HUS
MCID: HML001
MIFTS: 55

Hemolytic-Uremic Syndrome (HUS)

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

Aliases & Classifications for Hemolytic-Uremic Syndrome

MalaCards integrated aliases for Hemolytic-Uremic Syndrome:

Name: Hemolytic-Uremic Syndrome 12 77 30 56 6 45 15 74
Hemolytic Uremic Syndrome 12 54
Acute Renal Failure, Thrombocytopenia, and Microangiopathic Hemolytic Anemia Associated with Distorted Erythrocytes 54
Haemolytic-Uraemic Syndrome 12
Hus 54

Classifications:



External Ids:

Disease Ontology 12 DOID:12554
ICD9CM 36 283.11
MeSH 45 D006463
NCIt 51 C75545
ICD10 34 D59.3
UMLS 74 C0019061

Summaries for Hemolytic-Uremic Syndrome

NIH Rare Diseases : 54 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.

MalaCards based summary : Hemolytic-Uremic Syndrome, also known as hemolytic uremic syndrome, is related to nephrotic syndrome, type 7 and complement factor h deficiency. An important gene associated with Hemolytic-Uremic Syndrome is DGKE (Diacylglycerol Kinase Epsilon), and among its related pathways/superpathways are Innate Immune System and Creation of C4 and C2 activators. The drugs Epoetin alfa and Hematinics have been mentioned in the context of this disorder. Affiliated tissues include kidney, endothelial and heart, and related phenotypes are hematopoietic system and homeostasis/metabolism

Disease Ontology : 12 A kidney disease that is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs.

Wikipedia : 77 Hemolytic-uremic syndrome (HUS) is a group of blood disorders characterized by low red blood cells,... 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 422)
# Related Disease Score Top Affiliating Genes
1 nephrotic syndrome, type 7 33.9 CFH DGKE
2 complement factor h deficiency 32.9 CD46 CFH CFHR1 CFI
3 hemolytic uremic syndrome, atypical 1 31.8 ADAMTS13 C3 C5 CD46 CFB CFH
4 purpura 31.2 ADAMTS13 F3 THBD VWF
5 proliferative glomerulonephritis 30.9 C3 CFH
6 glomerulonephritis 30.8 C3 CD46 CFH CFHR5 CFI
7 thrombotic thrombocytopenic purpura 30.7 ADAMTS13 CD46 CFH F3 THBD VWF
8 disseminated intravascular coagulation 30.5 ADAMTS13 F3 THBD
9 c3 glomerulopathy 30.5 C3 CFB CFHR1 CFHR3 CFHR5
10 membranoproliferative glomerulonephritis 30.4 C3 CD46 CFB CFH CFHR5
11 thrombophilia 30.3 F3 THBD VWF
12 vasculitis 30.3 CFI THBD VWF
13 hemolytic anemia 30.1 ADAMTS13 C3 CFH CFI HP
14 hemoglobinuria 30.0 C3 C5 HP
15 catastrophic antiphospholipid syndrome 29.9 ADAMTS13 CFH F3
16 hemophilia 29.9 F3 VWF
17 evans' syndrome 29.9 ADAMTS13 HP
18 hemophilia a 29.8 F3 VWF
19 thrombotic thrombocytopenic purpura, congenital 29.8 ADAMTS13 CFH HP
20 von willebrand's disease 29.8 ADAMTS13 F3 VWF
21 stroke, ischemic 29.6 ADAMTS13 F3 THBD VWF
22 hepatic veno-occlusive disease 29.6 F3 THBD
23 pulmonary hypertension 29.5 F3 THBD VWF
24 antiphospholipid syndrome 29.4 ADAMTS13 F3 THBD VWF
25 thrombosis 29.4 ADAMTS13 F3 THBD VWF
26 enterocolitis 29.4 C3 CD46 CFB CFH CFHR1 CFHR3
27 essential thrombocythemia 29.3 F3 THBD VWF
28 malaria 29.2 ADAMTS13 F3 HP THBD VWF
29 systemic lupus erythematosus 29.2 ADAMTS13 C3 C5 CFB F3 THBD
30 macular degeneration, age-related, 1 29.0 C3 CD46 CFB CFH CFHR1 CFHR3
31 hellp syndrome 28.4 ADAMTS13 CD46 CFH CFI F3 HP
32 hemolytic uremic syndrome, atypical 3 12.8
33 hemolytic uremic syndrome, atypical 4 12.8
34 hemolytic uremic syndrome, atypical 5 12.8
35 hemolytic uremic syndrome, atypical 6 12.8
36 hemolytic uremic syndrome, atypical 2 12.8
37 hemolytic uremic syndrome, atypical, childhood 12.7
38 genetic atypical hemolytic-uremic syndrome 12.6
39 d-minus hemolytic uremic syndrome 12.4
40 d-plus hemolytic uremic syndrome 12.4
41 atypical hemolytic-uremic syndrome with h factor anomaly 12.4
42 shigellosis 11.8
43 combined immunodeficiency and megaloblastic anemia with or without hyperhomocysteinemia 11.5
44 pancreatitis, pediatric 11.3
45 dupuytren contracture 11.2
46 retinitis pigmentosa 3 11.1
47 diarrhea 10.9
48 colitis 10.8
49 encephalopathy 10.6
50 leukemia 10.5

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.97 ADAMTS13 C3 CFB CFH CFI F3
2 homeostasis/metabolism MP:0005376 9.96 ADAMTS13 C3 C5 CFB CFH CFI
3 cardiovascular system MP:0005385 9.91 C3 CD46 CFH F3 HP THBD
4 immune system MP:0005387 9.81 ADAMTS13 C3 C5 CFB CFH F3
5 normal MP:0002873 9.5 ADAMTS13 C3 C5 CFB CFH F3
6 renal/urinary system MP:0005367 9.1 C3 CFB CFH CFI HP THBD

Drugs & Therapeutics for Hemolytic-Uremic Syndrome

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

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Epoetin alfa Phase 4 113427-24-0
2 Hematinics Phase 4
3
Ravulizumab Approved, Investigational Phase 3 1803171-55-2
4 Complement System Proteins Phase 2, Phase 3,Phase 3
5 Immunologic Factors Phase 3,Phase 2
6 Shiga Toxins Phase 2, Phase 3,Phase 3,Phase 1
7 Pharmaceutical Solutions Phase 3,Phase 2,Phase 1,Not Applicable
8 Antibodies Phase 3,Phase 2
9 Immunoglobulins Phase 3,Phase 2
10 Immunosuppressive Agents Phase 3,Phase 2
11 Complement Inactivating Agents Phase 3
12 Factor VIII Phase 3
13 Antibodies, Bispecific Phase 3
14 Coagulants Phase 3
15
rituximab Approved Phase 2 174722-31-7 10201696
16
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
17
Promethazine Approved, Investigational Phase 2 60-87-7 4927
18
Acetaminophen Approved Phase 2 103-90-2 1983
19
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
20
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
21
Bevacizumab Approved, Investigational Phase 2 216974-75-3
22
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
23
Cyclophosphamide Approved, Investigational Phase 2 6055-19-2, 50-18-0 2907
24
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
25
Vinorelbine Approved, Investigational Phase 2 71486-22-1 60780 44424639
26
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
27
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
28
Emodepside Investigational, Vet_approved Phase 2 155030-63-0
29 Antineoplastic Agents, Immunological Phase 2
30 Antirheumatic Agents Phase 2
31 Antibodies, Monoclonal Phase 2
32 Anti-Infective Agents Phase 2
33 Antineoplastic Agents, Phytogenic Phase 2
34 Mitogens Phase 2
35 Immunoglobulin G Phase 2
36 Anti-Bacterial Agents Phase 2
37 Alkylating Agents Phase 2
38 Antimitotic Agents Phase 2
39 Angiogenesis Inhibitors Phase 2
40 Angiogenesis Modulating Agents Phase 2
41 Antibiotics, Antitubercular Phase 2
42 Endothelial Growth Factors Phase 2
43 Antifungal Agents Phase 2
44 Antineoplastic Agents, Alkylating Phase 2
45 Immunotoxin HA22 Phase 1, Phase 2,Phase 2
46 Immunotoxins Phase 1, Phase 2,Phase 2
47 Androgen Receptor Antagonists Phase 2
48 BB 1101 Phase 2
49
Metoclopramide Approved, Investigational Not Applicable 364-62-5 4168
50 dipyrone Not Applicable

Interventional clinical trials:

(show all 50)
# Name Status NCT ID Phase Drugs
1 Study Assessing an Algorithm-based Strategy of Eculizumab Discontinuation in Children and Adults With aHUS Active, not recruiting NCT02574403 Phase 4 eculizumab
2 Eval. of Potential Predictors of Disease Progression in Patients With aHUS Including Genetics, Biomarkers and Treatment Active, not recruiting NCT02614898 Phase 4
3 Erythropoietin in Hemolytic Uremic Syndrome Not yet recruiting NCT03776851 Phase 4 erythropoietin
4 Safety and Efficacy Study of Eculizumab in Shiga-Toxin Producing Escherichia Coli Hemolytic-Uremic Syndrome (STEC-HUS) Completed NCT01410916 Phase 2, Phase 3 Eculizumab (Soliris®)
5 Eculizumab in Shiga-toxin Related Hemolytic and Uremic Syndrome Pediatric Patients - ECULISHU Completed NCT02205541 Phase 3 Eculizumab;Placebo
6 Safety and Efficacy Study of OMS721 in Patients With Atypical Hemolytic Uremic Syndrome Recruiting NCT03205995 Phase 3
7 Study of ALXN1210 in Children and Adolescents With Atypical Hemolytic Uremic Syndrome (aHUS) Recruiting NCT03131219 Phase 3
8 rVA576 (Coversin) Long Term Safety and Efficacy Surveillance Study Recruiting NCT03829449 Phase 3 rVA576 (Coversin)
9 Improvements Through the Use of a Rapid Multiplex PCR Enteric Pathogen Detection Kit in Children With Hematochezia Recruiting NCT03362970 Phase 3
10 Single Arm Study of ALXN1210 in Complement Inhibitor Treatment-Naïve Adult and Adolescent Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Active, not recruiting NCT02949128 Phase 3
11 A Study to Evaluate the Safety and Tolerability of Prophylactic Emicizumab in Hemophilia A Patients With Inhibitors Active, not recruiting NCT03191799 Phase 3 Emicizumab
12 Phase III Randomized Study of SYNSORB Pk in Children With E. Coli-Associated Hemolytic Uremic Syndrome Terminated NCT00004465 Phase 3 SYNSORB Pk;Placebo
13 The Plasma Large-Volume Exchange RCT Withdrawn NCT01433003 Phase 3
14 Rituximab in Patients With Relapsed or Refractory TTP-HUS Unknown status NCT00531089 Phase 2 Rituximab
15 An Open-label, Multi-center Clinical Trial of Eculizumab in Adult Patients With Atypical Hemolytic-uremic Syndrome Completed NCT01194973 Phase 2 Eculizumab
16 An Open-Label, Multi-Center Clinical Trial of Eculizumab in Pediatric Patients With Atypical Hemolytic-Uremic Syndrome Completed NCT01193348 Phase 2 Eculizumab
17 Open Label Controlled Trial of Eculizumab in Adult Patients With Plasma Therapy-sensitive Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT00838513 Phase 2 eculizumab
18 Open Label Controlled Trial of Eculizumab in Adult Patients With Plasma Therapy-Resistant aHUS Completed NCT00844545 Phase 2 Eculizumab
19 Open Label Controlled Trial of Eculizumab in Adolescent Patients With Plasma Therapy-Resistant aHUS Completed NCT00844844 Phase 2 Eculizumab
20 Open Label Controlled Trial of Eculizumab in Adolescent Patients With Plasma Therapy-Sensitive aHUS Completed NCT00844428 Phase 2 eculizumab
21 The Safety and Efficacy of Eculizumab in Japanese Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01757431 Phase 2 Eculizumab
22 Study of Chimeric Monoclonal Antibodies to Shiga Toxins 1 and 2 Completed NCT01252199 Phase 2 cαStx1/cαStx2;Placebo
23 Vinorelbine Tartrate and Cyclophosphamide in Combination With Bevacizumab or Temsirolimus in Treating Patients With Recurrent or Refractory Rhabdomyosarcoma Completed NCT01222715 Phase 2 Cyclophosphamide;Temsirolimus;Vinorelbine Tartrate
24 Safety Study of CAT-8015 to Treat Advanced B-cell Non-Hodgkin Lymphoma and Chronic Lymphocytic Leukemia (NHL or CLL) Completed NCT01030536 Phase 1, Phase 2 CAT-8015 20 mcg/kg;CAT-8015 30 mcg/kg;CAT-8015 40 mcg/kg;CAT-8015 50 mcg/kg;CAT-8015 60 mcg/kg
25 Eculizumab to Treat Thrombotic Microangiopathy/Atypical Hemolytic Uremic Syndrome -Associated Multiple Organ Dysfunction Syndrome in Hematopoietic Stem Cell Transplant Recipients Recruiting NCT03518203 Phase 2 Eculizumab
26 Safety and Efficacy Study of OMS721 in Patients With Thrombotic Microangiopathies Recruiting NCT02222545 Phase 2
27 Complement Inhibition in aHUS Dialysis Patients Terminated NCT02464891 Phase 2 CCX168
28 Moxetumomab Pasudotox (CAT-8015, HA22) in Children With B-lineage Acute Lymphoblastic Leukemia and Minimal Residual Disease Prior to Allogeneic Hematopoietic Stem Cell Transplantation Terminated NCT02338050 Phase 2
29 A Study of an Investigational Drug, Cemdisiran (ALN-CC5), in Patients With Atypical Hemolytic Uremic Syndrome Withdrawn NCT03303313 Phase 2 Cemdisiran
30 Shiga Toxin Producing Escherichia Coli (STEC) Volume Expansion Not yet recruiting NCT03275792 Phase 1 D5-0.9%NS;Routine home oral rehydration
31 Complement Activation During Hemodialysis in Atypical Hemolytic Uraemic Syndrome as Underlying Kidney Disease Unknown status NCT00930423
32 Study of 'Vascular Competence' Profile and Endothelial Activation in the Hemolytic Uremic Syndrome in Children and Adults Unknown status NCT02904863 Not Applicable
33 Outbreak of Hemolytic Uremic Syndrome Linked to Escherichia Coli of Serotype O104:H4 Completed NCT01406288
34 A Retrospective, Observational, Non-interventional Trial to Assess Eculizumab Treatment Effect in Patients With Atypical Hemolytic Uremic Syndrome (aHUS) Completed NCT01770951
35 Haemolytic Uraemic Syndrome in Childhood: Clinical, Cognitive and Psychological Aspects Completed NCT01666548
36 To Characterize the Safety and Tolerability of Eculizumab in Two Japanese aHUS Patients Completed NCT01755429
37 Study of Repetitive Intestinal Lavage in Patients With EHEC Associated Hemorrhagic Colitis Completed NCT01561248 Not Applicable polyethylene glycol solution for daily bowel lavage.
38 The Role of Endothelium Dysfunction in Progression of CKD (Chronic Kidney Disease) After AKI (Acute Kidney Injury) Completed NCT00358306
39 Promising Bimarker Prediction of Outcome of HELLP Syndrome. Completed NCT03246542
40 Early Predictive Factors of Cardiac and Cerebral Involvement in TMA Completed NCT02134171 Not Applicable
41 Atypical Hemolytic-Uremic Syndrome (aHUS) Registry Recruiting NCT01522183
42 TTP and aHUS in Complicated Pregnancies Recruiting NCT03605511
43 Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford Recruiting NCT01793168
44 Collection of Human Samples to Study Hairy Cell and Other Leukemias, and to Develop Recombinant Immunotoxins for Cancer Treatment Recruiting NCT01087333
45 Eculizumab Use in the Postpartum Period for the Treatment of Pregnancy Associated aHUS: A Case Series Active, not recruiting NCT03574506
46 Usefulness of a Diagnostic Algorithm to Diagnose Thrombotic Microangiopathies in Pregnancy Active, not recruiting NCT03580941
47 Outcame of Cases With Hemolytic Uremic Syndrome Attending Assiut University Child Hospital Not yet recruiting NCT03690024
48 aHUS Observational Long Term Follow-Up Terminated NCT01522170
49 International Registry and Biorepository for TMA(Thrombotic Microangiopathy) Terminated NCT00593229
50 The Role of Microparticles as a Biomarker Withdrawn NCT02626663

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:

# Genetic test Affiliating Genes
1 Hemolytic-Uremic Syndrome 30

Anatomical Context for Hemolytic-Uremic Syndrome

MalaCards organs/tissues related to Hemolytic-Uremic Syndrome:

42
Kidney, Endothelial, Heart, Liver, Bone, Bone Marrow, Colon

Publications for Hemolytic-Uremic Syndrome

Articles related to Hemolytic-Uremic Syndrome:

(show top 50) (show all 2458)
# Title Authors Year
1
Hemolytic uremic syndrome associated with Bordetella pertussis infection in a 2-month-old infant carrying a pathogenic variant in complement factor H. ( 30560448 )
2019
2
Purtscher-Like Retinopathy in Hemolytic Uremic Syndrome. ( 30629118 )
2019
3
Complement Activation Contributes to the Pathophysiology of Shiga Toxin-Associated Hemolytic Uremic Syndrome. ( 30634669 )
2019
4
Correction to: Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use. ( 30645679 )
2019
5
Complement Gene Variants and Shiga Toxin-Producing Escherichia coli-Associated Hemolytic Uremic Syndrome: Retrospective Genetic and Clinical Study. ( 30674459 )
2019
6
Atypical hemolytic-uremic syndrome: recurrent phenotypic expression of a patient with MCP gene mutation combined with risk haplotypes. ( 30676336 )
2019
7
Optimal duration of treatment with eculizumab in atypical hemolytic uremic syndrome (aHUS)-a question to be addressed in a scientific way. ( 30693384 )
2019
8
Thrombotic Microangiopathy, Hemolytic Uremic Syndrome, and Thrombotic Thrombocytopenic Purpura Following Hump-nosed Pit Viper (Genus: Hypnale) Envenoming in Sri Lanka. ( 30711421 )
2019
9
Hyperfunctional complement C3 promotes C5-dependent atypical hemolytic uremic syndrome in mice. ( 30714990 )
2019
10
Three months interval therapy of Eculizumab in a patient with atypical hemolytic uremic syndrome with hybrid CFHR1/CFH gene. ( 30715674 )
2019
11
Eculizumab for atypical hemolytic-uremic syndrome. How long should we maintain it? ( 30798995 )
2019
12
Seizure as the Presenting Symptom for Atypical Hemolytic Uremic Syndrome. ( 30826084 )
2019
13
Optic coherence tomography angiography follow-up in a case of Purtscher-like retinopathy due to atypical hemolytic uremic syndrome. ( 30841747 )
2019
14
Chronic Neurological Complications in Hemolytic Uremic Syndrome in Children. ( 30851717 )
2019
15
An Ex Vivo Test of Complement Activation on Endothelium for Individualized Eculizumab Therapy in Hemolytic Uremic Syndrome. ( 30851964 )
2019
16
Atypical hemolytic uremic syndrome and complement blockade: established and emerging uses of complement inhibition. ( 30865166 )
2019
17
Hemolytic Uremic Syndrome in an Infant with Primary Hyperoxaluria Type II: An Unreported Clinical Association. ( 30889567 )
2019
18
Impact of hypertensive emergency and complement rare variants on presentation and outcome of atypical hemolytic uremic syndrome. ( 30890598 )
2019
19
Postpartum Renal Cortical Necrosis Is Associated With Atypical Hemolytic Uremic Syndrome in Developing Countries. ( 30899869 )
2019
20
Eculizumab Use for Kidney Transplantation in Patients With a Diagnosis of Atypical Hemolytic Uremic Syndrome. ( 30899871 )
2019
21
Whole-exome sequencing detects mutations in pediatric patients with atypical hemolytic uremic syndrome in Taiwan. ( 30905589 )
2019
22
Hemolytic Uremic Syndrome Associated With Non-Shigatoxin-producing Infectious Agents: Expanding the Shigatoxin Theory. ( 29683956 )
2019
23
Steroid Responsive Atypical Hemolytic Uremic Syndrome Triggered by Influenza B Infection. ( 29702545 )
2019
24
An Atypical Case of Atypical Hemolytic Uremic Syndrome. ( 29750742 )
2019
25
A pediatric neurologic assessment score may drive the eculizumab-based treatment of Escherichia coli-related hemolytic uremic syndrome with neurological involvement. ( 30362078 )
2019
26
Haploidentical Hematopoietic Stem Cell Transplant Complicated by Atypical Hemolytic Uremic Syndrome and Kidney Transplant From the Same Donor With No Immunosuppression but C5 Inhibition. ( 30365467 )
2019
27
No More Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome Please. ( 30367852 )
2019
28
Hemolytic Uremic Syndrome. ( 30454746 )
2019
29
Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome due to Complement Factor H Deficiency: Impact of Liver Transplantation. ( 29215813 )
2018
30
Blocking Properdin Prevents Complement-Mediated Hemolytic Uremic Syndrome and Systemic Thrombophilia. ( 29858280 )
2018
31
Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype andA outcome. ( 29907460 )
2018
32
Pathogenic role of inflammatory response during Shiga toxin-associated hemolytic uremic syndrome (HUS). ( 29372302 )
2018
33
Atypical Presentation of Pregnancy-Related Hemolytic Uremic Syndrome. ( 29331477 )
2018
34
Which should be the correct treatment for monoclonal gammopathy of renal significance with complement alternative pathway dysregulation (C3 glomerulopathy and atypical hemolytic uremic syndrome): clone-directed or anticomplement therapy? ( 29808447 )
2018
35
An outbreak of hemolytic uremic syndrome in southern Romania during 2015-2016: Epidemiologic, clinical, laboratory, microbiologic, therapeutic and outcome characteristics. ( 29807724 )
2018
36
CCR2-dependent Gr1high monocytes promote kidney injury in shiga toxin-induced hemolytic uremic syndrome in mice. ( 29446073 )
2018
37
Complement activation in atypical hemolytic uremic syndrome and scleroderma renal crisis: a critical analysis of pathophysiology. ( 29796581 )
2018
38
Case Series of 3 Patients Diagnosed With Atypical Hemolytic Uremic Syndrome Successfully Treated With Steroids, Plasmapheresis, and Rituximab. ( 29308213 )
2018
39
Atypical hemolytic uremic syndrome: An unusual postoperative complication. ( 29426622 )
2018
40
Gastrointestinal pathogens in anti-FH antibody positive and negative Hemolytic Uremic Syndrome. ( 29795200 )
2018
41
Thrombomodulin and Endothelial Dysfunction: A Disease-Modifier Shared between Malignant Hypertension and Atypical Hemolytic Uremic Syndrome. ( 29940557 )
2018
42
Bevacizumab-induced atypical hemolytic uremic syndrome and treatment with eculizumab. ( 29768958 )
2018
43
Poststreptococcal glomerulonephritis with atypical hemolytic uremic syndrome: An unusual presentation. ( 29970754 )
2018
44
C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction. ( 29594148 )
2018
45
Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy. ( 29500241 )
2018
46
Safety and effectiveness of eculizumab for adult patients with atypical hemolytic-uremic syndrome in Japan: interim analysis of post-marketing surveillance. ( 29959568 )
2018
47
Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. ( 29356300 )
2018
48
50 Years Ago in The Journal of Pediatrics: The Hemolytic-Uremic Syndrome: Renal Status of 76 Patients at Long-Term Follow-Up. ( 29801537 )
2018
49
Atypical adult-onset methylmalonic acidemia and homocystinuria presenting as hemolytic uremic syndrome. ( 29294253 )
2018
50
Is eculizumab efficacious in Shigatoxin-associated hemolytic uremic syndrome? A narrative review of current evidence. ( 29288280 )
2018

Variations for Hemolytic-Uremic Syndrome

ClinVar genetic disease variations for Hemolytic-Uremic Syndrome:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 DGKE NM_003647.2(DGKE): c.888+40A> G single nucleotide variant Pathogenic rs1555599211 GRCh38 Chromosome 17, 56848105: 56848105
2 DGKE NM_003647.2(DGKE): c.888+40A> G single nucleotide variant Pathogenic rs1555599211 GRCh37 Chromosome 17, 54925466: 54925466

Expression for Hemolytic-Uremic Syndrome

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

Pathways for Hemolytic-Uremic Syndrome

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.62 C3 C5 CD46 CFB CFH CFHR3
2
Show member pathways
11.86 C3 C5 CD46 CFB CFH CFHR3
3
Show member pathways
11.78 F3 THBD VWF
4
Show member pathways
11.74 C3 C5 CD46 CFB CFH
5
Show member pathways
11.63 C3 C5 CD46 CFB CFH CFHR3
6 11.49 C3 C5 CFB CFH CFI
7 11.42 C3 C5 CD46 CFB CFH CFI

GO Terms for Hemolytic-Uremic Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.91 C3 C5 CD46 CFB CFH CFHR3
2 extracellular region GO:0005576 9.9 ADAMTS13 C3 C5 CFB CFH CFHR1
3 extracellular space GO:0005615 9.7 ADAMTS13 C3 C5 CFB CFH CFHR1
4 blood microparticle GO:0072562 9.1 C3 CFB CFH CFHR1 CFHR3 HP

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

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.88 ADAMTS13 C3 CFB CFI HP
2 innate immune response GO:0045087 9.8 C3 C5 CD46 CFB CFH CFI
3 blood coagulation GO:0007596 9.71 ADAMTS13 F3 THBD VWF
4 positive regulation of angiogenesis GO:0045766 9.67 C3 C5 F3
5 complement activation, classical pathway GO:0006958 9.67 C3 C5 CD46 CFI
6 platelet activation GO:0030168 9.63 ADAMTS13 DGKE VWF
7 hemostasis GO:0007599 9.56 ADAMTS13 F3 THBD VWF
8 complement activation GO:0006956 9.55 C3 C5 CFB CFH CFHR1
9 positive regulation of vascular endothelial growth factor production GO:0010575 9.51 C3 C5
10 positive regulation of cytolysis GO:0045919 9.43 CFHR1 CFHR5
11 complement activation, alternative pathway GO:0006957 9.35 C3 C5 CFB CFH CFHR5
12 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:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.77 ADAMTS13 C3 C5 CD46 CFB CFH
2 endopeptidase inhibitor activity GO:0004866 9.16 C3 C5
3 serine-type endopeptidase activity GO:0004252 9.02 C3 CFB CFI F3 HP

Sources for Hemolytic-Uremic Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....