Raras
Buscar doenças, sintomas, genes...
Síndrome hemolítico-urêmico
ORPHA:544458CID-10 · D59.3PCDT · SUSDOENÇA RARA

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.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

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.

Pesquisas ativas
22 ensaios
73 total registrados no ClinicalTrials.gov
Publicações científicas
7.440 artigos
Último publicado: 2026 Apr 11
Medicamentos
8 registrados
RAVULIZUMAB, ECULIZUMAB, NARSOPLIMAB

Tem tratamento?

8 medicamentos registrados
Ver detalhes, fases e interações →
RAVULIZUMABECULIZUMABNARSOPLIMABIPTACOPANCROVALIMABRITUXIMABCEMDISIRANAVACOPAN
🏥
SUS: Cobertura mínimaScore: 30%
PCDT disponívelCID-10: D59.3
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
12 sintomas
🩸
Sangue
11 sintomas
🫘
Rins
8 sintomas
🧠
Neurológico
5 sintomas
😀
Face
4 sintomas
🛡️
Imunológico
3 sintomas

+ 49 sintomas em outras categorias

Características mais comuns

Hiponatremia
Hipocalemia
Desidratação
Reticulocitose
Edema generalizado
Crise hipertensiva
103sintomas
Sem dados (103)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 103 características clínicas mais associadas, ordenadas por frequência.

HiponatremiaHyponatremia
HipocalemiaHypokalemia
DesidrataçãoDehydration
ReticulocitoseReticulocytosis
Edema generalizadoGeneralized edema

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico7.440PubMed
Últimos 10 anos200publicações
Pico2025118 papers
Linha do tempo
2026Hoje · 2026🧪 1997Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

8 genes identificados com associação a esta condição.

C1GALT1C1C1GALT1-specific chaperone 1Disease-causing germline mutation(s) inDesconhecido
FUNÇÃO

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)

LOCALIZAÇÃO

Membrane

VIAS BIOLÓGICAS (1)
O-linked glycosylation of mucins
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (2)
Tn polyagglutination syndromehemolytic uremic syndrome, atypical, 8, with rhizomelic short stature
HGNC:HGNC:24338UniProt:Q96EU7
CFIComplement factor ICandidate gene tested inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

Secreted, extracellular spaceSecreted

VIAS BIOLÓGICAS (1)
Regulation of Complement cascade
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (6)
complement factor I deficiencyDoyne honeycomb retinal dystrophyatypical hemolytic uremic syndrome with complement gene abnormalityHELLP syndrome
HGNC:5394UniProt:P05156
CFBComplement factor BCandidate gene tested inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

SecretedCell surface

VIAS BIOLÓGICAS (1)
Alternative complement activation
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (4)
complement factor b deficiencyatypical hemolytic uremic syndrome with complement gene abnormalityage related macular degeneration 14atypical hemolytic-uremic syndrome with B factor anomaly
HGNC:1037UniProt:P00751
CFHComplement factor HCandidate gene tested inAltamente restrito
FUNÇÃO

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

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
Regulation of Complement cascade
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (10)
complement factor H deficiencybasal laminar drusendense deposit diseaseDoyne honeycomb retinal dystrophy
HGNC:4883UniProt:P08603
DGKEDiacylglycerol kinase epsilonCandidate gene tested inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

MembraneCytoplasm

VIAS BIOLÓGICAS (1)
Effects of PIP2 hydrolysis
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
20.3 TPM
Cerebelo
15.9 TPM
Útero
10.9 TPM
Pituitária
10.6 TPM
Cervix Ectocervix
10.4 TPM
OUTRAS DOENÇAS (2)
immunoglobulin-mediated membranoproliferative glomerulonephritisatypical hemolytic-uremic syndrome with DGKE deficiency
HGNC:2852UniProt:P52429
CD46Membrane cofactor proteinCandidate gene tested inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

Cytoplasmic vesicle, secretory vesicle, acrosome inner membrane

VIAS BIOLÓGICAS (1)
Regulation of Complement cascade
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (3)
HELLP syndromeatypical hemolytic uremic syndrome with complement gene abnormalityatypical hemolytic-uremic syndrome with MCP/CD46 anomaly
HGNC:6953UniProt:P15529
C3Complement C3Candidate gene tested inRestrito
FUNÇÃO

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

LOCALIZAÇÃO

SecretedCell surface

VIAS BIOLÓGICAS (2)
Post-translational protein phosphorylationRegulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (4)
complement component 3 deficiencyatypical hemolytic uremic syndrome with complement gene abnormalityatypical hemolytic-uremic syndrome with C3 anomalyage related macular degeneration 9
HGNC:1318UniProt:P01024
THBDThrombomodulinCandidate gene tested inDesconhecido
FUNÇÃO

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

LOCALIZAÇÃO

Membrane

VIAS BIOLÓGICAS (2)
Common Pathway of Fibrin Clot FormationCell surface interactions at the vascular wall
MECANISMO DE DOENÇA

Thrombophilia due to thrombomodulin defect

A hemostatic disorder characterized by a tendency to thrombosis.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Sun Exposed Lower leg
112.0 TPM
Pulmão
110.4 TPM
Skin Not Sun Exposed Suprapubic
108.2 TPM
Artéria coronária
91.3 TPM
Aorta
87.3 TPM
OUTRAS DOENÇAS (3)
thrombomodulin-related bleeding disorderatypical hemolytic uremic syndrome with complement gene abnormalityatypical hemolytic-uremic syndrome with thrombomodulin anomaly
HGNC:11784UniProt:P07204

Medicamentos e terapias

RAVULIZUMABPhase 4

Mecanismo: Complement C5 inhibitor

ECULIZUMABPhase 4

Mecanismo: Complement C5 inhibitor

NARSOPLIMABPhase 3

Mecanismo: Mannan-binding lectin serine protease 2 inhibitor

IPTACOPANPhase 3

Mecanismo: Complement factor B inhibitor

CROVALIMABPhase 3

Mecanismo: Complement C5 inhibitor

RITUXIMABPhase 2

Mecanismo: B-lymphocyte antigen CD20 binding agent

CEMDISIRANPhase 2

Mecanismo: Complement C5 mRNA RNAi inhibitor

AVACOPANPhase 2

Mecanismo: C5a anaphylatoxin chemotactic receptor antagonist

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

431 variantes patogênicas registradas no ClinVar.

🧬 C1GALT1C1: GRCh37/hg19 Xq23-28(chrX:113417246-155233731)x1 ()
🧬 C1GALT1C1: GRCh37/hg19 Xq13.1-27.1(chrX:71017904-140066710)x4 ()
🧬 C1GALT1C1: GRCh37/hg19 Xq24-26.2(chrX:118228490-133002510)x3 ()
🧬 C1GALT1C1: GRCh37/hg19 Xq21.32-26.1(chrX:92712119-129831493)x1 ()
🧬 C1GALT1C1: NM_001011551.3(C1GALT1C1):c.553G>A (p.Gly185Arg) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 2,247 variantes classificadas pelo ClinVar.

674
1236
337
Patogênica (30.0%)
VUS (55.0%)
Benigna (15.0%)
VARIANTES MAIS SIGNIFICATIVAS
CFH: NM_000186.4(CFH):c.10_11delinsG (p.Leu4fs) [Likely pathogenic]
CFH: NM_000186.4(CFH):c.2791_2792delinsCT (p.Cys931Leu) [Likely pathogenic]
CFI: NM_000204.5(CFI):c.796T>C (p.Cys266Arg) [Likely pathogenic]
CFH: NM_000186.4(CFH):c.629G>T (p.Cys210Phe) [Likely pathogenic]
CFH: NM_000186.4(CFH):c.3301C>T (p.Gln1101Ter) [Likely pathogenic]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado3
3Fase 310
2Fase 24
·Pré-clínico11
Medicamentos catalogadosEnsaios clínicos· 8 medicamentos · 20 ensaios
✓ Aprovados — podem ser usados hoje
RAVULIZUMABECULIZUMAB
Carregando informações de tratamento...

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.

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Ensaios clínicos abertos e novidades científicas recentes

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73 ensaios clínicos encontrados, 22 ativos.

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Publicações mais relevantes

Timeline de publicações
3.001 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 3.001

#1

Circuit Survival in Children with Hemolytic Uremic Syndrome Requiring Continuous Renal Replacement Therapy.

Blood purification2026 Mar 20

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.

#2

[Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].

Zhonghua er ke za zhi = Chinese journal of pediatrics2026 Mar 14

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。.

#3

Cellular signaling pathway of Shiga toxin-induced ATP release.

Frontiers in cellular and infection microbiology2026

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.

#4

Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.

JAMA network open2026 Mar 02

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.

#5

Engineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.

Nature communications2026 Feb 03

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.

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2026

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 reports
2026

Recurrent pancreatitis and atypical hemolytic uremic syndrome (aHUS): an unusual presentation in childhood.

Pediatric nephrology (Berlin, Germany)
2026

Erythrodermic psoriasis complicated by immune complex-mediated crescentic glomerulonephritis and atypical hemolytic uremic syndrome: a case report.

BMC nephrology
2026

Reversible cerebral lesions in adult STEC-associated hemolytic uremic syndrome.

Kidney international
2026

Circuit Survival in Children with Hemolytic Uremic Syndrome Requiring Continuous Renal Replacement Therapy.

Blood purification
2026

Hemoglobinuria-associated acute kidney injury in hemolytic uremic syndrome without renal thrombotic microangiopathy.

BMC nephrology
2026

Ischemic colitis complicated by Shiga toxin-producing Escherichia coli hemolytic uremic syndrome.

Pediatrics international : official journal of the Japan Pediatric Society
2026

Comprehensive gene profiling by Next-Generation sequencing in a cohort of Egyptian pediatric Atypical HUS.

Journal, genetic engineering & biotechnology
2026

Comparison 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)
2026

Recurrent Atypical Hemolytic-Uremic Syndrome (aHUS) Associated With CD46 Genetic Mutation: A Report of a Rare Case.

Cureus
2026

[Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2026

Analysis of adverse drug reactions associated with ravulizumab: a retrospective pharmacovigilance study utilizing the FAERS database.

Frontiers in immunology
2026

Thrombotic microangiopathy after kidney transplantation: diagnosis and management strategies.

Clinical kidney journal
2026

The 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 : JTH
2026

Cellular signaling pathway of Shiga toxin-induced ATP release.

Frontiers in cellular and infection microbiology
2026

Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.

JAMA network open
2026

Eliglustat prevents acute kidney injury caused by Shiga toxin 2 in lethal and sublethal rat models of hemolytic uremic syndrome.

Frontiers in pharmacology
2026

Clinical Characteristics of 30 Cases of Childhood Haemolytic Uremic Syndrome in a Single Centre.

Nephrology (Carlton, Vic.)
2026

From post-infectious glomerulonephritis to complement-mediated aHUS: a diagnostic challenge.

Pediatric nephrology (Berlin, Germany)
2026

Hematologic disorders in pregnancy: the role of the complement system.

Expert review of hematology
2026

Clinical 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)
2026

Eculizumab Successfully Rescues Against de novo Atypical Hemolytic Uremic Syndrome Following Retransplantation.

Transplantation proceedings
2026

Serology 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 microbiology
2026

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 bulletin
2026

Antibody Screening and Binding Prediction Analysis Targeting Stx2.

Antibodies (Basel, Switzerland)
2026

Pathogenic potential and phylogenomic analysis of stx2-carrying O26:H11 Shiga toxin-producing Escherichia coli isolated from dairy products in France (2014-2024).

Microbial genomics
2026

Not So Normal Skin: A Review of the "Random" Skin Biopsy.

The American Journal of dermatopathology
2026

An unusual trilogy: a case of comorbid aHUS, Fabry disease, and hypertrophic cardiomyopathy.

Frontiers in medicine
2026

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.

CEN case reports
2026

C3 mutation-associated atypical hemolytic uremic syndrome with severe renal dysfunction and hypertensive emergency successfully treated with ravulizumab and sacubitril/valsartan: a case report.

BMC nephrology
2026

A 'complement' problem: Complement-mediated hemolytic uremic syndrome triggered by Klebsiella pneumoniae liver abscess.

The American journal of medicine
2025

C3 molecular structural and histopathological analyses in a pediatric case of atypical hemolytic uremic syndrome with life-threatening gastrointestinal bleeding-a case report.

Frontiers in pediatrics
2026

Actinomycotic Cholecystitis and Pancreatitis: Report of an Unusual Case.

The American journal of case reports
2026

Shiga Toxin 1a Blunts Shiga Toxin 2a-pathogenic Effects in Blood.

Thrombosis and haemostasis
2026

Practical Considerations for Infection Prevention with the Clinical Use of Complement Inhibitors.

Clinical journal of the American Society of Nephrology : CJASN
2026

Hemolytic 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 Society
2026

Delayed-Onset Hemolysis in a Case of Hemolytic Uremic Syndrome: A Diagnostic Challenge.

Case reports in hematology
2025

RpoS impacts global gene expression and carbon source utilization in Escherichia coli O104:H4.

Frontiers in microbiology
2025

Clinical value of serum and urine endocan in children with hemolytic uremic syndrome.

The Turkish journal of pediatrics
2026

Efficacy of Plasma Exchange in Secondary Thrombotic Microangiopathy: A Case Report and Literature Review.

Cureus
2026

Engineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.

Nature communications
2026

Acute kidney injury requiring temporary hemodialysis due to HELLP syndrome: a case report.

CEN case reports
2026

Real-World Effectiveness of Ravulizumab Among C5 Inhibitor-Naive Patients With Atypical Hemolytic Uremic Syndrome: A Physician Panel-Based Chart Review (aHUS IMPACT Study).

Kidney medicine
2025

Prevalence of Escherichia coli 0157: H7 in chicken livers.

Open veterinary journal
2026

A Case of Severe Pre-Eclampsia Complicated by HELLP Syndrome and Extensive Hepatic Infarction.

Clinical case reports
2025

Complement-mediated HUS revisited: evolving insights into pathophysiology, diagnosis, and treatment.

Frontiers in immunology
2026

Successful term pregnancy after renal transplant in end-stage renal disease with complement factor H-related mutation: A case report.

World journal of transplantation
2026

From dog bite to dialysis: complement-mediated haemolytic uraemic syndrome .

BMJ case reports
2026

Epidemiology of Bloody Diarrhea, Shiga Toxin-producing Escherichia coli and Hemolytic Uremic Syndrome in Children.

The Pediatric infectious disease journal
2026

Developing a simple, cost-effective proof-of-concept vaccine candidate against EHEC for cattle.

PloS one
2026

Salvaging sepsis-associated atypical hemolytic uremic syndrome with terminal complement blockade: A case report.

Medicine
2025

[Microangiopathies thrombotiques].

La Revue du praticien
2026

Tailoring Eculizumab Treatment: Evaluation of Model-Informed Precision Dosing for Eculizumab in Patients with Paroxysmal Nocturnal Hemoglobinuria and Atypical Hemolytic Uremic Syndrome.

Therapeutic drug monitoring
2026

Complement Alternative Pathway Dominance in Atypical Hemolytic Uremic Syndrome Revealed by Endothelial Bioassays.

Kidney international reports
2025

CFHR3*B Haplotype, Complement Activation, and Risk of IgA Nephropathy.

Journal of the American Society of Nephrology : JASN
2026

Acute kidney injury due to diarrhoeal diseases in children: a systematic review.

BMJ paediatrics open
2025

Case Report: Possible C3 nephritic factor-driven complement-mediated severe hemolytic anemia and acute kidney injury in a child with Bordetella parapertussis infection.

Frontiers in immunology
2026

Antimeningococcal Protection in Patients Receiving Terminal Complement Inhibitors.

Kidney international reports
2026

Treatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.

Blood advances
2026

From uterine atony to aHUS: A case of postpartum thrombotic microangiopathy.

Revista espanola de anestesiologia y reanimacion
2026

Redefining 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 America
2026

Evaluation of ravulizumab trough levels in pediatric complement-mediated hemolytic uremic syndrome in remission.

Pediatric nephrology (Berlin, Germany)
2026

Improving hemolytic uremic syndrome diagnosis in children: the role of serology for Shiga toxin-producing Escherichia coli detection.

Pediatric nephrology (Berlin, Germany)
2025

Therapeutic Antibodies in Hematology: Advances in Malignant and Non-Malignant Disorders.

Cells
2026

Discovery of (3R,4R)-15: An Advanced Factor B Inhibitor Entering Phase 3 for Complement-Mediated Diseases.

Journal of medicinal chemistry
2026

Shiga Toxin Induces Apoptosis via ROS-Caspase Activation in Human Cerebral Endothelial Cell Line hCMEC/D3 and Astrocyte Co-Culture.

Journal of microbiology and biotechnology
2026

Diagnostic 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 reports
2026

Crovalimab Rescue Therapy in a Case With Genetic Complement Mediated Thrombotic Microangiopathy.

Kidney medicine
2026

An outbreak of Shiga toxin-producing Escherichia coli O26:H11 associated with dried fruit, UK 2023.

Epidemiology and infection
2026

Petting Zoo Perils: Escherichia coli O157:h7 Associated Hemolytic Uremic Syndrome Secondary to Animal Exposure.

Hemodialysis international. International Symposium on Home Hemodialysis
2026

Integrated 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 health
2026

Deleterious consequences of Shiga toxin in the CNS.

Microbiology and molecular biology reviews : MMBR
2026

[Targeting complement in atypical hemolytic and uremic syndrome: development and current status of eculizumab and ravulizumab].

Nephrologie & therapeutique
2026

Lateral geniculate body hemorrhage in pediatric hemolytic uremic syndrome.

Kidney international
2025

Atypical Hemolytic Uremic Syndrome After Post-Abortion Infection: Case Report and Literature Review.

International journal of women's health
2025

Renal Autotransplantation After Yang-Monti Neoureter Procedure: Surgical Case Report and Brief Literature Review.

Case reports in urology
2025

Liver Involvement in Children with Hemolytic Uremic Syndrome: Clinical Significance and Prognostic Value.

Children (Basel, Switzerland)
2026

Modern challenges in infection prevention: encapsulated organisms in the era of novel complement inhibitors.

Kidney international
2026

O‑Island 28 encodes a type I secretion and RTX adhesion system regulated by RstA and required for early EHEC O157:H7 adherence.

Gut microbes
2025

Click Detect: A Rapid and Sensitive Assay for Shiga Toxin 2 Detection.

Biosensors
2026

Bilateral root absence of permanent first molars: literature review and case report.

European journal of paediatric dentistry
2025

Post-miscarriage Complement-Mediated Thrombotic Microangiopathy in a 27-Year-Old Woman: A Case Highlighting Diagnostic and Therapeutic Gaps in Brazil.

Cureus
2025

Hypertriglyceridaemic pancreatitis associated with atypical haemolytic uraemic syndrome and secondary haemophagocytic lymphohistiocytosis: an immune system gone rogue.

BMJ case reports
2025

The Patient-Reported Outcomes Measurement Information System perspective of adults with long-standing atypical hemolytic uremic syndrome.

Research and practice in thrombosis and haemostasis
2025

Hemolytic Uremic Syndrome Complicated by Severe Neuropsychiatric Symptoms: A Case Report and Review of the Literature.

Cureus
2025

[The night has many faces: When schistocytes step out of line].

Praxis
2025

Ravulizumab in treatment-naïve patients with atypical hemolytic uremic syndrome: a real-world case series.

Drug target insights
2025

Enterohemorrhagic 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 microbiology
2025

Late Onset Thrombotic Microangiopathy in Kidney Transplants; Poor Outcome Despite Eculizumab Treatment.

Transplant international : official journal of the European Society for Organ Transplantation
2025

Incidence and severity of extrarenal manifestations and outcomes in pediatric hemolytic uremic syndrome: a retrospective cohort study.

Pediatric nephrology (Berlin, Germany)
2025

Commentary: Complement genetic variants and FH desialylation in S. pneumoniae-haemolytic uraemic syndrome.

Frontiers in immunology
2025

Clinical and Radiographic Evaluation of Molar Root-Incisor Malformation (MRIM): A Case Series.

Case reports in dentistry
2026

Thrombotic 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 Foundation
2025

Long-term outcome and management of complement-mediated thrombotic microangiopathy/aHUS.

Hematology. American Society of Hematology. Education Program
2025

Update in the diagnosis of complement-mediated thrombotic microangiopathy/atypical hemolytic uremic syndrome.

Hematology. American Society of Hematology. Education Program
2025

Rise 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 bulletin
2025

Terminal complement inhibition in atypical haemolytic uremic syndrome: a single-centre experience.

Frontiers in pharmacology
2025

Successful treatment of adult atypical haemolytic uraemic syndrome with multi-organ involvement: a case report.

Hong Kong medical journal = Xianggang yi xue za zhi
2025

Immunodeficiency and hemolytic uremic syndrome: a case report.

The Turkish journal of pediatrics
2025

Pseudotumor Cerebri and Hemolytic Uremic Syndrome, A Rare Association.

Clinical case reports
2025

Navigating Pediatric Atypical Hemolytic Uremic Syndrome: A Two-Year Case Series From Eastern India.

Cureus
2025

Therapeutic Plasma Exchange in Pediatric Care: Insights from a Tertiary Care Centre Experience.

Indian pediatrics
2025

Evaluation of Hypertension in Pediatric Hemolytic Uremic Syndrome (A Cross-Sectional Study).

Health science reports
2025

Antifactor H Autoantibody Characterization in Atypical Hemolytic Uremic Syndrome.

Kidney international reports
2025

Shiga Toxin-Producing Escherichia coli-Associated Hemolytic Uremic Syndrome in Adult Kidney Transplant Recipients.

Kidney international reports
2025

Ocular manifestations of primary thrombotic microangiopathies : a descriptive systematic review.

BMC ophthalmology
2026

Severe hypertension with thrombotic microangiopathy: the need for pathogenically targeted treatments.

Kidney international
2025

Case Report: Effective use of eculizumab in treating recurrent atypical HUS following renal transplantation triggered by SARS-CoV-2 infection.

Frontiers in medicine
2025

[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 pediatrics
2025

Diagnostic oriented discrimination of different Shiga toxins via PCA-assisted SERS-based plasmonic metasurface.

Nanophotonics (Berlin, Germany)
2025

Multidisciplinary management of concurrent postpartum eclampsia, HELLP syndrome, ruptured hepatic subcapsular hematoma, and atypical hemolytic uremic syndrome: a case report.

BMC pregnancy and childbirth
2026

Malignant Hypertension-Induced Thrombotic Microangiopathy Mimicking Atypical Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura.

JACC. Case reports
2025

Spectrum and outcomes of thrombotic microangiopathies in nephrology: a 17-year cohort from a North African center.

BMC nephrology
2025

Spatially and Multivalent Matched Neutralizing Aptamer-Based DNA Nanostructure Alleviates Shiga Toxin Type 2 Infection.

Analytical chemistry
2025

Genetic and Functional Evaluation of the Complement System in De Novo Thrombotic Microangiopathy After Transplantation: A Single-Center Experience.

Clinical transplantation
2025

Advancing 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 & interfaces
2026

Renal Histology Findings in Malignant Hypertension, a Systematic Review.

Hypertension (Dallas, Tex. : 1979)
2025

Sustained remission with eculizumab in refractory lupus nephritis with atypical haemolytic uraemic syndrome.

BMJ case reports
2026

Complement-targeting therapies in hemolytic diseases.

Current opinion in immunology
2025

Characteristics 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 nephrology
2025

Presumed successful steroid therapy for suspected thrombotic microangiopathy in a dog.

The Canadian veterinary journal = La revue veterinaire canadienne
2025

Atypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities.

Nagoya journal of medical science
2025

A rare case of hemolytic-uremic syndrome due to a Capnocytophaga canimorsus sepsis: Case report.

Medicine
2025

Iptacopan in C5 blockade refractory atypical hemolytic uremic syndrome with associated Castleman's disease: case report.

BMC nephrology
2025

Pathogenic cytokines in thrombotic microangiopathies: molecular insights and therapeutic targets.

Molecular medicine (Cambridge, Mass.)
2026

Unmasking hidden shapes: unusual red cell morphologies in urine sediment.

Clinica chimica acta; international journal of clinical chemistry
2026

Comparing German medical guidelines with recommendations by large language models using the example of hemolytic uremic syndrome (TMA).

Pediatric nephrology (Berlin, Germany)
2025

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 nephrology
2025

Extracorporeal Membrane Oxygenation and Eculizumab for Cardiac Failure in STEC-HUS.

Pediatrics
2025

Hybrid pathotypes of Shiga toxin-producing Escherichia coli.

Journal of applied microbiology
2025

Atypical hemolytic uremic syndrome associated with pregnancy: A case report.

Medicine international
2026

Pericardial effusion in pediatric Shiga toxin-producing E. coli hemolytic uremic syndrome: experience from an Argentinean center.

Pediatric nephrology (Berlin, Germany)
2025

Successful Management of Oxacillin-Induced Eosinophilic Myocarditis and Review of the Literature.

JACC. Case reports
2025

A Complement Factor B Mutation in a Chinese Family with Atypical Hemolytic Uremic Syndrome: A Case Report and Systematic Review.

Case reports in nephrology and dialysis
2025

Calcific Changes in an End-Stage Kidney following Long-Term Dialysis, Tertiary Hyperparathyroidism, and Treatment for Complement-Mediated Thrombotic Microangiopathy: A Case Report.

Glomerular diseases
2025

Glucose-6-Phosphate Dehydrogenase Deficiency Presenting as Atypical Hemolytic Uremic Syndrome: A Case Series and Literature Review.

Case reports in nephrology
2025

Structural characterization of Factor H via computational methods: Implications for disease and therapy.

Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie
2025

Microscopic vision of mouse kidney experimentally infected with Escherichia coli O157:H7 to determine the therapeutic effect by Trimethoprim-Sulfamethoxazole compared to Ciprofloxacin.

Open veterinary journal
2025

Investigating enterohemorrhagic E. coli (O157:H7) in live sheep from Central Jordan: Prevalence and antibiotic sensitivity analysis.

Open veterinary journal
2025

Early Identification and Eculizumab Treatment of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report.

Cureus
2025

Whole-exome sequencing in pediatric patients with glomerulonephritis.

Frontiers in genetics
2025

Hemolytic Uremic Syndrome Presenting With Acute Renal Failure in an Adult: A Case Report.

Cureus
2025

Reference Range for the Automated Fragmented Red Cell Parameter and Its Diagnostic Utility in Red Blood Cell (RBC) Fragment Quantification: A Prospective Study.

Cureus
2025

[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 xue
2025

Targeted optimization of single-chain variable fragment (scFv) expression in E. coli using a design-of-experiment approach.

International journal of biological macromolecules
2025

Thrombotic microangiopathy multidisciplinary assessment team: demographics, final diagnosis, treatment, and outcomes.

BMC nephrology
2025

Relevance of the interactions between the complement and contact coagulation systems on renal pathology.

Nefrologia
2025

Iptacopan/LNP023 and rituximab as rescue therapy in a patient with systemic lupus erythematosus-associated atypical haemolytic uraemic syndrome.

Journal of nephrology
2025

Case 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 immunology
2025

Identification of a new CD46 gene mutation site in a family with atypical hemolytic uremic syndrome.

BMC nephrology
2025

First Pediatric Lung Transplant Using the Paragonix BAROguard Preservation System.

Pediatric transplantation
2025

Pregnancy-Induced Atypical Hemolytic Uremic Syndrome.

Cureus
2025

Pregnancy-Related Acute Kidney Injury: Causes and Its Impact on Perinatal Outcomes-A Systematic Review.

Journal of clinical medicine
2026

Which Factors Influence Decisions to Withdraw from Eculizumab: A Qualitative Study of Patients Diagnosed with aHUS.

The patient
2025

Management of Atypical Haemolytic Uraemic Syndrome With Triggers: Diagnostic and Treatment Algorithms From an Asia-Pacific Perspective.

Nephrology (Carlton, Vic.)
2025

C3 p.Asp1115Asn Variant-associated Atypical Hemolytic Uremic Syndrome with Spontaneous Remission Triggered by Influenza and COVID-19.

Internal medicine (Tokyo, Japan)
2025

Glycoproteomics analysis of complement factor H and its complement-regulatory function during Streptococcus pneumoniae-associated hemolytic uremic syndrome.

Frontiers in immunology
2026

Is pericardial effusion restricted to STEC-HUS? Observations in anti-factor H associated atypical hemolytic uremic syndrome.

Pediatric nephrology (Berlin, Germany)
2025

Advanced Three-Dimensional Cell Culture Models for Investigating Enterohaemorrhagic Escherichia coli Pathogenesis and Shiga Toxin-Mediated Injury.

Journal of microbiology and biotechnology
2025

Shiga 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 biotechnology
2025

Cytokine storm and microvascular fate: mechanistic insights into endothelial injury in thrombotic microangiopathies.

Annals of medicine and surgery (2012)
2025

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 immunology
2025

Neutralizing antibodies in the intestinal mucosa are essential to control gastrointestinal infection by Shiga toxin-producing Escherichia coli.

mLife
2026

Long-term outcomes of volume expansion treatment in diarrhea-associated hemolytic uremic syndrome: a six-year follow-up.

Pediatric nephrology (Berlin, Germany)
2025

Effectiveness and Safety of Switching to Ravulizumab From Eculizumab in Kidney Transplant Recipients With Atypical Hemolytic Uremic Syndrome: A Global aHUS Registry Analysis.

Clinical transplantation
2025

A Case of Spontaneous Resolution: Atypical Hemolytic Uremic Syndrome in a 4-Year-Old Boy.

Pediatric blood & cancer
2025

In 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)
2025

Characterization of the small RNAs carried by outer membrane vesicles produced by hlyF-positive Shiga toxin-producing Escherichia coli.

Frontiers in cellular and infection microbiology
2025

COVID-19 Microangiopathy: Insights into plasma exchange as a therapeutic strategy.

Hematology, transfusion and cell therapy
2025

An ultrasensitive diagnostic system for minuscule level of hemolytic uremic syndrome.

Biosensors & bioelectronics
2026

Bloody diarrhea, STEC infection, and HUS in the molecular microbiology era.

Pediatric nephrology (Berlin, Germany)
2026

Cost-effectiveness of lifelong eculizumab versus disease monitoring of aHUS.

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
2025

Shiga toxin-producing Escherichia coli, food contamination, and bacteriophages as a control strategy.

Microbiology and molecular biology reviews : MMBR
2025

A presentation of posterior reversible encephalopathy syndrome after heart transplantation: a case report and review of literature.

Journal of medical case reports
2025

Paroxysmal nocturnal hemoglobinuria masquerading as hemolytic uremic syndrome: a Case Report.

Frontiers in medicine
2025

Genetic variability of Shiga toxin-producing Escherichia coli strains isolated from Paraguayan cattle.

Microbiology spectrum
2025

Efficacy of eculizumab discontinuation in atypical hemolytic uremic syndrome: a systematic review and meta-analysis.

Blood advances
2025

The Economic and Public Health Burden of Foodborne Illness in Somalia: Prevalence, Costs, and Policy Imperatives.

Public health challenges
2025

Adult Hemolytic Uremic Syndrome as a Diagnostic Challenge.

Cureus
2025

A Case Report of Atypical Hemolytic Uremic Syndrome Presenting With Disseminated Intravascular Coagulation.

Cureus
2025

Shiga 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 health
2025

Demographics and baseline disease characteristics of UK patients within the global aHUS registry.

BMC nephrology
2025

How I Treat: Genetic Testing in Atypical Hemolytic Uremic Syndrome.

Clinical journal of the American Society of Nephrology : CJASN
2025

A rare complication of thrombotic microangiopathy induced by chemotherapy for second breast cancer in a Hodgkin lymphoma survivor: a case report.

AME case reports
2025

Bilateral tractional retinal detachments complicating hemolytic-uremic syndrome.

American journal of ophthalmology case reports
2025

An antibiotic derivative as a new potential tool in the prevention of hemolytic uremic syndrome.

iScience
2025

Targeting the Roots of Kidney Disease: Systematic Review of the Therapies Targeting the Complement System.

Medicina (Kaunas, Lithuania)
2025

Influenza A H1N1 Triggering Complement-Mediated Hemolytic Uremic Syndrome.

Journal of medical cases
2025

Neurological Manifestations of Hemolytic Uremic Syndrome: A Comprehensive Review.

Brain sciences
2025

Highly sensitive Escherichia coli detection via cell surface in situ cytocompatible ATRP.

Analytical and bioanalytical chemistry
2025

A Rare Case of Atypical Haemolytic Syndrome Following Right Retrograde Intrarenal Surgery (RIRS).

Cureus
2026

Pericardial effusion in pediatric Shiga toxin-producing E. coli hemolytic uremic syndrome: a French multicentre study.

Pediatric nephrology (Berlin, Germany)
2025

Household transmission and carriage of Shiga toxin-producing Escherichia coli (STEC) O145, Stx1c: a family report.

The Turkish journal of pediatrics
2025

Shiga 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 microbiology
2025

Gene-Environment Interaction: Lessons From Complement-Mediated Kidney Disease.

Seminars in nephrology
2025

The epidemiology of haemolytic uraemic syndrome in England, 2009-2023: An analysis of national surveillance data.

The Journal of infection
2025

The Hideous Side of Acute Pancreatitis: A Case of Pancreatitis-Induced Atypical Hemolytic Uremic Syndrome.

Cureus
2025

Potentially reversible severe cardiac involvement in thrombotic microangiopathies with malignant hypertension.

Journal of nephrology
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Referências e fontes

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Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Circuit Survival in Children with Hemolytic Uremic Syndrome Requiring Continuous Renal Replacement Therapy.
    Blood purification· 2026· PMID 41861052mais citado
  2. [Clinical efficacy and safety of eculizumab in pediatric atypical hemolytic uremic syndrome].
    Zhonghua er ke za zhi = Chinese journal of pediatrics· 2026· PMID 41834207mais citado
  3. Cellular signaling pathway of Shiga toxin-induced ATP release.
    Frontiers in cellular and infection microbiology· 2026· PMID 41809984mais citado
  4. Shiga Toxin-Producing Escherichia coli Outbreak in Canadian Daycare Centers.
    JAMA network open· 2026· PMID 41805954mais citado
  5. Engineered bacterial therapy suppresses Enterohemorrhagic Escherichia coli through metabolic competition and virulence silencing.
    Nature communications· 2026· PMID 41633995mais citado
  6. Virulent phages MM-1 and MM-2 attack enterohemorrhagic Escherichia coli and Salmonella enterica strains.
    Int J Med Microbiol· 2026· PMID 41990421recente
  7. Case Report: COVID-19 unmasks factor H mutation-driven hemolytic uremic syndrome in a previously undiagnosed septuagenarian kidney transplant recipient.
    Front Med (Lausanne)· 2026· PMID 41987803recente
  8. A case report on the effective and safe use of ravulizumab in atypical hemolytic uremic syndrome during pregnancy.
    BMC Nephrol· 2026· PMID 41987101recente
  9. Hemolytic Uremic Syndrome Outbreak in Adults and Shiga Toxin-Producing Escherichia coli Negative for Locus of Enterocyte Effacement, France, 2025.
    Emerg Infect Dis· 2026· PMID 41986986recente
  10. [Clinical analysis of eculizumab in the treatment of atypical hemolytic uremic syndrome in children].
    Zhonghua Er Ke Za Zhi· 2026· PMID 41986268recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:544458(Orphanet)
  2. MONDO:0001549(MONDO)
  3. Sindrome Hemolitico-Uremica Atipica(PCDT · Ministério da Saúde)
  4. GARD:22233(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. 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.

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