Um problema repentino nos rins, associado a um tipo de anemia onde os glóbulos vermelhos são destruídos nos vasinhos (pequenos vasos sanguíneos), e com plaquetas baixas.
Introdução
O que você precisa saber de cara
A síndrome hemolítico-urêmica é um problema repentino nos rins associado à destruição de glóbulos vermelhos nos pequenos vasos sanguíneos e à queda de plaquetas no sangue. A pessoa pode apresentar sinais como diarreia sanguinolenta, vômitos, palidez, inchaço pelo corpo e diminuição perceptível da quantidade de urina. O quadro pode envolver fatores genéticos ligados ao sistema complemento ou ser desencadeado por quadros infecciosos. No Brasil, a condição não consta na lista de PCDTs de doenças raras do Ministério da Saúde e não tem medicamentos dispensados pelo SUS catalogados, sendo o cuidado conduzido pela rede hospitalar e de referência.
Um problema repentino nos rins, associado a um tipo de anemia onde os glóbulos vermelhos são destruídos nos vasinhos (pequenos vasos sanguíneos), e com plaquetas baixas.
O que está sendo pesquisado
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 49 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 103 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.
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.
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.
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.
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.
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.
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.
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.
Thrombophilia due to thrombomodulin defect
A hemostatic disorder characterized by a tendency to thrombosis.
Medicamentos e terapias
Mecanismo: Complement C5 inhibitor
Mecanismo: Complement C5 inhibitor
Mecanismo: Mannan-binding lectin serine protease 2 inhibitor
Mecanismo: Complement factor B inhibitor
Mecanismo: Complement C5 inhibitor
Mecanismo: B-lymphocyte antigen CD20 binding agent
Mecanismo: Complement C5 mRNA RNAi inhibitor
Mecanismo: C5a anaphylatoxin chemotactic receptor antagonist
Variantes genéticas (ClinVar)
431 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 2.247 variantes classificadas pelo 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
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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
Ensaios em destaque
Com ensaio aberto em 23 países. O ponto verde marca onde há vaga agora.
🟢 Recrutando agora
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Outros ensaios clínicos
65 ensaios clínicos encontrados, 19 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 3.001
Circuit Survival in Children with Hemolytic Uremic Syndrome Requiring Continuous Renal Replacement Therapy.
Hemolytic uremic syndrome (HUS) is a significant cause of acute kidney injury (AKI) in children that is caused by Shiga toxin-producing Escherichia coli (STEC). Continuous renal replacement therapy (CRRT) is frequently used for managing AKI in critically ill pediatric patients. The filter lifespan is a crucial factor affecting its effectiveness and cost. This study aimed to investigate the impact of HUS on the lifespan of CRRT filters compared with that of other etiologies. This retrospective longitudinal study was conducted in a pediatric intensive care unit (PICU) in Nancy, France, from January 2013 to December 2023. The study included 54 children requiring CRRT, with 388 filters (202 in the HUS group and 186 in the other etiologies group). Data on filter lifespan and various parameters were collected and analyzed according to disease (HUS group or other etiologies) and the type of anticoagulation used. The median lifespan of the filters in the HUS group was shorter than that in the other etiologies group (13.3 hours vs. 17.6 hours, P=0.05). This study revealed no significant difference in filter lifespan between regional citrate anticoagulation (RCA) and systemic unfractionated heparin (UFH) in pediatric patients with typical HUS (P=1.00). The prothrombotic nature of HUS, characterized by platelet activation, may contribute to the observed decrease in lifespan. The mechanism of coagulation activation in HUS and its interaction with hemofiltration, including the effects of platelets and blood product transfusions, remain to be explored. The lifespan of the filters is shorter in patients with HUS than in those with other etiologies, regardless of the type of anticoagulation used.
[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.
Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.
Shiga toxin-producing Escherichia coli (STEC) infections have significant morbidity, with 15% to 20% of children developing hemolytic uremic syndrome (HUS), more than half of whom require kidney replacement therapy (KRT). To describe health care resource utilization, clinical outcomes, and outcomes associated with daily laboratory monitoring for thrombotic microangiopathy (TMA) in a large point-source STEC outbreak. This retrospective cohort study was conducted among a population-based cohort of individuals exposed to a contaminated food item served in 11 childcare centers in Calgary, Alberta, Canada, on August 29, 2023. Children and adults with confirmed STEC infection linked to the outbreak were included. Data were analyzed from June 9, 2025, to January 5, 2026. E coli O157:H7 possessing genes encoding Shiga toxins 1 and 2. Health care utilization, symptoms and signs of infection, occurrence of HUS and KRT, and results of TMA screening. There were 359 outbreak-linked confirmed STEC infections, including 285 children (median [IQR] age, 3.3 [2.3-4.2] years; 141 [62.4%] male) and 71 adults (median [IQR] age, 38.8 [31.9-46.4] years; 58 [81.7%] female) and 3 out-of-province cases; 326 primary and 33 secondary infections were identified. Of 359 infected individuals overall, 66 (18.4%) were asymptomatic. HUS occurred in 21 children (7.4%), all of whom had diarrhea (21 of 206 children with diarrhea [10.2%]). No adults developed HUS. This outbreak was linked to 508 and 395 visits by children with STEC to an emergency department and a dedicated STEC clinic, respectively; 591 visits occurred during a 7-day period, starting 6 days after exposure. Forty children were hospitalized, 9 children required KRT, and 3 children required intensive care. The most common symptoms in children and adults were diarrhea (206 children [91.2%]; 16 of 20 adults with data [80.0%]), abdominal pain (141 children [62.4%]; 11 of 20 adults with data [55.0%]), and bloody diarrhea (127 children [56.2%]; 7 of 20 adults with data [35.0%]). TMA testing had 100% (95% CI, 83.9%-100%) sensitivity, 95.7% (95% CI, 92.0%-98.0%) specificity, and a diagnostic accuracy of 96.1% (95% CI, 92.7%-98.2%) for development of HUS. This cohort study reporting on a point-source STEC outbreak identified significant use of health care resources. Only 7% of children infected with STEC developed HUS, which is lower than reported in most prior outbreaks and sporadic case series. These findings highlight the importance of a coordinated public health response integrated with clinical care and suggest that proactive screening for TMA has the potential to mitigate adverse outcomes.
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.
Publicações recentes
Virulent phages MM-1 and MM-2 attack enterohemorrhagic Escherichia coli and Salmonella enterica strains.
Case Report: COVID-19 unmasks factor H mutation-driven hemolytic uremic syndrome in a previously undiagnosed septuagenarian kidney transplant recipient.
A case report on the effective and safe use of ravulizumab in atypical hemolytic uremic syndrome during pregnancy.
Hemolytic Uremic Syndrome Outbreak in Adults and Shiga Toxin-Producing Escherichia coli Negative for Locus of Enterocyte Effacement, France, 2025.
[Clinical analysis of eculizumab in the treatment of atypical hemolytic uremic syndrome in children].
📚 EuropePMC3.905 artigos no totalmostrando 198
Early recognition of atypical hemolytic uremic syndrome to prevent irreversible kidney injury: cardiac failure and refractory hypertension as critical clues in young patients.
CEN case reportsRecurrent pancreatitis and atypical hemolytic uremic syndrome (aHUS): an unusual presentation in childhood.
Pediatric nephrology (Berlin, Germany)Erythrodermic psoriasis complicated by immune complex-mediated crescentic glomerulonephritis and atypical hemolytic uremic syndrome: a case report.
BMC nephrologyReversible cerebral lesions in adult STEC-associated hemolytic uremic syndrome.
Kidney internationalCircuit Survival in Children with Hemolytic Uremic Syndrome Requiring Continuous Renal Replacement Therapy.
Blood purificationHemoglobinuria-associated acute kidney injury in hemolytic uremic syndrome without renal thrombotic microangiopathy.
BMC nephrologyIschemic colitis complicated by Shiga toxin-producing Escherichia coli hemolytic uremic syndrome.
Pediatrics international : official journal of the Japan Pediatric SocietyComprehensive gene profiling by Next-Generation sequencing in a cohort of Egyptian pediatric Atypical HUS.
Journal, genetic engineering & biotechnologyComparison between effectiveness and safety of plasma therapy and complement inhibitors for the treatment of atypical hemolytic uremic syndrome: a systematic review and meta-analysis of real-world data.
Pediatric nephrology (Berlin, Germany)Recurrent 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 pediatricsAnalysis of adverse drug reactions associated with ravulizumab: a retrospective pharmacovigilance study utilizing the FAERS database.
Frontiers in immunologyThrombotic 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 microbiologyShiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.
JAMA network openEliglustat prevents acute kidney injury caused by Shiga toxin 2 in lethal and sublethal rat models of hemolytic uremic syndrome.
Frontiers in pharmacologyClinical 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)Hematologic disorders in pregnancy: the role of the complement system.
Expert review of hematologyClinical and cost-effectiveness of eculizumab withdrawal in atypical haemolytic uraemic syndrome: the SETS aHUS multi-centre, open-label, prospective and single-arm study.
Health technology assessment (Winchester, England)Eculizumab Successfully Rescues Against de novo Atypical Hemolytic Uremic Syndrome Following Retransplantation.
Transplantation proceedingsSerology as an early diagnostic tool in pediatric patients with Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome: a post hoc analysis of a phase 2 clinical trial.
Journal of clinical microbiologyNationwide 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 bulletinAntibody Screening and Binding Prediction Analysis Targeting Stx2.
Antibodies (Basel, Switzerland)Pathogenic potential and phylogenomic analysis of stx2-carrying O26:H11 Shiga toxin-producing Escherichia coli isolated from dairy products in France (2014-2024).
Microbial genomicsNot So Normal Skin: A Review of the "Random" Skin Biopsy.
The American Journal of dermatopathologyAn 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 nephrologyA 'complement' problem: Complement-mediated hemolytic uremic syndrome triggered by Klebsiella pneumoniae liver abscess.
The American journal of medicineC3 molecular structural and histopathological analyses in a pediatric case of atypical hemolytic uremic syndrome with life-threatening gastrointestinal bleeding-a case report.
Frontiers in pediatricsActinomycotic Cholecystitis and Pancreatitis: Report of an Unusual Case.
The American journal of case reportsShiga Toxin 1a Blunts Shiga Toxin 2a-pathogenic Effects in Blood.
Thrombosis and haemostasisPractical Considerations for Infection Prevention with the Clinical Use of Complement Inhibitors.
Clinical journal of the American Society of Nephrology : CJASNHemolytic uremic syndrome without diarrhea in an infant due to non-O157 Shiga toxin-producing enterohemorrhagic Escherichia coli.
Pediatrics international : official journal of the Japan Pediatric SocietyDelayed-Onset Hemolysis in a Case of Hemolytic Uremic Syndrome: A Diagnostic Challenge.
Case reports in hematologyRpoS impacts global gene expression and carbon source utilization in Escherichia coli O104:H4.
Frontiers in microbiologyClinical value of serum and urine endocan in children with hemolytic uremic syndrome.
The Turkish journal of pediatricsEfficacy of Plasma Exchange in Secondary Thrombotic Microangiopathy: A Case Report and Literature Review.
CureusEngineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.
Nature communicationsAcute kidney injury requiring temporary hemodialysis due to HELLP syndrome: a case report.
CEN case reportsReal-World Effectiveness of Ravulizumab Among C5 Inhibitor-Naive Patients With Atypical Hemolytic Uremic Syndrome: A Physician Panel-Based Chart Review (aHUS IMPACT Study).
Kidney medicinePrevalence of Escherichia coli 0157: H7 in chicken livers.
Open veterinary journalA Case of Severe Pre-Eclampsia Complicated by HELLP Syndrome and Extensive Hepatic Infarction.
Clinical case reportsComplement-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 transplantationFrom dog bite to dialysis: complement-mediated haemolytic uraemic syndrome .
BMJ case reportsEpidemiology of Bloody Diarrhea, Shiga Toxin-producing Escherichia coli and Hemolytic Uremic Syndrome in Children.
The Pediatric infectious disease journalDeveloping a simple, cost-effective proof-of-concept vaccine candidate against EHEC for cattle.
PloS oneSalvaging sepsis-associated atypical hemolytic uremic syndrome with terminal complement blockade: A case report.
Medicine[Microangiopathies thrombotiques].
La Revue du praticienTailoring Eculizumab Treatment: Evaluation of Model-Informed Precision Dosing for Eculizumab in Patients with Paroxysmal Nocturnal Hemoglobinuria and Atypical Hemolytic Uremic Syndrome.
Therapeutic drug monitoringComplement Alternative Pathway Dominance in Atypical Hemolytic Uremic Syndrome Revealed by Endothelial Bioassays.
Kidney international reportsCFHR3*B Haplotype, Complement Activation, and Risk of IgA Nephropathy.
Journal of the American Society of Nephrology : JASNAcute kidney injury due to diarrhoeal diseases in children: a systematic review.
BMJ paediatrics openCase Report: Possible C3 nephritic factor-driven complement-mediated severe hemolytic anemia and acute kidney injury in a child with Bordetella parapertussis infection.
Frontiers in immunologyAntimeningococcal Protection in Patients Receiving Terminal Complement Inhibitors.
Kidney international reportsTreatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
Blood advancesFrom uterine atony to aHUS: A case of postpartum thrombotic microangiopathy.
Revista espanola de anestesiologia y reanimacionRedefining 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 AmericaEvaluation of ravulizumab trough levels in pediatric complement-mediated hemolytic uremic syndrome in remission.
Pediatric nephrology (Berlin, Germany)Improving hemolytic uremic syndrome diagnosis in children: the role of serology for Shiga toxin-producing Escherichia coli detection.
Pediatric nephrology (Berlin, Germany)Therapeutic Antibodies in Hematology: Advances in Malignant and Non-Malignant Disorders.
CellsDiscovery of (3R,4R)-15: An Advanced Factor B Inhibitor Entering Phase 3 for Complement-Mediated Diseases.
Journal of medicinal chemistryShiga Toxin Induces Apoptosis via ROS-Caspase Activation in Human Cerebral Endothelial Cell Line hCMEC/D3 and Astrocyte Co-Culture.
Journal of microbiology and biotechnologyDiagnostic 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 infectionPetting Zoo Perils: Escherichia coli O157:h7 Associated Hemolytic Uremic Syndrome Secondary to Animal Exposure.
Hemodialysis international. International Symposium on Home HemodialysisIntegrated 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 healthDeleterious consequences of Shiga toxin in the CNS.
Microbiology and molecular biology reviews : MMBR[Targeting complement in atypical hemolytic and uremic syndrome: development and current status of eculizumab and ravulizumab].
Nephrologie & therapeutiqueLateral geniculate body hemorrhage in pediatric hemolytic uremic syndrome.
Kidney internationalAtypical Hemolytic Uremic Syndrome After Post-Abortion Infection: Case Report and Literature Review.
International journal of women's healthRenal Autotransplantation After Yang-Monti Neoureter Procedure: Surgical Case Report and Brief Literature Review.
Case reports in urologyLiver Involvement in Children with Hemolytic Uremic Syndrome: Clinical Significance and Prognostic Value.
Children (Basel, Switzerland)Modern challenges in infection prevention: encapsulated organisms in the era of novel complement inhibitors.
Kidney internationalO‑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.
BiosensorsBilateral root absence of permanent first molars: literature review and case report.
European journal of paediatric dentistryPost-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 reportsThe Patient-Reported Outcomes Measurement Information System perspective of adults with long-standing atypical hemolytic uremic syndrome.
Research and practice in thrombosis and haemostasisHemolytic Uremic Syndrome Complicated by Severe Neuropsychiatric Symptoms: A Case Report and Review of the Literature.
Cureus[The night has many faces: When schistocytes step out of line].
PraxisRavulizumab in treatment-naïve patients with atypical hemolytic uremic syndrome: a real-world case series.
Drug target insightsEnterohemorrhagic Escherichia coli O157 outer membrane vesicles administered by oral gavage cause renal tubular injury and acute kidney failure in mice.
Frontiers in cellular and infection microbiologyLate Onset Thrombotic Microangiopathy in Kidney Transplants; Poor Outcome Despite Eculizumab Treatment.
Transplant international : official journal of the European Society for Organ TransplantationIncidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study.
Pediatric nephrology (Berlin, Germany)Commentary: 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 dentistryThrombotic Microangiopathy During Pregnancy: Role of Soluble Fms-like Tyrosine Kinase-1-Placental Growth Factor Ratios.
American journal of kidney diseases : the official journal of the National Kidney FoundationLong-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 pharmacologySuccessful treatment of adult atypical haemolytic uraemic syndrome with multi-organ involvement: a case report.
Hong Kong medical journal = Xianggang yi xue za zhiImmunodeficiency and hemolytic uremic syndrome: a case report.
The Turkish journal of pediatricsPseudotumor Cerebri and Hemolytic Uremic Syndrome, A Rare Association.
Clinical case reportsNavigating Pediatric Atypical Hemolytic Uremic Syndrome: A Two-Year Case Series From Eastern India.
CureusTherapeutic Plasma Exchange in Pediatric Care: Insights from a Tertiary Care Centre Experience.
Indian pediatricsEvaluation of Hypertension in Pediatric Hemolytic Uremic Syndrome (A Cross-Sectional Study).
Health science reportsAntifactor H Autoantibody Characterization in Atypical Hemolytic Uremic Syndrome.
Kidney international reportsShiga Toxin-Producing Escherichia coli-Associated Hemolytic Uremic Syndrome in Adult Kidney Transplant Recipients.
Kidney international reportsOcular manifestations of primary thrombotic microangiopathies : a descriptive systematic review.
BMC ophthalmologySevere 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 pediatricsDiagnostic oriented discrimination of different Shiga toxins via PCA-assisted SERS-based plasmonic metasurface.
Nanophotonics (Berlin, Germany)Multidisciplinary management of concurrent postpartum eclampsia, HELLP syndrome, ruptured hepatic subcapsular hematoma, and atypical hemolytic uremic syndrome: a case report.
BMC pregnancy and childbirthMalignant Hypertension-Induced Thrombotic Microangiopathy Mimicking Atypical Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura.
JACC. Case reportsSpectrum and outcomes of thrombotic microangiopathies in nephrology: a 17-year cohort from a North African center.
BMC nephrologySpatially and Multivalent Matched Neutralizing Aptamer-Based DNA Nanostructure Alleviates Shiga Toxin Type 2 Infection.
Analytical chemistryGenetic and Functional Evaluation of the Complement System in De Novo Thrombotic Microangiopathy After Transplantation: A Single-Center Experience.
Clinical transplantationAdvancing Medical Diagnostics: Rapid, Label-Free Detection and Differentiation of Shiga Toxin Variants in Human Serum Using a Cost-Effective PCA-Assisted SERS Platform.
ACS applied materials & interfacesRenal Histology Findings in Malignant Hypertension, a Systematic Review.
Hypertension (Dallas, Tex. : 1979)Sustained remission with eculizumab in refractory lupus nephritis with atypical haemolytic uraemic syndrome.
BMJ case reportsComplement-targeting therapies in hemolytic diseases.
Current opinion in immunologyCharacteristics and clinical courses of patients with atypical haemolytic uraemic syndrome on dialysis withdrawal after eculizumab treatment: sub-analysis of post-marketing surveillance in Japan.
Journal of nephrologyPresumed successful steroid therapy for suspected thrombotic microangiopathy in a dog.
The Canadian veterinary journal = La revue veterinaire canadienneAtypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities.
Nagoya journal of medical scienceA rare case of hemolytic-uremic syndrome due to a Capnocytophaga canimorsus sepsis: Case report.
MedicineIptacopan in C5 blockade refractory atypical hemolytic uremic syndrome with associated Castleman's disease: case report.
BMC nephrologyPathogenic cytokines in thrombotic microangiopathies: molecular insights and therapeutic targets.
Molecular medicine (Cambridge, Mass.)Unmasking hidden shapes: unusual red cell morphologies in urine sediment.
Clinica chimica acta; international journal of clinical chemistryComparing German medical guidelines with recommendations by large language models using the example of hemolytic uremic syndrome (TMA).
Pediatric nephrology (Berlin, Germany)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 nephrologyExtracorporeal Membrane Oxygenation and Eculizumab for Cardiac Failure in STEC-HUS.
PediatricsHybrid pathotypes of Shiga toxin-producing Escherichia coli.
Journal of applied microbiologyAtypical hemolytic uremic syndrome associated with pregnancy: A case report.
Medicine internationalPericardial effusion in pediatric Shiga toxin-producing E. coli hemolytic uremic syndrome: experience from an Argentinean center.
Pediatric nephrology (Berlin, Germany)Successful Management of Oxacillin-Induced Eosinophilic Myocarditis and Review of the Literature.
JACC. Case reportsA Complement Factor B Mutation in a Chinese Family with Atypical Hemolytic Uremic Syndrome: A Case Report and Systematic Review.
Case reports in nephrology and dialysisCalcific Changes in an End-Stage Kidney following Long-Term Dialysis, Tertiary Hyperparathyroidism, and Treatment for Complement-Mediated Thrombotic Microangiopathy: A Case Report.
Glomerular diseasesGlucose-6-Phosphate Dehydrogenase Deficiency Presenting as Atypical Hemolytic Uremic Syndrome: A Case Series and Literature Review.
Case reports in nephrologyStructural characterization of Factor H via computational methods: Implications for disease and therapy.
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapieMicroscopic vision of mouse kidney experimentally infected with Escherichia coli O157:H7 to determine the therapeutic effect by Trimethoprim-Sulfamethoxazole compared to Ciprofloxacin.
Open veterinary journalInvestigating enterohemorrhagic E. coli (O157:H7) in live sheep from Central Jordan: Prevalence and antibiotic sensitivity analysis.
Open veterinary journalEarly Identification and Eculizumab Treatment of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report.
CureusWhole-exome sequencing in pediatric patients with glomerulonephritis.
Frontiers in geneticsHemolytic Uremic Syndrome Presenting With Acute Renal Failure in an Adult: A Case Report.
CureusReference Range for the Automated Fragmented Red Cell Parameter and Its Diagnostic Utility in Red Blood Cell (RBC) Fragment Quantification: A Prospective Study.
Cureus[Exploring critical thinking in the management of diagnosis and treatment of fulminant pregnancy-associated atypical haemolytic uraemic syndrome].
Zhonghua wei zhong bing ji jiu yi xueTargeted optimization of single-chain variable fragment (scFv) expression in E. coli using a design-of-experiment approach.
International journal of biological macromoleculesThrombotic microangiopathy multidisciplinary assessment team: demographics, final diagnosis, treatment, and outcomes.
BMC nephrologyRelevance of the interactions between the complement and contact coagulation systems on renal pathology.
NefrologiaIptacopan/LNP023 and rituximab as rescue therapy in a patient with systemic lupus erythematosus-associated atypical haemolytic uraemic syndrome.
Journal of nephrologyCase 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 nephrologyFirst Pediatric Lung Transplant Using the Paragonix BAROguard Preservation System.
Pediatric transplantationPregnancy-Induced Atypical Hemolytic Uremic Syndrome.
CureusPregnancy-Related Acute Kidney Injury: Causes and Its Impact on Perinatal Outcomes-A Systematic Review.
Journal of clinical medicineWhich Factors Influence Decisions to Withdraw from Eculizumab: A Qualitative Study of Patients Diagnosed with aHUS.
The patientManagement of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.
Nephrology (Carlton, Vic.)C3 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 immunologyIs pericardial effusion restricted to STEC-HUS? Observations in anti-factor H associated atypical hemolytic uremic syndrome.
Pediatric nephrology (Berlin, Germany)Advanced Three-Dimensional Cell Culture Models for Investigating Enterohaemorrhagic Escherichia coli Pathogenesis and Shiga Toxin-Mediated Injury.
Journal of microbiology and biotechnologyShiga Toxin Type 2 Aggravates G1/S Phase Cell Cycle Arrest, Mediating Caspase-Independent Cell Death under Hyperosmotic Conditions in the Kidney.
Journal of microbiology and biotechnologyCytokine 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.
mLifeLong-term outcomes of volume expansion treatment in diarrhea-associated hemolytic uremic syndrome: a six-year follow-up.
Pediatric nephrology (Berlin, Germany)Effectiveness and Safety of Switching to Ravulizumab From Eculizumab in Kidney Transplant Recipients With Atypical Hemolytic Uremic Syndrome: A Global aHUS Registry Analysis.
Clinical transplantationA Case of Spontaneous Resolution: Atypical Hemolytic Uremic Syndrome in a 4-Year-Old Boy.
Pediatric blood & cancerIn Silico Design of a Multiepitope Vaccine Against Intestinal Pathogenic Escherichia coli Based on the 2011 German O104:H4 Outbreak Strain Using Reverse Vaccinology and an Immunoinformatic Approach.
Diseases (Basel, Switzerland)Characterization of the small RNAs carried by outer membrane vesicles produced by hlyF-positive Shiga toxin-producing Escherichia coli.
Frontiers in cellular and infection microbiologyCOVID-19 Microangiopathy: Insights into plasma exchange as a therapeutic strategy.
Hematology, transfusion and cell therapyAn ultrasensitive diagnostic system for minuscule level of hemolytic uremic syndrome.
Biosensors & bioelectronicsBloody diarrhea, STEC infection, and HUS in the molecular microbiology era.
Pediatric nephrology (Berlin, Germany)Cost-effectiveness of lifelong eculizumab versus disease monitoring of aHUS.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationShiga toxin-producing Escherichia coli, food contamination, and bacteriophages as a control strategy.
Microbiology and molecular biology reviews : MMBRA presentation of posterior reversible encephalopathy syndrome after heart transplantation: a case report and review of literature.
Journal of medical case reportsParoxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report.
Frontiers in medicineGenetic variability of Shiga toxin-producing Escherichia coli strains isolated from Paraguayan cattle.
Microbiology spectrumEfficacy of eculizumab discontinuation in atypical hemolytic uremic syndrome: a systematic review and meta-analysis.
Blood advancesThe Economic and Public Health Burden of Foodborne Illness in Somalia: Prevalence, Costs, and Policy Imperatives.
Public health challengesAdult Hemolytic Uremic Syndrome as a Diagnostic Challenge.
CureusA Case Report of Atypical Hemolytic Uremic Syndrome Presenting With Disseminated Intravascular Coagulation.
CureusShiga 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 reportsAn antibiotic derivative as a new potential tool in the prevention of hemolytic uremic syndrome.
iScienceTargeting the Roots of Kidney Disease: Systematic Review of the Therapies Targeting the Complement System.
Medicina (Kaunas, Lithuania)Influenza A H1N1 Triggering Complement-Mediated Hemolytic Uremic Syndrome.
Journal of medical casesNeurological Manifestations of Hemolytic Uremic Syndrome: A Comprehensive Review.
Brain sciencesHighly sensitive Escherichia coli detection via cell surface in situ cytocompatible ATRP.
Analytical and bioanalytical chemistryA Rare Case of Atypical Haemolytic Syndrome Following Right Retrograde Intrarenal Surgery (RIRS).
CureusPericardial effusion in pediatric Shiga toxin-producing E. coli hemolytic uremic syndrome: a French multicentre study.
Pediatric nephrology (Berlin, Germany)Household transmission and carriage of Shiga toxin-producing Escherichia coli (STEC) O145, Stx1c: a family report.
The Turkish journal of pediatricsShiga toxin-producing Escherichia coli illness in Aotearoa | New Zealand, 2016-2022: epidemiological, genomic and traditional typing analyses provide insight into a significant endemic disease while highlighting knowledge gaps.
Frontiers in microbiologyGene-Environment Interaction: Lessons From Complement-Mediated Kidney Disease.
Seminars in nephrologyThe epidemiology of haemolytic uraemic syndrome in England, 2009-2023: An analysis of national surveillance data.
The Journal of infectionThe Hideous Side of Acute Pancreatitis: A Case of Pancreatitis-Induced Atypical Hemolytic Uremic Syndrome.
CureusPotentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.
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É um problema repentino nos rins que ocorre junto com a destruição de glóbulos vermelhos nos vasos e contagem baixa de plaquetas. Ela é classificada na Orphanet sob o código ORPHA:544458 como um grupo de fenótipos.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Síndrome hemolítico-urêmico. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/sindrome-hemolitico-uremico
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Síndrome hemolítico-urêmico
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Moléculas estudadas na doença
- fonte: OpenTargets
- Ensaios clínicos
- fonte: ClinicalTrials.gov
