HES
MCID: HYP098
MIFTS: 67

Hypereosinophilic Syndrome (HES)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Hypereosinophilic Syndrome

MalaCards integrated aliases for Hypereosinophilic Syndrome:

Name: Hypereosinophilic Syndrome 12 74 52 58 36 54 15 17 71
Eosinophilia 12 74 29 54 6 43 15 71 32
Hes 52 58 47
Disseminated Eosinophilic Collagen Disease 71
Hypereosinophilic Syndrome, Idiopathic 52
Idiopathic Hypereosinophilic Syndrome 71
Eosinophilic Leukocytosis 12
Eosinophilic Disorder 71

Characteristics:

Orphanet epidemiological data:

58
hypereosinophilic syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (United States),1-9/100000 (Europe),1-9/100000 (United States); Age of onset: All ages; Age of death: any age;

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

Disease Ontology 12 DOID:999
KEGG 36 H01599
ICD9CM 34 288.3
MeSH 43 D004802
ICD10 32 D72.1
MESH via Orphanet 44 D017681
ICD10 via Orphanet 33 D47.5
UMLS via Orphanet 72 C1540912
Orphanet 58 ORPHA168956
UMLS 71 C0014457 C0206141 C0263662 more

Summaries for Hypereosinophilic Syndrome

KEGG : 36 Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders characterized by marked blood or tissue eosinophilia resulting in a wide variety of clinical manifestations. A number of HES subtypes, or variants, have recently been identified. The best characterized of the clonal molecular defects implicated in myeloproliferative variant HES is the 800-kb interstitial deletion on chromosome 4q12 resulting in the fusion of 2 distinct genes, Fip1-like 1 (FIP1L1) and platelet-derived growth factor receptor-a (PDGFRA), leading to the FIP1L1-PDGFRA fusion kinase. PDGFR is a membrane-bound tyrosine kinase receptor and the fusion PDGFRA kinase has constitutive/unregulated tyrosine kinase activity. The lymphocytic HES variant is associated with T-cell clones producing interleukin-5 (IL-5) and can evolve into lymphoma. While myeloproliferative and lymphocytic HES are well established and permit elimination of the term, idiopathic, to these varieties, most HES patients do not fall into these categories and are classified as complex.

MalaCards based summary : Hypereosinophilic Syndrome, also known as eosinophilia, is related to hypereosinophilic syndrome, idiopathic and myeloproliferative disorder, chronic, with eosinophilia, and has symptoms including edema, pruritus and nausea and vomiting. An important gene associated with Hypereosinophilic Syndrome is PDGFRA (Platelet Derived Growth Factor Receptor Alpha), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Vancomycin and Nitric Oxide have been mentioned in the context of this disorder. Affiliated tissues include lung, t cells and myeloid, and related phenotypes are Decreased viability and Decreased viability

NIH Rare Diseases : 52 Hypereosinophilic syndrome (HES) refers to a rare group of conditions that are associated with persistent eosinophilia with evidence of organ involvement. Signs and symptoms vary significantly based on which parts of the body are affected. Although any organ system can be involved in HES, the heart, central nervous system , skin, and respiratory tract are the most commonly affected. The condition was originally thought to be "idiopathic" or of unknown cause. However, recent advances in diagnostic testing have allowed a cause to be identified in approximately a quarter of cases. Management varies based on the severity of the condition and whether or not an underlying cause has been identified but generally includes imatinib or corticosteroids as an initial treatment.

Wikipedia : 74 Eosinophilia is a condition in which the eosinophil count in the peripheral blood exceeds 0.5×109/l... more...

Related Diseases for Hypereosinophilic Syndrome

Diseases in the Hypereosinophilic Syndrome family:

Secondary Hypereosinophilic Syndrome Primary Hypereosinophilic Syndrome

Diseases related to Hypereosinophilic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 1445)
# Related Disease Score Top Affiliating Genes
1 hypereosinophilic syndrome, idiopathic 35.0 PDGFRB PDGFRA KIT IL5 IL3 FIP1L1
2 myeloproliferative disorder, chronic, with eosinophilia 34.8 PDGFRB FIP1L1
3 primary hypereosinophilic syndrome 34.7 PDGFRB PDGFRA FIP1L1 FGFR1
4 myeloid and lymphoid neoplasms with eosinophilia and abnormalities of pdgfra, pdgfrb, and fgfr1 34.5 PDGFRB PDGFRA FGFR1
5 pdgfra-associated chronic eosinophilic leukemia 33.2 PDGFRA FIP1L1
6 8p11 myeloproliferative syndrome 33.0 PDGFRB KIT FGFR1
7 aggressive systemic mastocytosis 32.9 PDGFRB PDGFRA KIT IFNA1 FIP1L1
8 paragonimiasis 32.8 RNASE3 IL5 IL13 IGHE
9 filarial elephantiasis 32.8 IL5 IL4 IL13
10 endomyocardial fibrosis 32.7 RNASE3 PRG2 PDGFRB PDGFRA IL5 IL4
11 polycythemia vera 32.5 PDGFRB PDGFRA KIT IL3 IFNA1 FIP1L1
12 myeloid and lymphoid neoplasms associated with fgfr1 abnormalities 32.5 IL3 IL2RA FGFR1
13 intestinal schistosomiasis 32.3 IL5 IL4 IL13
14 neutropenia, severe congenital, 3, autosomal recessive 32.1 IL3 CSF2
15 pulmonary eosinophilia 32.1 RNASE3 PRG2 IL5 IL4 IL13 EPX
16 chronic eosinophilic leukemia 32.1 PDGFRB PDGFRA KIT IL5 IFNA1 FIP1L1
17 chronic fatigue syndrome 32.0 IL5 IL4 IL13 CXCL8
18 myeloid and lymphoid neoplasms associated with pdgfra rearrangement 32.0 PDGFRB PDGFRA KIT FIP1L1 FGFR1 CSF2
19 aplastic anemia 31.7 IL4 IL3 IL2RA IFNA1 CXCL8 CSF2
20 eosinophilic colitis 31.3 RNASE3 PRG2
21 exanthem 31.2 KIT IFNA1 CXCL8 CSF2
22 churg-strauss syndrome 31.2 RNASE3 PRG2 IL5 IL3 CXCL8 CCL11
23 wells syndrome 31.0 RNASE3 IL5 IL2RA CXCL8
24 diarrhea 31.0 IL5 IL3 IL13 IFNA1 CXCL8
25 strongyloidiasis 31.0 IL5 IL3 IL13 IGHE CCL11
26 echinococcosis 31.0 RNASE3 IL5 IGHE
27 gastroesophageal reflux 31.0 IL5 IL4 CXCL8 CCL11
28 erythema multiforme 30.9 IL5 IL4 IL13 CXCL8
29 systemic mastocytosis 30.9 PDGFRB PDGFRA KIT IL2RA IFNA1 FIP1L1
30 cellulitis 30.9 RNASE3 IL5 IL4
31 loeffler endocarditis 30.8 RNASE3 PRG2 PDGFRA IL5 IL3 FIP1L1
32 chronic eosinophilic pneumonia 30.8 RNASE3 IL5 CCL11
33 cytokine deficiency 30.8 IL5 IL13
34 toxocariasis 30.7 RNASE3 IL5 IL4 IGHE FIP1L1
35 myeloproliferative neoplasm 30.7 PDGFRB PDGFRA KIT IL3 IFNA1 FIP1L1
36 urticaria 30.7 RNASE3 PRG2 IL5 IL4 IL13 IGHE
37 myelofibrosis 30.7 PDGFRB KIT IL3 IL2RA IFNA1 FGFR1
38 pancytopenia 30.7 KIT IL3 IL2RA CSF2
39 eosinophilic gastritis 30.6 IL5 IL13 FIP1L1 EPX CCL11
40 cutaneous t cell lymphoma 30.6 IL5 IL4 IL2RA IFNA1
41 mastocytosis, cutaneous 30.6 PDGFRA KIT IL3 FIP1L1
42 thrombocytopenia 30.6 PDGFRA IL4 IL3 IFNA1 CXCL8 CSF2
43 dermatitis 30.6 IL5 IL4 IL13 IGHE CXCL8 CCL11
44 chronic rhinitis 30.6 RNASE3 IL5 IL4 IL13 EPX
45 esophagitis 30.5 IL5 IL4 IL13 CXCL8 CCL11
46 trichinosis 30.5 IL5 IL4 IL3 IL13
47 eosinophilic gastroenteritis 30.5 RNASE3 PRG2 IL5 IL4 IL3 EPX
48 leukemia, chronic myeloid 30.5 PDGFRB PDGFRA KIT IL3 IFNA1 FGFR1
49 kimura disease 30.5 RNASE3 IL5 IL4 IL13 IGHE IGES
50 toxic oil syndrome 30.5 IL5 IL4 IL2RA IGHE

Comorbidity relations with Hypereosinophilic Syndrome via Phenotypic Disease Network (PDN):


Acute Cystitis Bronchitis
Deficiency Anemia Heart Disease
Hypertension, Essential

Graphical network of the top 20 diseases related to Hypereosinophilic Syndrome:



Diseases related to Hypereosinophilic Syndrome

Symptoms & Phenotypes for Hypereosinophilic Syndrome

UMLS symptoms related to Hypereosinophilic Syndrome:


edema, pruritus, nausea and vomiting, abdominal pain, myalgia, chest pain, angina pectoris, constipation, diarrhea, snoring, icterus, coughing, dyspepsia, heartburn, gastrointestinal gas

GenomeRNAi Phenotypes related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

26 (show all 16)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00173-A 9.98 PDGFRA
2 Decreased viability GR00221-A-1 9.98 ABL1 FGFR1 KIT PDGFRA PDGFRB
3 Decreased viability GR00221-A-2 9.98 ABL1 FGFR1
4 Decreased viability GR00221-A-3 9.98 ABL1 PDGFRA PDGFRB
5 Decreased viability GR00221-A-4 9.98 PDGFRA PDGFRB
6 Decreased viability GR00249-S 9.98 PDGFRA
7 Decreased viability GR00301-A 9.98 KIT
8 Decreased viability GR00342-S-1 9.98 ABL1 PDGFRB
9 Decreased viability GR00342-S-2 9.98 ABL1
10 Decreased viability GR00342-S-3 9.98 ABL1
11 Decreased viability GR00386-A-1 9.98 FGFR1
12 Decreased viability GR00402-S-2 9.98 PDGFRA
13 Decreased substrate adherent cell growth GR00193-A-1 9.63 KIT
14 Decreased substrate adherent cell growth GR00193-A-2 9.63 ABL1 KIT
15 Decreased substrate adherent cell growth GR00193-A-4 9.63 ABL1 FGFR1 KIT
16 Increased cell death in breast cancer cell lines (MCF10A, MDA-MB-435) GR00104-A-0 8.92 CSF2 CXCL8 IL2RA PDGFRB

MGI Mouse Phenotypes related to Hypereosinophilic Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.07 ABL1 CCL11 CSF2 EPX FGFR1 IL13
2 digestive/alimentary MP:0005381 10.02 ABL1 FGFR1 IL13 IL2RA IL4 IL5
3 endocrine/exocrine gland MP:0005379 9.97 ABL1 CSF2 FGFR1 IL13 IL2RA IL4
4 immune system MP:0005387 9.97 ABL1 CCL11 CSF2 EPX FGFR1 IL13
5 craniofacial MP:0005382 9.95 ABL1 CSF2 FGFR1 IL4 KIT PDGFRA
6 respiratory system MP:0005388 9.65 ABL1 CCL11 CSF2 IL13 IL2RA IL4
7 skeleton MP:0005390 9.32 ABL1 CSF2 EPX FGFR1 IL13 IL2RA

Drugs & Therapeutics for Hypereosinophilic Syndrome

Drugs for Hypereosinophilic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 256)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vancomycin Approved Phase 4 1404-90-6 14969 441141
2
Nitric Oxide Approved Phase 4 10102-43-9 145068
3
Omalizumab Approved, Investigational Phase 4 242138-07-4
4
Ciclesonide Approved, Investigational Phase 4 141845-82-1, 126544-47-6 444033
5
Diethylcarbamazine Approved, Investigational, Vet_approved Phase 4 90-89-1 3052
6
Cefpirome Approved Phase 4 84957-29-9 5479539
7
Moxifloxacin Approved, Investigational Phase 4 354812-41-2, 151096-09-2 152946
8
Nevirapine Approved Phase 4 129618-40-2 4463
9
Efavirenz Approved, Investigational Phase 4 154598-52-4 64139
10
Stavudine Approved, Investigational Phase 4 3056-17-5 18283
11
Lamivudine Approved, Investigational Phase 4 134678-17-4 60825
12
Ivermectin Approved, Investigational, Vet_approved Phase 4 70288-86-7 6474909
13
Omeprazole Approved, Investigational, Vet_approved Phase 4 73590-58-6 4594
14
Azithromycin Approved Phase 4 83905-01-5 447043 55185
15
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
16
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
17
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
18
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
19
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
20 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
21 Benralizumab Approved, Investigational Phase 4 1044511-01-4
22
tannic acid Approved Phase 4 1401-55-4
23
Benzocaine Approved, Investigational Phase 4 94-09-7, 1994-09-7 2337
24
Budesonide Approved Phase 4 51333-22-3 63006 5281004
25
Sotalol Approved Phase 4 3930-20-9, 959-24-0 5253
26
Lansoprazole Approved, Investigational Phase 4 103577-45-3 3883
27
Dexlansoprazole Approved, Investigational Phase 4 103577-45-3, 138530-94-6 9578005
28
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
29 Fluticasone-Salmeterol Drug Combination Phase 4
30 Sympathomimetics Phase 4
31 Parasympatholytics Phase 4
32 Cholinergic Agents Phase 4
33 Muscarinic Antagonists Phase 4
34 Tiotropium Bromide Phase 4 136310-93-5
35 Cholinergic Antagonists Phase 4
36 Antiemetics Phase 4
37 Immunoglobulins, Intravenous Phase 4
38 Neuroprotective Agents Phase 4
39 Antibiotics, Antitubercular Phase 4
40 Reverse Transcriptase Inhibitors Phase 4
41
Salmeterol xinafoate Phase 4 94749-08-3 56801
42 Immunoglobulin E Phase 4
43 Cytochrome P-450 Enzyme Inhibitors Phase 4
44 Norgestimate, ethinyl estradiol drug combination Phase 4
45 Antiviral Agents Phase 4
46 Anti-Bacterial Agents Phase 4
47 glucocorticoids Phase 4
48 Antineoplastic Agents, Hormonal Phase 4
49 Methylprednisolone Acetate Phase 4
50 Neurotransmitter Agents Phase 4

Interventional clinical trials:

(show top 50) (show all 294)
# Name Status NCT ID Phase Drugs
1 Randomized Double-blind Placebo-controlled Study of add-on Montelukast to Inhaled Budesonide in the Treatment of Nonasthmatic Eosinophilic Bronchitis Unknown status NCT01121016 Phase 4 Montelukast
2 Evaluation of Effectiveness and Safety of Oral Vancomycin in Treatment of Primary Sclerosing Cholangitis. Unknown status NCT02605213 Phase 4 Vancomycin;Placebo
3 The Treatment Effect of Inhaled Corticosteroid and Long-acting beta2 Agonist Combination Versus Long-acting Anti-cholinergic Agent on Stratified COPD Patients Based on the Levels of Exhaled Nitric Oxide Unknown status NCT02546349 Phase 4 fluticasone/salmeterol, tiotropium
4 Phase 4, Randomized Study of Three Months-prednisolone Therapy in the Treatment of Chronic Eosinophilic Pneumonia Completed NCT00632554 Phase 4 prednisolone 0.5 mg/kg/day for three months;prednisolone 0.5 mg/kg/day for six months
5 Small Particle Inhaled Steroids in Refractory Steroid-responsive Asthma Completed NCT01171365 Phase 4 Ciclesonide;Placebo
6 The Effects of Montelukast on Sputum Cells and Inflammatory Markers in Smokers With Asthma Completed NCT00712335 Phase 4 Fluticasone Propionate;Montelukast;Salmeterol
7 A Randomized, Multi-center, Double-blind, Placebo-controlled, Parallel-group Trial to Explore the Effects of 78 Weeks Omalizumab Treatment Given as Add on Therapy on Markers of Airway Inflammation and Remodeling in Patients With Moderate to Severe Persistent Allergic Asthma Receiving Inhaled Corticosteroids and Long Acting Beta-agonists Completed NCT00670930 Phase 4 omalizumab at a dose of 0.016mg/kg/IU/mL;Placebo
8 Efficacy and Safety of Lectranal® in Treatment of Seasonal Allergic Rhinitis Symptoms Completed NCT00460538 Phase 4
9 A Description of Inflammatory Cell Types in Moderate to Severe Pediatric Asthma: Eosinophilic and Non Eosinophilic Sputum Markers While on Anti-IgE Therapy (Xolair) Completed NCT00283504 Phase 4 ANTI-IGE THERAPY (XOLAIR)
10 A Multi-centre, Open Label, Single Arm, 32-week Treatment Study in Subjects With Severe Eosinophilic Asthma Not Optimally Controlled With Current Omalizumab Treatment Who Are Switched From Omalizumab to Mepolizumab 100mg Subcutaneous (Study Number 204471- the OSMO Study) Completed NCT02654145 Phase 4 Mepolizumab 100mg SC;Albuterol/salbutamol MDIs;Omalizumab
11 Plasma and Abscess Fluid Pharmacokinetics of Cefpirome and Moxifloxacin After Single and Multiple Dose Administration Completed NCT00280514 Phase 4 cefpirome and moxifloxacin administration
12 A Retrospective Study to Compare the 3-Year Antiviral Efficacy of Nevirapine and Efavirenz in Combination With D4t and 3tc in 2NN Patients and of Trizivir Versus Trizivir Plus Nevirapine in CHARM Patients Completed NCT00127972 Phase 4 stavudine;lamivudine;nevirapine;efavirenz;trizivir
13 Comparison Between the Post-Treatment Reactions After Single-dose Ivermectin or DEC in Subjects With Loa Loa Infection Completed NCT01593722 Phase 4 Diethylcarbamazine;Ivermectin
14 Assessment of Glucocorticoid Responsiveness and Mechanisms of Resistance in Hypereosinophilic Syndromes Recruiting NCT01524536 Phase 4 prednisone
15 Proton Pump Inhibitor (PPI) Response in Eosinophilic Esophagitis Assessed by Transnasal Endoscopy (TNE) Recruiting NCT04149470 Phase 4 Omeprazole 20mg BID
16 Effects of Azithromycin Treatment on Anti-viral Immunity in Patients With Asthma and COPD - A Randomized Double-blind, Placebo-controlled Trial Recruiting NCT04319705 Phase 4 Azithromycin;Placebo oral tablet
17 A Pragmatic Study to Investigate the Efficacy and Safety of Mepolizumab in Severe Uncontrolled Asthma in Brazil Recruiting NCT04228588 Phase 4 Mepolizumab 100 MG [Nucala]
18 Impact of Benralizumab Treatment on Circulating Dendritic Cells in Patients With Eosinophilic Asthma Not yet recruiting NCT03652376 Phase 4 Benralizumab
19 SHAMAL: A Multicentre, Randomised, Open-Label, Parallel-Group, Active-Controlled, Phase IV Study to Assess the Reduction of Daily Maintenance ICS/LABA Treatment Towards Anti-Inflammatory Reliever Treatment in Patients With Severe Eosinophilic Asthma Treated With Benralizumab Not yet recruiting NCT04159519 Phase 4 Symbicort®;Fasenra®;Ventolin®
20 A Phase 4, Open-label, Single Arm, 24-week, Phase 4 Study to Evaluate the Safety and Efficacy of Mepolizumab 100 mg SC Administered Every 4 Weeks in Indian Participants Aged >=18 Years With Severe Eosinophilic Asthma Requiring Oral Corticosteroid Treatment to Maintain Asthma Control (PRISM) Not yet recruiting NCT04276233 Phase 4 Prednisone/ Prednisolone;Salbutamol
21 A Prospective, Multi-Center, Randomized, Open Label Trial to Determine if a Common Atrial Fibrillation Risk Locus Modulates Differential Response to Antiarrhythmic Drugs Not yet recruiting NCT02347111 Phase 4 Flecainide;Sotalol
22 The Frequency of Eosinophilic Esophagitis in Patients With Heartburn That is Refractory to Proton Pump Inhibitors Terminated NCT01404832 Phase 4 Treat with lansoprazole 30 mg BID for 2 weeks, endoscopic examination with esophageal biopsy for patients with persistent symptoms
23 Effect of Concomitant Mansonella Perstans Microfilaremia on Immune Responses Following Single Dose Praziquantel in Subjects With Schistosomiasis: A Pilot Study Withdrawn NCT02734186 Phase 4 Praziquantel
24 Treatment of Asymptomatic Toxocariasis With Albendazole in Children. A Prospective, Randomized, Placebo-controlled Clinical Trial Unknown status NCT00755560 Phase 3 Albendazole;Placebo
25 The Treatment of Down Syndrome Children With Acute Myeloid Leukemia (AML) and Myelodysplastic Syndromes (MDS) Under the Age of 4 Years Unknown status NCT00369317 Phase 3 asparaginase;daunorubicin hydrochloride;cytarabine;thioguanine;etoposide
26 Prednisone in Chronic Rhinosinusitis Without Nasal Polyps. A Double-blind, Randomized, Placebo-controlled Trial Unknown status NCT02367118 Phase 3 Prednisone;Placebo
27 The Efficacy of Mepolizumab Treatment on Rhinovirus Induced Asthma Exacerbations Unknown status NCT01520051 Phase 3 Mepolizumab;Placebo
28 Vitamin D Supplementation as an Adjuvant Therapy for Specific Immunotherapy in Patients With Allergic Rhinitis. Unknown status NCT02738619 Phase 2, Phase 3 Vitamin D3;placebo
29 A Multicenter, Double-blind, Placebo-controlled, Study to to Evaluate the Corticosteroid- Sparing Effects of Mepolizumab in Subjects With Hypereosinophilic Syndromes (HES) and Evaluate Efficacy and Safety of Mepolizumab in Controlling the Clinical Signs and Symptoms of Subjects With HES Completed NCT00086658 Phase 3 mepolizumab
30 Study 200622: A Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of Mepolizumab in the Treatment of Adolescent and Adult Subjects With Severe Hypereosinophilic Syndrome Completed NCT02836496 Phase 3 Mepolizumab 300 mg;Placebo matching mepolizumab;Active OCS capsules (5 mg prednisolone or prednisone);Placebo matching OCS capsules
31 A Multi-centre, Open-label Extension, Safety Study to Describe the Long-term Clinical Experience of Mepolizumab in Participants With Hypereosinophilic Syndrome (HES) From Study 200622 Completed NCT03306043 Phase 3 Mepolizumab
32 INSPIRE: An Internet-based RCT for Long-term Survivors of Hematopoietic Stem Cell Transplantation Completed NCT00799461 Phase 3
33 A Randomized Double Blinded Trial of Topical Caphosol to Prevent Oral Mucositis in Children Undergoing Hematopoietic Stem Cell Transplantation Completed NCT01305200 Phase 3 supersaturated calcium phosphate rinse
34 An Open-label Extension Study to Assess Efficacy, Safety and Tolerability of Canakinumab and the Efficacy and Safety of Childhood Vaccinations in Patients With Cryopyrin Associated Periodic Syndromes (CAPS) Completed NCT01576367 Phase 3
35 A One-year Open-label, Multicenter Trial to Assess Efficacy, Safety and Tolerability of Canakinumab (ACZ885) and the Efficacy and Safety of Childhood Vaccinations in Patients Aged 4 Years or Younger With Cryopyrin Associated Periodic Syndromes (CAPS) Completed NCT01302860 Phase 3 ACZ885
36 A 12-Month, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Reslizumab (3.0 mg/kg) in the Reduction of Clinical Asthma Exacerbations in Patients (12-75 Years of Age) With Eosinophilic Asthma Completed NCT01285323 Phase 3 Reslizumab;Placebo
37 A 16-Week, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Reslizumab (0.3 or 3.0 mg/kg) as Treatment for Patients (12-75 Years of Age) With Eosinophilic Asthma Completed NCT01270464 Phase 3 Reslizumab;Placebo
38 A 16-Week, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Reslizumab (3.0 mg/kg) Treatment in Patients With Moderate to Severe Asthma Completed NCT01508936 Phase 3 Reslizumab;Placebo
39 Phase III Randomized Study of Induction Chemotherapy With or Without MDR-Modulation With PSC-833 (NSC # 648265, IND # 41121) Followed by Cytogenetic Risk-Adapted Intensification Therapy Followed by Immunotherapy With rIL-2 (NSC # 373364, IND # 1969) vs. Observation in Previously Untreated Patients With AML &lt; 60 Years Completed NCT00006363 Phase 3 cytarabine;daunorubicin hydrochloride;etoposide;valspodar;busulfan
40 Route of Administration of Anti-IL5 Monoclonal Antibody in Prednisone-dependent Eosinophilic Asthma Completed NCT02559791 Phase 2, Phase 3 Placebo
41 GPIAG and Leicester Asthma and Dysfunctional Breathing (GLAD) Study: a Randomised Controlled Study Completed NCT00515840 Phase 3
42 An Open-label, Efficacy and Safety Study of Canakinumab (Anti-interleukin-1β Monoclonal Antibody) Administered for 6 Months (24 Weeks) in Japanese Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease, Followed by an Extension Phase to Provide Canakinumab to Study Patients Until it is Approved and Marketed in Japan Completed NCT00991146 Phase 3 canakinumab
43 An Open-label, Long-term Safety and Efficacy Study of ACZ885 (Anti-interleukin-1β Monoclonal Antibody) Administered for at Least 6 Months in Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease Completed NCT00685373 Phase 3 Canakinumab (ACZ885)
44 Mepolizumab in COPD With Eosinophilic Bronchitis: A Randomized Clinical Trial Completed NCT01463644 Phase 3 Mepolizumab;placebo
45 A Randomised, Double-blind, Placebo-controlled, Parallel-group, Multi-centre 24-week Study to Evaluate the Efficacy and Safety of Mepolizumab Adjunctive Therapy in Subjects With Severe Eosinophilic Asthma on Markers of Asthma Control Completed NCT02281318 Phase 3 Placebo;SOC
46 A Three-part,Multicenter Study,With a Randomized,Double-blind,Placebo Controlled,Withdrawal Design in Part II to Assess Efficacy,Safety,and Tolerability of ACZ885(Anti-interleukin-1beta Monoclonal Antibody)in Patients With Muckle-Wells Syndrome Completed NCT00465985 Phase 3 ACZ885;Placebo
47 A 12-Month, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Reslizumab (3.0 mg/kg) in the Reduction of Clinical Asthma Exacerbations in Patients (12-75 Years of Age) With Eosinophilic Asthma Completed NCT01287039 Phase 3 Reslizumab;Placebo
48 A Multi-center, Randomized, Double-blind, Placebo Controlled, Parallel Group Study to Compare Cessation Versus Continuation of Long-term Mepolizumab Treatment in Patients With Severe Eosinophilic Asthma (201810) Completed NCT02555371 Phase 3 Placebo
49 A Double-blind Placebo Controlled Clinical Trial to Evaluate the Effects on Asthma Control of Rabeprazole Given Twice Daily in Subjects With Asthma. Completed NCT00214552 Phase 3 rabeprazole
50 Particulate Reduction Education in City Homes (PREACH) Completed NCT00466024 Phase 3

Search NIH Clinical Center for Hypereosinophilic Syndrome

Inferred drug relations via UMLS 71 / NDF-RT 50 :


hydroxyurea
imatinib
Imatinib mesylate
Interferon Alfa-2a
Interferon Alfa-2b
INTERFERON ALFA-3N,HUMAN LEUKOCYTE DERIVED
interferon alfacon-1
Interferon gamma-1b
Interferons
peginterferon alfa-2a
peginterferon alfa-2b
Recombinant interferon beta-1a
Recombinant interferon beta-1b

Cochrane evidence based reviews: eosinophilia

Genetic Tests for Hypereosinophilic Syndrome

Genetic tests related to Hypereosinophilic Syndrome:

# Genetic test Affiliating Genes
1 Eosinophilia 29

Anatomical Context for Hypereosinophilic Syndrome

MalaCards organs/tissues related to Hypereosinophilic Syndrome:

40
Lung, T Cells, Myeloid, Skin, Testes, Bone, Liver

Publications for Hypereosinophilic Syndrome

Articles related to Hypereosinophilic Syndrome:

(show top 50) (show all 20936)
# Title Authors PMID Year
1
Early phase resolution of mucosal eosinophilic inflammation in allergic rhinitis. 54 61
20459697 2010
2
Delayed-type asthmatic response to bronchial challenge with allergen, I: clinical features. 61 54
20486329 2010
3
IL-13 Gene Polymorphisms are Associated With Rhinosinusitis and Eosinophilic Inflammation in Aspirin Intolerant Asthma. 54 61
20358028 2010
4
Thrombotic storm in Kimura disease. 54 61
19468829 2010
5
Airway wall expression of OX40/OX40L and interleukin-4 in asthma. 54 61
20139223 2010
6
Allergen-induced, eotaxin-rich, proangiogenic bone marrow progenitors: a blood-borne cellular envoy for lung eosinophilia. 54 61
20227754 2010
7
Critical role of the platelet-derived growth factor receptor (PDGFR) beta transmembrane domain in the TEL-PDGFRbeta cytosolic oncoprotein. 54 61
20164181 2010
8
Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. 54 61
20053713 2010
9
Vernal keratoconjunctivitis--a rare but serious comorbidity of allergic rhinitis and eustachian tube dysfunction. 61 54
19900723 2010
10
Intranasal immunization of mice with a bovine respiratory syncytial virus vaccine induces superior immunity and protection compared to those by subcutaneous delivery or combinations of intranasal and subcutaneous prime-boost strategies. 61 54
19864487 2010
11
Hyper-IgE syndrome with STAT3 mutation: a case report in Mainland China. 61 54
20490271 2010
12
Effects of omalizumab on markers of inflammation in patients with allergic asthma. 61 54
19839977 2009
13
Concomitant occurrence of kimura disease and mycosis fungoides in a Lebanese woman: significance and response to rituximab. 61 54
19786854 2009
14
A diagnostically difficult case of chronic myeloid neoplasm with eosinophilia and abnormalities of PDGFRA effectively treated with imatinib in accelerated phase: case report. 54 61
20010473 2009
15
A low dose of Mycoplasma pneumoniae infection enhances an established allergic inflammation in mice: the role of the prostaglandin E2 pathway. 61 54
19552640 2009
16
Triptolide abrogates oncogene FIP1L1-PDGFRalpha addiction and induces apoptosis in hypereosinophilic syndrome. 54 61
19671059 2009
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Myeloproliferative neoplasms: contemporary diagnosis using histology and genetics. 54 61
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Kimura's disease presenting with a giant suspensory tumor and associated with membranoproliferative glomerulonephritis. 61 54
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Is serum total IgE levels a good predictor of allergies in children? 61 54
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Leukotriene E4-induced pulmonary inflammation is mediated by the P2Y12 receptor. 61 54
19822647 2009
21
Recurrent giant chalazia in hyperimmunoglobulin E (Job's) syndrome. 54 61
18528638 2009
22
Changed concepts and definitions of myeloproliferative neoplasms (MPN), myelodysplastic syndromes (MDS) and myelodysplastic/myeloproliferative neoplasms (MDS/MPN) in the updated 2008 WHO classification. 61 54
20309429 2009
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Innate cells and T helper 2 cell immunity in airway inflammation. 61 54
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T-helper type 2-driven inflammation defines major subphenotypes of asthma. 54 61
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Recent breakthroughs in the understanding and management of chronic eosinophilic leukemia. 61 54
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Similar colds in subjects with allergic asthma and nonatopic subjects after inoculation with rhinovirus-16. 54 61
19596142 2009
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Formulation of bovine respiratory syncytial virus fusion protein with CpG oligodeoxynucleotide, cationic host defence peptide and polyphosphazene enhances humoral and cellular responses and induces a protective type 1 immune response in mice. 54 61
19386785 2009
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Blockade of CCR4 in a humanized model of asthma reveals a critical role for DC-derived CCL17 and CCL22 in attracting Th2 cells and inducing airway inflammation. 61 54
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Clinical significance of eosinophilic cationic protein levels in nasal secretions of patients with nasal polyposis. 54 61
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Production of interleukin-10 in asthmatic children after Beta-1-3-glucan. 61 54
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A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome. 54 61
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Imatinib has limited therapeutic activity for hypereosinophilic syndrome patients with unknown or negative PDGFRalpha mutation status. 61 54
19013640 2009
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Eosinophilia-associated muscle disorders: an immunohistological study with tissue localisation of major basic protein in distinct clinicopathological forms. 54 61
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Pathogenesis of Churg-Strauss syndrome: recent insights. 54 61
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VCAM-1 and eosinophilia in diffuse sino-nasal polyps. 54 61
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Reciprocal regulation of iNOS and PARP-1 during allergen-induced eosinophilia. 54 61
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Molecular drug targets in myeloproliferative neoplasms: mutant ABL1, JAK2, MPL, KIT, PDGFRA, PDGFRB and FGFR1. 61 54
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Five years since the discovery of FIP1L1-PDGFRA: what we have learned about the fusion and other molecularly defined eosinophilias. 61 54
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TARC and IL-5 expression correlates with tissue eosinophilia in peripheral T-cell lymphomas. 54 61
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CD4+CD25+ regulatory T cells reverse established allergic airway inflammation and prevent airway remodeling. 54 61
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Variations for Hypereosinophilic Syndrome

ClinVar genetic disease variations for Hypereosinophilic Syndrome:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 JAK2 , ZBTB20 t(3;9)(q13.31;p24.1)Translocation Likely pathogenic 869413 3:114069121-114069122

Expression for Hypereosinophilic Syndrome

Search GEO for disease gene expression data for Hypereosinophilic Syndrome.

Pathways for Hypereosinophilic Syndrome

Pathways related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

(show top 50) (show all 51)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.95 RNASE3 PRG2 PDGFRB PDGFRA KIT IL5
2
Show member pathways
13.84 PRG2 PDGFRB PDGFRA KIT IL5 IL4
3
Show member pathways
13.72 PDGFRB PDGFRA KIT IL5 IL4 IL3
4
Show member pathways
13.59 PDGFRB PDGFRA KIT IL5 IL4 IL3
5
Show member pathways
13.53 PDGFRB PDGFRA KIT IL5 IL3 IL2RA
6
Show member pathways
13.44 PDGFRB PDGFRA KIT IL5 IL4 IL3
7
Show member pathways
13.28 PDGFRB PDGFRA KIT IL5 IL4 IL3
8
Show member pathways
13.28 PDGFRB PDGFRA KIT IL5 IL4 IL3
9
Show member pathways
13.1 PRG2 PDGFRB PDGFRA KIT IL3 IL2RA
10
Show member pathways
13.09 PDGFRB PDGFRA KIT IL4 IL3 IL2RA
11
Show member pathways
13.04 PDGFRB PDGFRA KIT IL4 IL3 IL2RA
12
Show member pathways
12.93 PDGFRB PDGFRA KIT IL3 FGFR1 CSF2
13
Show member pathways
12.74 PDGFRB PDGFRA KIT FGFR1 ABL1
14
Show member pathways
12.66 IL5 IL4 IL3 IL13 IGHE CSF2
15 12.66 PDGFRB PDGFRA KIT IL5 IL4 IL3
16 12.59 KIT IL4 IL3 IL2RA IFNA1
17
Show member pathways
12.52 PDGFRB PDGFRA IL5 IL2RA IL13 FGFR1
18
Show member pathways
12.47 PDGFRB PDGFRA IL5 IL4 IL3 IL2RA
19
Show member pathways
12.41 IL5 IL4 IL13 CXCL8 CSF2 CCL11
20
Show member pathways
12.39 PDGFRB PDGFRA KIT CXCL8
21
Show member pathways
12.39 PDGFRB PDGFRA KIT IL3 FGFR1
22 12.2 PDGFRB PDGFRA KIT FGFR1 ABL1
23
Show member pathways
12.18 RNASE3 PRG2 PDGFRA IL5 IL4 IL3
24
Show member pathways
12.17 IL5 IL3 IL2RA CSF2
25 12.1 KIT IL5 IL4 IL3 IL2RA CSF2
26 12.07 IL4 IL13 IGHE CXCL8 CCL11
27 12.01 KIT IL5 IL4 IL3 IL2RA CSF2
28
Show member pathways
11.94 PDGFRB PDGFRA KIT IL5 IL4 IL3
29 11.9 IL5 IL4 IL13 CXCL8 CSF2
30 11.88 KIT IL5 IL4 IL2RA IL13
31
Show member pathways
11.84 IL5 IL4 IL3 IL2RA CXCL8 CSF2
32 11.83 PDGFRB PDGFRA FGFR1
33
Show member pathways
11.8 PDGFRB KIT FGFR1
34 11.8 PDGFRB PDGFRA KIT FGFR1
35 11.75 IL5 IL4 IGHE CCL11
36 11.73 IL5 IL4 IL13 CXCL8 CSF2 CCL11
37
Show member pathways
11.64 PDGFRB PDGFRA KIT ABL1
38 11.61 IL4 IL13 CXCL8 CSF2
39 11.61 IL5 IL4 IL2RA IL13 CCL11
40 11.55 IL5 IL3 CSF2
41 11.54 IL2RA CXCL8 CCL11
42 11.52 PDGFRB PDGFRA FGFR1
43 11.49 PDGFRB PDGFRA FGFR1
44
Show member pathways
11.39 PDGFRB PDGFRA KIT
45 11.37 IL5 IL4 IL2RA
46 11.36 IL5 IL4 IL3 IL13 CSF2
47 11.35 IL5 IL4 IL13
48 11.25 IL5 IL4 IL13
49 10.94 PRG2 PDGFRB PDGFRA KIT IL5 IL4
50 10.89 IL5 IL4 IL13

GO Terms for Hypereosinophilic Syndrome

Cellular components related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.7 RNASE3 KIT IL5 IL4 IL3 IL13
2 receptor complex GO:0043235 9.56 PDGFRB PDGFRA KIT FGFR1
3 external side of plasma membrane GO:0009897 9.55 PDGFRA KIT IL2RA IL13 IGHE
4 extracellular region GO:0005576 9.44 RNASE3 PRG2 IL5 IL4 IL3 IL13

Biological processes related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

(show all 42)
# Name GO ID Score Top Affiliating Genes
1 protein phosphorylation GO:0006468 10.09 PDGFRB PDGFRA KIT FGFR1 CCL11 ABL1
2 defense response to bacterium GO:0042742 9.98 RNASE3 PRG2 IGHE EPX
3 inflammatory response GO:0006954 9.97 KIT IL5 IL2RA IL13 CXCL8 CCL11
4 positive regulation of cell proliferation GO:0008284 9.97 PDGFRB PDGFRA KIT IL5 IL4 IL3
5 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.96 PDGFRB PDGFRA CCL11 ABL1
6 regulation of cell proliferation GO:0042127 9.95 KIT FGFR1 CSF2 ABL1
7 positive regulation of protein kinase B signaling GO:0051897 9.93 PDGFRB PDGFRA KIT FGFR1
8 chemotaxis GO:0006935 9.93 PDGFRB PDGFRA CXCL8 CCL11
9 positive regulation of cell migration GO:0030335 9.92 PDGFRB PDGFRA KIT IL4 CCL11
10 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.88 PDGFRB PDGFRA KIT FGFR1
11 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.86 IL5 IL3 ABL1
12 cell chemotaxis GO:0060326 9.85 PDGFRB PDGFRA KIT
13 protein autophosphorylation GO:0046777 9.85 PDGFRB PDGFRA KIT FGFR1 ABL1
14 B cell differentiation GO:0030183 9.84 KIT IL4 IFNA1
15 hematopoietic progenitor cell differentiation GO:0002244 9.84 PDGFRB PDGFRA KIT
16 positive regulation of MAP kinase activity GO:0043406 9.82 PDGFRB KIT FGFR1
17 phosphatidylinositol-mediated signaling GO:0048015 9.81 PDGFRB PDGFRA FGFR1
18 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.81 PDGFRB PDGFRA KIT FGFR1
19 peptidyl-tyrosine phosphorylation GO:0018108 9.8 PDGFRB PDGFRA KIT FGFR1 ABL1
20 skeletal system morphogenesis GO:0048705 9.78 PDGFRB PDGFRA FGFR1
21 positive regulation of kinase activity GO:0033674 9.78 PDGFRB PDGFRA KIT FGFR1
22 positive regulation of B cell proliferation GO:0030890 9.77 IL5 IL4 IL13
23 negative regulation of endothelial cell apoptotic process GO:2000352 9.77 IL4 IL13 ABL1
24 MAPK cascade GO:0000165 9.76 PDGFRB PDGFRA KIT IL5 IL3 IL2RA
25 platelet-derived growth factor receptor signaling pathway GO:0048008 9.72 PDGFRB PDGFRA ABL1
26 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.72 KIT IL4 IL3 IL13 CSF2
27 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.71 PDGFRB PDGFRA KIT
28 dendritic cell differentiation GO:0097028 9.69 IL4 CSF2
29 positive regulation of podosome assembly GO:0071803 9.68 IL5 CSF2
30 positive regulation of interleukin-10 secretion GO:2001181 9.67 IL4 IL13
31 positive regulation of mast cell degranulation GO:0043306 9.67 IL4 IL13
32 response to fluid shear stress GO:0034405 9.66 PDGFRB CSF2
33 retina vasculature development in camera-type eye GO:0061298 9.65 PDGFRB PDGFRA
34 immune response GO:0006955 9.65 PRG2 IL5 IL4 IL3 IL2RA IL13
35 platelet-derived growth factor receptor-beta signaling pathway GO:0035791 9.62 PDGFRB ABL1
36 mast cell chemotaxis GO:0002551 9.62 KIT CCL11
37 macrophage activation GO:0042116 9.61 IL4 IL13 CSF2
38 negative regulation of complement-dependent cytotoxicity GO:1903660 9.58 IL4 IL13
39 metanephric glomerular capillary formation GO:0072277 9.57 PDGFRB PDGFRA
40 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.56 PDGFRB PDGFRA
41 positive regulation of phospholipase C activity GO:0010863 9.56 PDGFRB PDGFRA KIT FGFR1
42 cytokine-mediated signaling pathway GO:0019221 9.36 KIT IL5 IL4 IL3 IL2RA IL13

Molecular functions related to Hypereosinophilic Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein kinase activity GO:0004672 9.88 PDGFRB PDGFRA KIT FGFR1 ABL1
2 growth factor activity GO:0008083 9.67 IL5 IL4 IL3 CSF2
3 growth factor binding GO:0019838 9.58 PDGFRB PDGFRA KIT
4 SH2 domain binding GO:0042169 9.5 KIT FGFR1 ABL1
5 platelet-derived growth factor binding GO:0048407 9.46 PDGFRB PDGFRA
6 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.46 PDGFRB PDGFRA KIT FGFR1
7 vascular endothelial growth factor binding GO:0038085 9.43 PDGFRB PDGFRA
8 cytokine receptor binding GO:0005126 9.43 IL4 IL13 IFNA1
9 protein tyrosine kinase activity GO:0004713 9.35 PDGFRB PDGFRA KIT FGFR1 ABL1
10 cytokine activity GO:0005125 9.23 IL5 IL4 IL3 IL13 IFNA1 CXCL8

Sources for Hypereosinophilic Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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