HUS
MCID: HML001
MIFTS: 51

Hemolytic-Uremic Syndrome (HUS) malady

Categories: Rare diseases, Immune diseases, Blood diseases, Nephrological diseases

Aliases & Classifications for Hemolytic-Uremic Syndrome

Aliases & Descriptions for Hemolytic-Uremic Syndrome:

Name: Hemolytic-Uremic Syndrome 12 52 42 14 69
Hemolytic Uremic Syndrome 12 50 29
Acute Renal Failure, Thrombocytopenia, and Microangiopathic Hemolytic Anemia Associated with Distorted Erythrocytes 50
Haemolytic-Uraemic Syndrome 12
Hus 50

Classifications:



External Ids:

Disease Ontology 12 DOID:12554
ICD10 33 D59.3
ICD9CM 35 283.11
MeSH 42 D006463
NCIt 47 C75545
SNOMED-CT 64 111407006 123308008
UMLS 69 C0019061

Summaries for Hemolytic-Uremic Syndrome

NIH Rare Diseases : 50 hemolytic uremic syndrome (hus) is a disorder that usually occurs when an  e. coli bacterial infection in the digestive system produces toxic substances that destroy red blood cells. symptoms include vomiting and diarrhea, fever, lethargy, and weakness. in severe cases it can lead to kidney failure or death. while this condition is most common in children, it often has a more complicated presentation in adults. treatment may include dialysis, corticosteroids, transfusions of packed red blood cells and plasmapheresis.   hemolytic uremic syndrome should be distinguished from atypical hemolytic uremic syndrome (ahus). the two conditions have different causes and different signs and symptoms. last updated: 1/3/2015

MalaCards based summary : Hemolytic-Uremic Syndrome, also known as hemolytic uremic syndrome, is related to complement factor h deficiency and mesangial proliferative glomerulonephritis. An important gene associated with Hemolytic-Uremic Syndrome is CFH (Complement Factor H), and among its related pathways/superpathways are Innate Immune System and Creation of C4 and C2 activators. The drugs Complement System Proteins and Shiga Toxins have been mentioned in the context of this disorder. Affiliated tissues include kidney, endothelial and liver, and related phenotypes are hematopoietic system and homeostasis/metabolism

Wikipedia : 71 Hemolytic-uremic syndrome (or haemolytic-uraemic syndrome), abbreviated HUS, is a disease characterized... more...

Related Diseases for Hemolytic-Uremic Syndrome

Diseases in the Hemolytic-Uremic Syndrome family:

D-Plus Hemolytic Uremic Syndrome

Diseases related to Hemolytic-Uremic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 212)
id Related Disease Score Top Affiliating Genes
1 complement factor h deficiency 31.9 CFH CFHR1 CFHR3 CFHR5 CFI DGKE
2 mesangial proliferative glomerulonephritis 29.7 CD40LG CD79A
3 membranoproliferative glomerulonephritis 29.6 ADAMTS13 CD40LG CD79A VWF
4 homocystinuria 29.5 CD40LG CD79A
5 hemolytic uremic syndrome, atypical 1 12.7
6 hemolytic uremic syndrome, atypical 6 12.5
7 hemolytic uremic syndrome, atypical 3 12.5
8 hemolytic uremic syndrome, atypical 4 12.5
9 hemolytic uremic syndrome, atypical 5 12.5
10 hemolytic uremic syndrome, atypical 2 12.5
11 hemolytic uremic syndrome, atypical, childhood 12.4
12 genetic atypical hemolytic-uremic syndrome 12.1
13 thbd-related atypical hemolytic-uremic syndrome 12.1
14 d-minus hemolytic uremic syndrome 12.1
15 d-plus hemolytic uremic syndrome 12.1
16 atypical hemolytic-uremic syndrome with h factor anomaly 12.1
17 cfhr3 and cfhr1-related atypical hemolytic-uremic syndrome 12.0
18 cfi-related atypical hemolytic-uremic syndrome 12.0
19 dgke-related atypical hemolytic-uremic syndrome 12.0
20 c3-related atypical hemolytic-uremic syndrome 12.0
21 cd46-related atypical hemolytic-uremic syndrome 12.0
22 cfb-related atypical hemolytic-uremic syndrome 12.0
23 cfh-related atypical hemolytic-uremic syndrome 12.0
24 cfhr1 and cfhr4-related atypical hemolytic-uremic syndrome 12.0
25 nephrotic syndrome, type 7 11.9
26 shigellosis 11.6
27 pancreatitis, pediatric 11.1
28 purpura 10.7
29 thrombotic thrombocytopenic purpura 10.7
30 diarrhea 10.5
31 pneumonia 10.5
32 endotheliitis 10.4
33 extraventricular neurocytoma 10.4 CFH CFI
34 acute ackee fruit intoxication 10.4 CFH CFI
35 glomerulonephritis 10.3
36 multiple vertebral anomalies unusual facies 10.3 CFB CFH
37 multiple synostoses syndrome 1 10.3 CFH DGKE
38 elane-related neutropenia 10.3 CFH CFHR5
39 hand, foot and mouth disease 10.3 CFB CFH
40 endosalpingiosis 10.3 CFB CFH
41 stomatocytosis i 10.3 ADAMTS13 CFH
42 pancreatitis 10.3
43 rhizomelic chondrodysplasia punctata 10.2 C3 CFB CFH
44 urogenital adysplasia 10.2 THBD VWF
45 encephalopathy 10.2
46 macular degeneration, age-related, 13 10.2 C3 CFI
47 anuria 10.2
48 influenza 10.2
49 colitis 10.2
50 macular degeneration, age-related, 4 10.1 CD46 CFH CFHR1 CFI

Comorbidity relations with Hemolytic-Uremic Syndrome via Phenotypic Disease Network (PDN):


Acute Kidney Failure Chronic Kidney Failure
Deficiency Anemia Heart Disease

Graphical network of the top 20 diseases related to Hemolytic-Uremic Syndrome:



Diseases related to Hemolytic-Uremic Syndrome

Symptoms & Phenotypes for Hemolytic-Uremic Syndrome

MGI Mouse Phenotypes related to Hemolytic-Uremic Syndrome:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 ADAMTS13 C3 CD40LG CD79A CFB CFH
2 homeostasis/metabolism MP:0005376 9.96 C5 CD40LG CD79A CFB CFH CFI
3 immune system MP:0005387 9.81 ADAMTS13 C3 C5 CD40LG CD79A CFB
4 normal MP:0002873 9.5 ADAMTS13 C3 C5 CD79A CFB CFH
5 renal/urinary system MP:0005367 9.23 C3 C5 CD40LG CD79A CFB CFH

Drugs & Therapeutics for Hemolytic-Uremic Syndrome

Drugs for Hemolytic-Uremic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Complement System Proteins Phase 2, Phase 3
2 Shiga Toxins Phase 2, Phase 3
3 Antibodies Phase 3,Phase 2
4 Immunoglobulins Phase 3,Phase 2
5 Pharmaceutical Solutions Phase 3
6 Complement Inactivating Agents Phase 3
7
Acetaminophen Approved Phase 2 103-90-2 1983
8
Diphenhydramine Approved Phase 2 58-73-1, 147-24-0 3100
9
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
10
Promethazine Approved Phase 2 60-87-7 4927
11
rituximab Approved Phase 2 174722-31-7 10201696
12 Antirheumatic Agents Phase 2
13 Antibodies, Monoclonal Phase 2
14
Metoclopramide Approved, Investigational 364-62-5 4168
15 dipyrone
16 Immunotoxins
17 Natriuretic Agents
18 Natriuretic Peptide, Brain

Interventional clinical trials:

(show all 33)
id Name Status NCT ID Phase
1 Study Assessing an Algorithm-based Strategy of Eculizumab Discontinuation in Children and Adults With aHUS Recruiting NCT02574403 Phase 4
2 Eval. of Potential Predictors of Disease Progression in Patients With aHUS Including Genetics, Biomarkers and Treatment Enrolling by invitation NCT02614898 Phase 4
3 Safety and Efficacy Study of Eculizumab in Shiga-Toxin Producing Escherichia Coli Hemolytic-Uremic Syndrome (STEC-HUS) Completed NCT01410916 Phase 2, Phase 3
4 Single Arm Study of ALXN1210 in Complement Inhibitor Treatment-Naïve Adult and Adolescent Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Recruiting NCT02949128 Phase 3
5 Eculizumab in Shiga-toxin Related Hemolytic and Uremic Syndrome Pediatric Patients - ECULISHU Recruiting NCT02205541 Phase 3
6 Study of ALXN1210 in Children and Adolescents With Atypical Hemolytic Uremic Syndrome (aHUS) Not yet recruiting NCT03131219 Phase 3
7 Phase III Randomized Study of SYNSORB Pk in Children With E. Coli-Associated Hemolytic Uremic Syndrome Terminated NCT00004465 Phase 3
8 The Plasma Large-Volume Exchange RCT Withdrawn NCT01433003 Phase 3
9 Rituximab in Patients With Relapsed or Refractory TTP-HUS Unknown status NCT00531089 Phase 2
10 An Open-label, Multi-center Clinical Trial of Eculizumab in Adult Patients With Atypical Hemolytic-uremic Syndrome Completed NCT01194973 Phase 2
11 An Open-Label, Multi-Center Clinical Trial of Eculizumab in Pediatric Patients With Atypical Hemolytic-Uremic Syndrome Completed NCT01193348 Phase 2
12 Open Label Controlled Trial of Eculizumab in Adult Patients With Plasma Therapy-sensitive Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT00838513 Phase 2
13 Open Label Controlled Trial of Eculizumab in Adult Patients With Plasma Therapy-Resistant aHUS Completed NCT00844545 Phase 2
14 Open Label Controlled Trial of Eculizumab in Adolescent Patients With Plasma Therapy-Resistant aHUS Completed NCT00844844 Phase 2
15 Open Label Controlled Trial of Eculizumab in Adolescent Patients With Plasma Therapy-Sensitive aHUS Completed NCT00844428 Phase 2
16 The Safety and Efficacy of Eculizumab in Japanese Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01757431 Phase 2
17 Study of Chimeric Monoclonal Antibodies to Shiga Toxins 1 and 2 Completed NCT01252199 Phase 2
18 Safety and Efficacy Study of OMS721 in Patients With Thrombotic Microangiopathies Recruiting NCT02222545 Phase 2
19 Complement Inhibition in aHUS Dialysis Patients Terminated NCT02464891 Phase 2
20 Complement Activation During Hemodialysis in Atypical Hemolytic Uraemic Syndrome as Underlying Kidney Disease Unknown status NCT00930423
21 Outbreak of Hemolytic Uremic Syndrome Linked to Escherichia Coli of Serotype O104:H4 Completed NCT01406288
22 A Retrospective, Observational, Non-interventional Trial to Assess Eculizumab Treatment Effect in Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01770951
23 Haemolytic Uraemic Syndrome in Childhood: Clinical, Cognitive and Psychological Aspects Completed NCT01666548
24 To Characterize the Safety and Tolerability of Eculizumab in Two Japanese aHUS Patients Completed NCT01755429
25 Study of Repetitive Intestinal Lavage in Patients With EHEC Associated Hemorrhagic Colitis Completed NCT01561248
26 The Role of Endothelium Dysfunction in Progression of CKD (Chronic Kidney Disease) After AKI (Acute Kidney Injury) Completed NCT00358306
27 Atypcial Hemolytic-Uremic Syndrome (aHUS) Registry Recruiting NCT01522183
28 Study of 'Vascular Competence' Profile and Endothelial Activation in the Hemolytic Uremic Syndrome in Children and Adults Recruiting NCT02904863
29 Collection of Human Samples to Study Hairy Cell and Other Leukemias, and to Develop Recombinant Immunotoxins for Cancer Treatment Recruiting NCT01087333
30 Early Predictive Factors of Cardiac and Cerebral Involvement in TMA Active, not recruiting NCT02134171
31 The Role of Microparticles as a Biomarker Enrolling by invitation NCT02626663
32 aHUS Observational Long Term Follow-Up Terminated NCT01522170
33 International Registry and Biorepository for TMA(Thrombotic Microangiopathy) Terminated NCT00593229

Search NIH Clinical Center for Hemolytic-Uremic Syndrome

Cochrane evidence based reviews: hemolytic-uremic syndrome

Genetic Tests for Hemolytic-Uremic Syndrome

Genetic tests related to Hemolytic-Uremic Syndrome:

id Genetic test Affiliating Genes
1 Hemolytic Uremic Syndrome 29

Anatomical Context for Hemolytic-Uremic Syndrome

MalaCards organs/tissues related to Hemolytic-Uremic Syndrome:

39
Kidney, Endothelial, Liver, Lung, Heart, Monocytes, Prostate

Publications for Hemolytic-Uremic Syndrome

Articles related to Hemolytic-Uremic Syndrome:

(show top 50) (show all 846)
id Title Authors Year
1
Pathogenic Variants in Complement Genes and Risk of Atypical Hemolytic Uremic Syndrome Relapse after Eculizumab Discontinuation. ( 27799617 )
2017
2
Clinical characteristics and long-term outcome of diarrhea-associated hemolytic uremic syndrome: a single center experience. ( 28074307 )
2017
3
Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic granulomatosis with polyangiitis (EGPA): a possible relationship. ( 28509134 )
2017
4
Blood urea nitrogen to serum creatinine ratio is an accurate predictor of outcome in diarrhea-associated hemolytic uremic syndrome, a preliminary study. ( 28078430 )
2017
5
Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients. ( 28056875 )
2017
6
Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation. ( 28057640 )
2017
7
Gemcitabine-induced hemolytic-uremic syndrome treated with eculizumab or plasmapheresis: two case reportsa8c. ( 27879189 )
2017
8
Complement and the kidney in the setting of Shiga-toxin hemolytic uremic syndrome, organ transplantation, and C3 glomerulonephritis. ( 27156109 )
2016
9
Greenbaum LA, Fila M, Ardissino G, etA al. Eculizumab is a safe and effective treatment in pediatric patients withA atypical hemolytic uremic syndrome. Kidney Int. 2016;89:701-711. ( 27521120 )
2016
10
Clinical guides for atypical hemolytic uremic syndrome in Japan. ( 27460397 )
2016
11
Low Serum Fetuin-A as a Biomarker to Predict Pneumococcal Necrotizing Pneumonia and Hemolytic Uremic Syndrome in Children. ( 27043691 )
2016
12
Thrombotic Thrombocytopenic Purpura and Atypical Hemolytic Uremic Syndrome Microangiopathy in Pregnancy. ( 27648610 )
2016
13
Prevalence and clinical course of typical hemolytic uremic syndrome among sibling. ( 27869414 )
2016
14
Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab. ( 27848226 )
2016
15
Prevention of recurrence of atypical hemolytic uremic syndrome post renal transplant with the use of higher-dose eculizumaba8c. ( 27616760 )
2016
16
CFH gene mutation in a case of Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS). ( 26399238 )
2016
17
Association among Complement Factor H Autoantibodies, Deletions of CFHR, and the Risk of Atypical Hemolytic Uremic Syndrome. ( 27929404 )
2016
18
A case of renal recovery in atypical hemolytic uremic syndrome treated with eculizumab. ( 27049376 )
2016
19
Brief Review and a Clinical Case of Hemolytic Uremic Syndrome Associated with Interferon I^ Treatment. ( 27951532 )
2016
20
From Gestational Hypertension and Preeclampsia to Atypical Hemolytic Uremic Syndrome. ( 27548541 )
2016
21
No association between dysplasminogenemia with p.Ala620Thr mutation and atypical hemolytic uremic syndrome. ( 27194432 )
2016
22
Diarrhea, Urosepsis and Hemolytic Uremic Syndrome Caused by the Same Heteropathogenic Escherichia Coli Strain. ( 27195604 )
2016
23
Livedo racemosa and hemolytic uremic syndrome induced by gemcitabine. ( 27125732 )
2016
24
Development of camelid single chain antibodies against Shiga toxin type 2 (Stx2) with therapeutic potential against Hemolytic Uremic Syndrome (HUS). ( 27118524 )
2016
25
Adjustment of Eculizumab Dosage Pattern in Patients with Atypical Hemolytic Uremic Syndrome with Suboptimal Response to Standard Treatment Pattern. ( 28025630 )
2016
26
Small-molecule Factor D inhibitors selectively block the alternative pathway of complement in paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome. ( 27810992 )
2016
27
Whole-Genome Characterization and Strain Comparison of VT2f-Producing Escherichia coli Causing Hemolytic Uremic Syndrome. ( 27584691 )
2016
28
Atypical hemolytic uremic syndrome: a clinical conundrum. ( 27139899 )
2016
29
Genetic analysis and functional characterization of novel mutations in a series of patients with atypical hemolytic uremic syndrome. ( 26826462 )
2016
30
Investigation of enteropathogenic Escherichia coli and Shiga toxin-producingEscherichia coli associated with hemolytic uremic syndrome in A^zmir Province, Turkey. ( 27513249 )
2016
31
Maintenance eculizumab dose adjustment in the treatment of atypical hemolytic uremic syndrome: a case report and review of the literature. ( 27525082 )
2016
32
Defining the role of pneumococcal neuraminidases and O-glycosidase in pneumococcal hemolytic uremic syndrome. ( 27469261 )
2016
33
Angiotensinogen and interleukin-18 as markers of chronic kidney damage in children with a history of hemolytic uremic syndrome. ( 27982687 )
2016
34
Erratum to: Health-related quality of life and mental health in parents of children with hemolytic uremic syndrome. ( 27048229 )
2016
35
Indications of underdiagnosis of Atypical Hemolytic Uremic Syndrome in a cohort referred to the Coagulation Unit in Malmo, Sweden, for analysis of ADAMTS13 2007-2012. ( 27175932 )
2016
36
Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea. ( 27550478 )
2016
37
Postoperative Atypical Hemolytic Uremic Syndrome Treated Successfully with Eculizumab. ( 27150874 )
2016
38
Rituximab to treat gemcitabine-induced hemolytic-uremic syndrome (HUS) in pancreatic adenocarcinoma: a case series and literature review. ( 27497971 )
2016
39
Disturbed sialic acid recognition on endothelial cells and platelets in complement attack causes atypical hemolytic uremic syndrome. ( 27006390 )
2016
40
The clinical and laboratory features of Chinese Han anti-factor H autoantibody-associated hemolytic uremic syndrome. ( 28035470 )
2016
41
Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference. ( 27989322 )
2016
42
Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus. ( 27556240 )
2016
43
Hemolytic uremic syndrome associated with snakebite envenomation- A retrospective case series. ( 27727880 )
2016
44
Digital gangrene in a child with atypical hemolytic uremic syndrome associated with anti-factor H antibodies. ( 27194846 )
2016
45
Pneumococcal hemolytic uremic syndrome and steroid resistant nephrotic syndrome. ( 27478599 )
2016
46
A Case Report and Literature Review of Eculizumab Withdrawal in Atypical Hemolytic-Uremic Syndrome. ( 27974740 )
2016
47
Coexistence of atypical hemolytic uremic syndrome with membranoproliferative glomerulonephritis and antineutrophil cytoplasmic antibodies-associated vasculitis. ( 27424702 )
2016
48
Terminal Complement Inhibitor Eculizumab in Adult Patients With Atypical Hemolytic Uremic Syndrome: A Single-Arm, Open-Label Trial. ( 27012908 )
2016
49
C3:CH50 ratio as a proposed composite marker for eculizumab monitoring in atypical hemolytic uremic syndrome: Preliminary results. ( 27617501 )
2016
50
Cerebral Hemodynamics in Patients with Hemolytic Uremic Syndrome Assessed by Susceptibility Weighted Imaging and Four-Dimensional Non-Contrast MR Angiography. ( 27802295 )
2016

Variations for Hemolytic-Uremic Syndrome

Expression for Hemolytic-Uremic Syndrome

Search GEO for disease gene expression data for Hemolytic-Uremic Syndrome.

Pathways for Hemolytic-Uremic Syndrome

GO Terms for Hemolytic-Uremic Syndrome

Cellular components related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.91 C3 C5 CD46 CFB CFH CFHR1
2 extracellular region GO:0005576 9.85 ADAMTS13 C3 C5 CD40LG CFB CFH
3 blood microparticle GO:0072562 9.35 C3 CFB CFH CFHR1 CFHR3
4 extracellular space GO:0005615 9.32 ADAMTS13 C3 C5 CD40LG CFB CFH

Biological processes related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.85 ADAMTS13 C3 CD46 CFB CFI
2 immune system process GO:0002376 9.83 C3 C5 CD46 CD79A CFB
3 innate immune response GO:0045087 9.73 C3 C5 CD46 CFB CFH CFI
4 complement activation, classical pathway GO:0006958 9.67 C3 C5 CD46 CFI
5 platelet activation GO:0030168 9.62 ADAMTS13 CD40LG DGKE VWF
6 hemostasis GO:0007599 9.58 ADAMTS13 THBD VWF
7 complement activation GO:0006956 9.55 C3 C5 CFB CFH CFHR1
8 positive regulation of interleukin-10 production GO:0032733 9.48 CD40LG CD46
9 complement activation, alternative pathway GO:0006957 9.35 C3 C5 CFB CFH CFHR5
10 regulation of complement activation GO:0030449 9.23 C3 C5 CD46 CFB CFH CFHR1

Molecular functions related to Hemolytic-Uremic Syndrome according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 complement binding GO:0001848 8.62 CD46 CFB

Sources for Hemolytic-Uremic Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
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70 UMLS via Orphanet
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