEC) infections probably linked to an increased use of multiplex PCR assays, Germany, 2023.
Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletinTerminal complement inhibition in atypical haemolytic uremic syndrome: a single-centre experience.
Frontiers in pharmacologyNavigating Pediatric Atypical Hemolytic Uremic Syndrome: A Two-Year Case Series From Eastern India.
CureusSevere hypertension with thrombotic microangiopathy: the need for pathogenically targeted treatments.
Kidney internationalCase Report: Effective use of eculizumab in treating recurrent atypical HUS following renal transplantation triggered by SARS-CoV-2 infection.
Frontiers in medicine[Ultra-early administration of eculizumab in a child with atypical hemolytic uremic syndrome: a case report].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsGenetic and Functional Evaluation of the Complement System in De Novo Thrombotic Microangiopathy After Transplantation: A Single-Center Experience.
Clinical transplantationAtypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities.
Nagoya journal of medical sciencePathogenic cytokines in thrombotic microangiopathies: molecular insights and therapeutic targets.
Molecular medicine (Cambridge, Mass.)Clinical and genetic characteristics of patients diagnosed with atypical hemolytic uremic syndrome (aHUS): epidemiological data from the Belgian cohort of the Global aHUS Registry.
Journal of nephrologyHybrid pathotypes of Shiga toxin-producing Escherichia coli.
Journal of applied microbiologyA Complement Factor B Mutation in a Chinese Family with Atypical Hemolytic Uremic Syndrome: A Case Report and Systematic Review.
Case reports in nephrology and dialysisGlucose-6-Phosphate Dehydrogenase Deficiency Presenting as Atypical Hemolytic Uremic Syndrome: A Case Series and Literature Review.
Case reports in nephrologyWhole-exome sequencing in pediatric patients with glomerulonephritis.
Frontiers in geneticsTargeted optimization of single-chain variable fragment (scFv) expression in E. coli using a design-of-experiment approach.
International journal of biological macromoleculesCase Report: Life-threatening overlap of hemophagocytic syndrome and atypical hemolytic uremic syndrome in a patient with autoimmune polyglandular syndrome type 1 successfully treated with targeted immunotherapy.
Frontiers in immunologyIdentification of a new CD46 gene mutation site in a family with atypical hemolytic uremic syndrome.
BMC nephrologyC3 p.Asp1115Asn Variant-associated Atypical Hemolytic Uremic Syndrome with Spontaneous Remission Triggered by Influenza and COVID-19.
Internal medicine (Tokyo, Japan)Glycoproteomics analysis of complement factor H and its complement-regulatory function during Streptococcus pneumoniae-associated hemolytic uremic syndrome.
Frontiers in immunologyCytokine storm and microvascular fate: mechanistic insights into endothelial injury in thrombotic microangiopathies.
Annals of medicine and surgery (2012)High prevalence of the hotspot complement factor I p.Ile357Met pathogenic variant in Tunisian atypical hemolytic uremic syndrome patients: report of three new cases and review of the literature.
Frontiers in immunologyNeutralizing antibodies in the intestinal mucosa are essential to control gastrointestinal infection by Shiga toxin-producing Escherichia coli.
mLifeEffectiveness and Safety of Switching to Ravulizumab From Eculizumab in Kidney Transplant Recipients With Atypical Hemolytic Uremic Syndrome: A Global aHUS Registry Analysis.
Clinical transplantationCharacterization of the small RNAs carried by outer membrane vesicles produced by hlyF-positive Shiga toxin-producing Escherichia coli.
Frontiers in cellular and infection microbiologyBloody diarrhea, STEC infection, and HUS in the molecular microbiology era.
Pediatric nephrology (Berlin, Germany)Genetic variability of Shiga toxin-producing Escherichia coli strains isolated from Paraguayan cattle.
Microbiology spectrumShiga toxin-producing Escherichia coli O26:H11 associated with a cluster of haemolytic uraemic syndrome cases following the mid-July 2021 floods in Belgium.
Journal of infection and public healthDemographics and baseline disease characteristics of UK patients within the global aHUS registry.
BMC nephrologyHow I Treat: Genetic Testing in Atypical Hemolytic Uremic Syndrome.
Clinical journal of the American Society of Nephrology : CJASNA rare complication of thrombotic microangiopathy induced by chemotherapy for second breast cancer in a Hodgkin lymphoma survivor: a case report.
AME case reportsBilateral tractional retinal detachments complicating hemolytic-uremic syndrome.
American journal of ophthalmology case reportsGene-Environment Interaction: Lessons From Complement-Mediated Kidney Disease.
Seminars in nephrologyPotentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.
Journal of nephrologyCFH nonsense mutation-mediated pregnancy-associated atypical hemolytic uremic syndrome: Case report.
Molecular immunologyApproach to the prevention of atypical hemolytic uremic syndrome recurrence using eculizumab in a pregnant kidney transplant recipient with complement factor H gene mutation: a case report and literature review.
Journal of nephrologyPostpartum renal cortical necrosis: experience from a tertiary care center in India.
Journal of nephrologyC3d-targeted complement inhibitors to correct complement dysregulation in aHUS patients.
Frontiers in immunologyComplement activation in a phase Ib study of fordadistrogene movaparvovec for Duchenne muscular dystrophy.
Molecular therapy : the journal of the American Society of Gene TherapyAAV mini-dystrophin gene therapy for Duchenne muscular dystrophy: a phase 1b trial.
Nature medicineMolecular basis of thrombotic microangiopathy.
Journal of thrombosis and haemostasis : JTHEnteropathogenic Escherichia coli revisited - New insights into old EPEC isolates using whole genome sequencing.
International journal of medical microbiology : IJMMAtypical Hemolytic Uremic Syndrome/Complement-Mediated Thrombotic Microangiopathy Triggered by SARS-CoV-2 Infection: A Case Report.
Case reports in nephrology and dialysis[Diagnosis, treatment, and genetic analysis of five cases of primary atypical hemolytic uremic syndrome].
Zhonghua nei ke za zhiComprehensive genetic analysis and genotype-phenotype correlations in pediatric patients with atypical hemolytic uremic syndrome.
Pediatric nephrology (Berlin, Germany)Emerging hybrid shigatoxigenic and enteropathogenic Escherichia coli serotype O80:H2 in humans and calves.
Clinical microbiology reviewsEnterohemorrhagic Escherichia coli O157:H7 Infection Inhibits Host Endoplasmic Reticulum Stress in Intestinal Epithelial Cells via the PERK Pathway.
Pathogens (Basel, Switzerland)Hypertensive Emergency and Atypical Hemolytic Uremic Syndrome Associated with Cocaine Use: A Diagnostic and Therapeutic Challenge.
Diseases (Basel, Switzerland)Cryo-EM structures of engineered Shiga toxin-based immunogens capable of eliciting neutralizing antibodies with therapeutic potential against hemolytic uremic syndrome.
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Microbial cell factoriesComplement-Mediated Kidney Disease and Living Donor Transplantation: Tailoring Approaches to Improve Outcomes.
Current transplantation reportsMarginal zone lymphoma with anti-factor H IgM and atypical hemolytic uremic syndrome successfully treated with odronextamab.
HaematologicaShigella flexneri Outbreak at a Rehabilitation Center: First Report from Saudi Arabia.
Healthcare (Basel, Switzerland)The 4 functional segments of Factor H: Role in physiological target recognition and contribution to disease.
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Indian journal of nephrologyDesign of ELISA-based diagnostic system for detection of enterohaemorrhagic Escherichia coli.
Iranian journal of microbiologyComplement dysregulation at lymphatics.
The Journal of allergy and clinical immunologyCharacterization of Seven Shiga Toxin Phages Induced from Human-Derived Shiga Toxin-Producing Escherichia coli.
MicroorganismsIsolation of Shiga toxin-producing Escherichia coli O157 and non-O157 from retail imported frozen beef marketed in Saudi Arabia using immunomagnetic separation and multiplex PCR.
GermsColorimetric dual DNAzyme reaction triggered by loop-mediated isothermal amplification for the visual detection of Shiga toxin-producing Escherichia coli in food matrices.
PloS oneAtypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement.
Journal of clinical medicineMultidisciplinary consensus on the diagnosis and management of patients with atypical Hemolytic Uremic Syndrome (complement-mediated TMA): Recommendations from Italian scientific societies, patient associations and regulators.
Pharmacological researchPregnancy and delivery in the context of hemolytic uremic syndrome: A surrogacy case report.
International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and ObstetricsTen tips for managing complement-mediated thrombotic microangiopathies (formerly atypical hemolytic uremic syndrome): narrative review.
BMC nephrologyCase-Control Study of Factors Associated with Hemolytic Uremic Syndrome among Shiga Toxin-Producing Escherichia coli Patients, Ireland, 2017-2020.
Emerging infectious diseasesAutoantibodies and therapeutic antibodies against complement factor H.
Immunology lettersAtypical presentation of H1N1-induced thrombotic microangiopathy with CD46 gene mutation .
Clinical nephrology. Case studiesAtypical Hemolytic Uremic Syndrome Triggered by COVID-19 Infection in a Pediatric Patient With CD46 Mutation.
Clinical case reportsThe Critical Importance of Diagnosing Atypical Hemolytic Uremic Syndrome in Postpartum Renal Dysfunction in a Patient With Systemic Lupus Erythematosus: A Case Report and Comprehensive Review.
CureusGermline Genetic Variant Classification Requires More Equitable Reference Database Representation.
PediatricsA Case of Atypical Hemolytic Uremic Syndrome With a Complement Factor I Mutation Triggered by a Femoral Neck Fracture.
Nephrology (Carlton, Vic.)Development of intimin-enriched outer membrane vesicles (OMVs) as a vaccine to control intestinal carriage of Enterohemorrhagic Escherichia coli.
VaccineRecommendations for diagnosis and treatment of Atypical Hemolytic Uremic Syndrome (aHUS): an expert consensus statement from the Rare Diseases Committee of the Brazilian Society of Nephrology (COMDORA-SBN).
Jornal brasileiro de nefrologiaAtypical hemolytic uremic syndrome with a C3 variant following COVID-19: a case report.
Frontiers in pediatricsPost-transplant Thrombotic Microangiopathy.
Journal of the American Society of Nephrology : JASNComplement-Mediated Hemolytic Uremic Syndrome Due to MCP/CD46 Mutation: A Case Report.
Journal of investigative medicine high impact case reportsThe membrane attack complex drives thrombotic microangiopathy in complement mediated atypical hemolytic uremic syndrome.
Kidney internationalA compound heterozygous ADAMTS13 mutation causes congenital thrombotic thrombocytopenic purpura: a case report.
Frontiers in medicineThrombotic Microangiopathy Associated with Systemic Adeno-Associated Virus Gene Transfer: Review of Reported Cases.
Human gene therapyGenetic Assessment of Living Kidney Transplant Donors: A Survey of Canadian Practices.
Canadian journal of kidney health and diseaseA mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear.
Journal of thrombosis and haemostasis : JTHPhytochemicals Controlling Enterohemorrhagic Escherichia coli (EHEC) Virulence-Current Knowledge of Their Mechanisms of Action.
International journal of molecular sciencesThe role of the complement system in Shiga toxin-associated hemolytic uremic syndrome.
Pediatric nephrology (Berlin, Germany)CRISPR/Cas Systems as Diagnostic and Potential Therapeutic Tools for Enterohemorrhagic Escherichia coli.
Archivum immunologiae et therapiae experimentalisOutcomes in patients with thrombotic microangiopathy associated with a trigger following plasma exchange: A systematic literature review.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisReduction in Renal Heme Oxygenase-1 Is Associated with an Aggravation of Kidney Injury in Shiga Toxin-Induced Murine Hemolytic-Uremic Syndrome.
ToxinsSerogroups and Toxin Variants of Clinical Shiga Toxin-Producing Escherichia coli Strains Isolated from Patients with Hemolytic Uremic Syndrome in Turkey.
Foodborne pathogens and diseaseExtrarenal manifestations of atypical hemolytic uremic syndrome: a systematic review and meta-analysis.
Pediatric researchAtypical hemolytic uremic syndrome: diagnosis, management, and discontinuation of therapy.
Hematology. American Society of Hematology. Education ProgramCross-sectoral genomic surveillance reveals a lack of insight in sources of human infections with Shiga toxin-producing Escherichia coli, the Netherlands, 2017 to 2023.
Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletinDe Novo C3 Glomerulonephritis of Allograft Associated With Factor H Autoantibody in a Patient with Systemic Lupus Erythematosus: A Case Report.
Transplantation proceedingsShiga Toxins Produced by Enterohaemorrhagic Escherichia coli Induce Inflammation in Toxin-Sensitive Cells through the p38 MAPK/MK2/Tristetraprolin Signaling Pathway.
Journal of microbiology and biotechnologyBeyond the Norm: Gastroenteritis-Induced Atypical Hemolytic Uremic Syndrome in the Absence of Complement Dysfunction.
CureusRapid Enzymatic Detection of Shiga-Toxin-Producing E. coli Using Fluorescence-Labeled Oligonucleotide Substrates.
ACS infectious diseasesCharacterization of non-O157 enterohemorrhagic Escherichia coli isolated from different sources in Egypt.
BMC microbiology[What is proven in the treatment of complement-mediated kidney diseases?].
Innere Medizin (Heidelberg, Germany)Gb3 trisaccharide-bearing exosomes as a novel neutralizer for Shiga toxin type 1.
Biochemical and biophysical research communicationsAcute Myeloid Leukemia as a Trigger for Hemolytic-Uremic Syndrome.
Journal of clinical medicineDairy effluent management systems as a potential persistence source of Shiga toxin-producing Escherichia coli (STEC) strains.
Revista Argentina de microbiologiaExploring the Intersection of Atypical Hemolytic Uremic Syndrome and Substance Use: A Comprehensive Narrative Review.
CureusClinical Outcomes and Virulence Factors of Shiga Toxin-Producing Escherichia coli (STEC) from Southern Alberta, Canada, from 2020 to 2022.
Pathogens (Basel, Switzerland)Association of Atypical Hemolytic Uremic Syndrome With Wilms' Tumor 1 Gene Mutations: A Case Series and Literature Review.
CureusInvolvement of aquaporins in Shiga toxin-induced swelling and water transport dysfunction in human renal microvascular endothelial cells.
Biochimica et biophysica acta. Molecular cell researchThe Inhibitory Effects of a Factor B-Binding DNA Aptamer Family Supersede the Gain of Function of Factor B Variants Associated with Atypical Hemolytic Uremic Syndrome.
Journal of immunology (Baltimore, Md. : 1950)Characterization of Escherichia coli strains producing Shiga Toxin 2f subtype from domestic Pigeon.
Scientific reportsBone impairment in atypical hemolytic and uremic syndrome treated by long-term eculizumab.
Pediatric nephrology (Berlin, Germany)Outcomes from the International Society of Nephrology Hemolytic Uremic Syndromes International Forum.
Kidney internationalIndividualised therapeutic approach to the patient with atypical haemolytic-uraemic syndrome.
Clinical medicine (London, England)Shiga toxin-producing Escherichia coli infection as a precipitating factor for atypical hemolytic-uremic syndrome.
Pediatric nephrology (Berlin, Germany)The Role of the N-Terminal Domain of Thrombomodulin and the Potential of Recombinant Human Thrombomodulin as a Therapeutic Intervention for Shiga Toxin-Induced Hemolytic-Uremic Syndrome.
ToxinsGlobal aHUS Registry Analysis of Patients Switching to Ravulizumab From Eculizumab.
Kidney international reportsAtypical Hemolytic Uremic Syndrome Following Influenza B: A Case Report.
HCA healthcare journal of medicineLipopolysaccharide Core Truncation in Invasive Escherichia coli O157:H7 ATCC 43895 Impairs Flagella and Curli Biosynthesis and Reduces Cell Invasion Ability.
International journal of molecular sciencesEmerging Thrombotic Disorders Associated with Virus-Based Innovative Therapies: From VITT to AAV Gene Therapy-Related Thrombotic Microangiopathy.
Thrombosis and haemostasisPhenotypic and genomic comparison of three human outbreak and one cattle-associated Shiga toxin-producing Escherichia coli O157:H7.
Microbiology spectrumCorrigendum: Genetic investigation of Nordic patients with complement-mediated kidney diseases.
Frontiers in immunologyUnusual Presentation of Aggressive Atypical Hemolytic Uremic Syndrome With Brugada Syndrome.
CureusA Rare Case of Atypical Hemolytic Uremic Syndrome Presenting as Chronic Interstitial Nephritis.
CureusAn expert discussion on the atypical hemolytic uremic syndrome nomenclature-identifying a road map to precision: a report of a National Kidney Foundation Working Group.
Kidney internationalDiversity of Shiga toxin transducing phages in Escherichia coli O145:H28 and the different Shiga toxin 2 production levels associated with short- or long-tailed phages.
Frontiers in microbiologyCase Report: C3 deficiency in two siblings.
Frontiers in pediatricsThe efficacy and safety of eculizumab in patients and the role of C5 polymorphisms.
Drug discovery today[Clinical characteristics and genetic profile of complement system in renal thrombotic microangiopathy in patients with severe forms of arterial hypertension].
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Terapevticheskii arkhivRavulizumab in Atypical Hemolytic Uremic Syndrome: An Analysis of 2-Year Efficacy and Safety Outcomes in 2 Phase 3 Trials.
Kidney medicineProtein-losing enteropathy as a new phenotype in atypical hemolytic uremic syndrome caused by CD46 gene mutation.
Pediatric nephrology (Berlin, Germany)Living with Atypical Hemolytic Uremic Syndrome in the Netherlands: Patient and Family Perspective.
Kidney international reportsEx vivo C5b-9 Deposition Test to Monitor Complement Activity in Clinical and Subclinical Atypical Hemolytic Uremic Syndrome and in Transplantation-Associated Thrombotic Microangiopathy.
Kidney international reportsDeletion of Sphingosine Kinase 2 Attenuates Acute Kidney Injury in Mice with Hemolytic-Uremic Syndrome.
International journal of molecular sciencesMutations in Genes Encoding Subunits of the RNA Exosome as a Potential Novel Cause of Thrombotic Microangiopathy.
International journal of molecular sciencesRapid and Sensitive Detection of Shiga Toxin-Producing Escherichia coli (STEC) from Food Matrices Using the CANARY Biosensor Assay.
ToxinsA Rare Case of Atypical Hemolytic Uremic Syndrome (HUS) in an Adult Male: A Catastrophic Presentation.
CureusA Copernican revolution of multigenic analysis: A retrospective study on clinical exome sequencing in unclear genetic disorders.
Computational and structural biotechnology journalVirulence genes and pulsed-field gel electrophoresis profiles of Shiga toxin-producing Escherichia coli isolated from different food samples and patients with acute diarrhea.
Iranian journal of microbiologyCase presentation: a severe case of cobalamin c deficiency presenting with nephrotic syndrome, malignant hypertension and hemolytic anemia.
BMC nephrologyAtypical hemolytic-uremic syndrome after COVID-19 vaccine: A case report.
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CureusComprehensive functional characterization of complement factor I rare variant genotypes identified in the SCOPE geographic atrophy cohort.
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Frontiers in microbiologyMoss-produced human complement factor H with modified glycans has an extended half-life and improved biological activity.
Frontiers in immunologyTranscriptomic and proteomic analysis of the virulence inducing effect of ciprofloxacin on enterohemorrhagic Escherichia coli.
PloS oneA Rare Case of Atypical Hemolytic Uremic Syndrome (aHUS) Precipitated by Dengue and the Treatment Landscape in Singapore.
CureusGenetic Diagnosis of Adult Hemodialysis Patients With Unknown Etiology.
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Kidney international reportsRecurrent complement-mediated Hemolytic uremic syndrome after kidney transplantation.
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BMC nephrologyEpidemiological Characteristics of Shiga Toxin-Producing Escherichia coli Responsible for Infections in the Polish Pediatric Population.
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Communications biologyAdolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?
Clinical and experimental nephrologySelective bacteriophages reduce the emergence of resistant bacteria in bacteriophage-antibiotic combination therapy.
Microbiology spectrumCharacterization of patients with aHUS and associated triggers or clinical conditions: A Global aHUS Registry analysis.
Nephrology (Carlton, Vic.)Cobalamin C Deficiency: An Uncommon Cause of Hemolytic Uremic Syndrome.
Indian pediatricsDiagnostic Challenges and Emerging Pathogeneses of Selected Glomerulopathies.
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- [Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].
- Cellular signaling pathway of Shiga toxin-induced ATP release.
- Engineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.
- Developing a simple, cost-effective proof-of-concept vaccine candidate against EHEC for cattle.
- Treatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
- Clinical Characteristics of 30 Cases of Childhood Haemolytic Uremic Syndrome in a Single Centre.
- Nationwide outbreak of Shiga toxin-producing Escherichia coli infections associated with frozen pizzas, France, 2022.
- Pathogenic potential and phylogenomic analysis of stx2-carrying O26:H11 Shiga toxin-producing Escherichia coli isolated from dairy products in France (2014-2024).
- Successful living kidney transplantation in a T-cell flow cytometry crossmatch-positive patient with atypical hemolytic uremic syndrome treated with an anti-C5 antibody: a case report.
- Complement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:576742(Orphanet)
- MONDO:0957097(MONDO)
- GARD:22330(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
