MCID: MCR225
MIFTS: 45

Macrophage Activation Syndrome

Categories: Blood diseases, Bone diseases, Rare diseases

Aliases & Classifications for Macrophage Activation Syndrome

MalaCards integrated aliases for Macrophage Activation Syndrome:

Name: Macrophage Activation Syndrome 53 59 73

Classifications:



External Ids:

Orphanet 59 ORPHA158061
MESH via Orphanet 45 D055501
UMLS via Orphanet 74 C1096155
UMLS 73 C1096155

Summaries for Macrophage Activation Syndrome

MalaCards based summary : Macrophage Activation Syndrome is related to griscelli syndrome and griscelli syndrome, type 2. An important gene associated with Macrophage Activation Syndrome is RAB27A (RAB27A, Member RAS Oncogene Family), and among its related pathways/superpathways are Innate Immune System and Legionellosis. The drugs leucovorin and Methotrexate have been mentioned in the context of this disorder. Affiliated tissues include bone, liver and bone marrow, and related phenotypes are Mildly decreased CFP-tsO45G cell surface transport and hematopoietic system

Wikipedia : 76 Macrophage-activation syndrome (MAS) is a severe, potentially life-threatening, complication of several... more...

Related Diseases for Macrophage Activation Syndrome

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

(show top 50) (show all 125)
# Related Disease Score Top Affiliating Genes
1 griscelli syndrome 32.2 RAB27A UNC13D
2 griscelli syndrome, type 2 32.2 RAB27A UNC13D
3 systemic onset juvenile idiopathic arthritis 30.5 PRF1 TNF
4 hemophagocytic lymphohistiocytosis 30.0 PRF1 RAB27A TNF UNC13D
5 rheumatoid arthritis 29.7 ANGPT1 HMGB1 IRF5 TNF
6 rheumatic disease 29.6 CD163 HMGB1 TNF
7 spondyloarthropathy 1 29.3 CD163 TNF
8 malaria 29.2 ANGPT1 ANGPT2 CD163 TNF
9 autoinflammation with infantile enterocolitis 11.4
10 arthritis 10.6
11 lupus erythematosus 10.4
12 systemic lupus erythematosus 10.4
13 adult-onset still's disease 10.3
14 juvenile rheumatoid arthritis 10.2
15 rheumatoid arthritis, systemic juvenile 10.2
16 kawasaki disease 10.2
17 pyogenic granuloma 10.1 ANGPT1 ANGPT2
18 twin-to-twin transfusion syndrome 10.1 ANGPT1 ANGPT2
19 lipodermatosclerosis 10.1 ANGPT1 ANGPT2
20 placenta accreta 10.1 ANGPT1 ANGPT2
21 hemophagocytic lymphohistiocytosis, familial, 4 10.1 RAB27A UNC13D
22 endophthalmitis 10.1 HMGB1 PRF1
23 epileptic encephalopathy, childhood-onset 10.1 ANGPT1 ANGPT2
24 dermatomyositis 10.1
25 chediak-higashi syndrome 10.1 RAB27A UNC13D
26 listeriosis 10.0 PRF1 TNF
27 spondylarthropathy 10.0 CD163 TNF
28 plasmodium vivax malaria 9.9 ANGPT2 TNF
29 bacterial vaginosis 9.9 PRF1 TNF
30 staphylococcal toxic shock syndrome 9.9 HMGB1 TNF
31 hemorrhagic fever with renal syndrome 9.9 CD163 TNF
32 anca-associated vasculitis 9.9 HMGB1 TNF
33 autoimmune disease 9.9
34 epstein-barr virus-associated gastric carcinoma 9.9 IRF5 TNF
35 inclusion conjunctivitis 9.9 HMGB1 TNF
36 churg-strauss syndrome 9.9 HMGB1 TNF
37 suppurative cholangitis 9.9 HMGB1 TNF
38 rasmussen encephalitis 9.9 HMGB1 TNF
39 limb ischemia 9.9 ANGPT2 TNF
40 idiopathic neutropenia 9.9 HMGB1 TNF
41 childhood type dermatomyositis 9.9
42 chronic granulomatous disease 9.9
43 hepatitis 9.9
44 lymphoma 9.9
45 panniculitis 9.9
46 influenza 9.9
47 vasculitis 9.9
48 adult respiratory distress syndrome 9.8 ANGPT2 TNF
49 lymphatic system disease 9.8 RAB27A TNF UNC13D
50 appendicitis 9.8 HMGB1 TNF

Graphical network of the top 20 diseases related to Macrophage Activation Syndrome:



Diseases related to Macrophage Activation Syndrome

Symptoms & Phenotypes for Macrophage Activation Syndrome

GenomeRNAi Phenotypes related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Mildly decreased CFP-tsO45G cell surface transport GR00360-A-1 9.17 ANGPT1 ANGPT2 CD163 HMGB1 NLRC4 PRF1

MGI Mouse Phenotypes related to Macrophage Activation Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 CD163 IRF5 NLRC4 PRF1 RAB27A TNF
2 homeostasis/metabolism MP:0005376 9.56 ANGPT1 ANGPT2 CD163 IRF5 PRF1 RAB27A
3 immune system MP:0005387 9.28 ANGPT1 ANGPT2 CD163 IRF5 NLRC4 PRF1

Drugs & Therapeutics for Macrophage Activation Syndrome

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

(show top 50) (show all 65)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
leucovorin Approved Phase 3 58-05-9 6006 143
2
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
3
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
4 Peripheral Nervous System Agents Phase 3,Phase 1,Phase 2
5 Analgesics Phase 3,Phase 1,Phase 2
6 Analgesics, Non-Narcotic Phase 3,Phase 1,Phase 2
7 Folate Phase 3
8 Anti-Inflammatory Agents, Non-Steroidal Phase 3
9 Anti-Inflammatory Agents Phase 3,Phase 1,Phase 2
10 Folic Acid Antagonists Phase 3
11 Vitamin B Complex Phase 3
12 Dermatologic Agents Phase 3
13 Nucleic Acid Synthesis Inhibitors Phase 3
14 Immunosuppressive Agents Phase 3,Phase 1,Phase 2
15 Antimetabolites Phase 3,Phase 1,Phase 2
16 Immunologic Factors Phase 3,Phase 1,Phase 2
17 Antirheumatic Agents Phase 3,Phase 2,Phase 1
18 Antimetabolites, Antineoplastic Phase 3,Phase 1,Phase 2
19 Vitamin B9 Phase 3
20 Krestin Phase 3
21 Pharmaceutical Solutions Phase 3,Phase 2,Phase 1
22 Interleukin 1 Receptor Antagonist Protein Phase 3,Phase 2,Phase 1
23
Aldesleukin Approved Phase 1, Phase 2 85898-30-2, 110942-02-4
24
Fludarabine Approved Phase 1, Phase 2,Phase 2,Early Phase 1 75607-67-9, 21679-14-1 30751
25
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 6055-19-2, 50-18-0 2907
26
Prednisolone Approved, Vet_approved Phase 1, Phase 2 50-24-8 5755
27
Methylprednisolone Approved, Vet_approved Phase 1, Phase 2 83-43-2 6741
28
Thiotepa Approved, Investigational Phase 1, Phase 2 52-24-4 5453
29
Tacrolimus Approved, Investigational Phase 1, Phase 2 104987-11-3 445643 439492
30
Prednisolone phosphate Approved, Vet_approved Phase 1, Phase 2 302-25-0
31
Methylprednisolone hemisuccinate Approved Phase 1, Phase 2 2921-57-5
32
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 6436030 5284616
33
Melphalan Approved Phase 1, Phase 2,Early Phase 1 148-82-3 4053 460612
34
Busulfan Approved, Investigational Phase 1, Phase 2,Early Phase 1 55-98-1 2478
35
alemtuzumab Approved, Investigational Phase 1, Phase 2,Early Phase 1 216503-57-0
36
Prednisolone hemisuccinate Experimental Phase 1, Phase 2 2920-86-7
37 Anti-HIV Agents Phase 1, Phase 2
38 Antiviral Agents Phase 1, Phase 2,Phase 2
39 Anti-Infective Agents Phase 1, Phase 2,Phase 2
40 Anti-Retroviral Agents Phase 1, Phase 2
41 Interleukin-2 Phase 1, Phase 2
42 Interferon-gamma Phase 2
43 Immunoglobulins Phase 2
44 Antibodies Phase 2
45 Antibodies, Monoclonal Phase 2
46 interferons Phase 2
47 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1, Phase 2
48 Hormone Antagonists Phase 1, Phase 2
49 Calcineurin Inhibitors Phase 1, Phase 2
50 Prednisolone acetate Phase 1, Phase 2

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 A Long-Term Extension Study of RoActemra/Actemra (Tocilizumab) in Patients With Juvenile Idiopathic Arthritis Who Completed WA19977 Core Study Completed NCT01667471 Phase 3 tocilizumab [RoActemra/Actemra]
2 A Study of RoActemra/Actemra (Tocilizumab) in Patients With Active Systemic Juvenile Idiopathic Arthritis (JIA) Completed NCT00642460 Phase 3 tocilizumab [RoActemra/Actemra];Placebo;Non-steroidal anti-inflammatory drugs (NSAIDs);methotrexate;corticosteroids
3 Therapeutic Use of Tadekinig Alfa in NLRC4 Mutation and XIAP Deficiency Recruiting NCT03113760 Phase 3 Tadekinig alfa
4 A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still's Disease (SJIA and AOSD) Recruiting NCT03265132 Phase 3 Placebo
5 Low-dose IL-2 ( Interleukin-2) Treatment in Macrophage Activation Syndrome(MAS) Unknown status NCT02569463 Phase 1, Phase 2 Interleukin-2
6 A Study to Investigate the Safety and Efficacy of an Anti-IFN-gamma mAb in Children With Systemic Juvenile Idiopathic Arthritis (sJIA) Developing Macrophage Activation Syndrome/ Secondary Hemophagocytic Lymphohistiocytosis (MAS/sHLH) Recruiting NCT03311854 Phase 2 NI-0501
7 A Trial of Validation and Restoration of Immune Dysfunction in Severe Infections and Sepsis Recruiting NCT03332225 Phase 2 Anakinra;Recombinant human interferon-gamma;Placebo
8 CD34+ (Malignant) Stem Cell Selection for Patients Receiving Allogenic Stem Cell Transplant Recruiting NCT02061800 Phase 1, Phase 2 Thiotepa;Cyclophosphamide;Alemtuzumab;Tacrolimus;Melphalan;Busulfan;Fludarabine;Methylprednisolone
9 CART19 in Adult Patients With Minimal Residual Disease During Upfront Treatment for ALL Active, not recruiting NCT02935543 Phase 2
10 Treatment of Macrophage Activation Syndrome (MAS) With Anakinra Recruiting NCT02780583 Phase 1 kineret;placebo
11 CAR19 Donor Lymphocytes for Relapsed CD19+ Malignancies Following Allogeneic Transplantation Recruiting NCT02893189 Phase 1
12 New Candidate Criteria for Diagnosis of Macrophage Activation Syndrome Unknown status NCT01095146
13 CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation Recruiting NCT01966367 Early Phase 1
14 Pharmacovigilance in Juvenile Idiopathic Arthritis Patients Treated With Biologic Agents and/or Methotrexate Recruiting NCT01399281
15 Characterization of the Inflammatory Profile of Patients With Macrophage Activation Syndrome Secondary to Bacterial Sepsis Not yet recruiting NCT03721809 Not Applicable

Search NIH Clinical Center for Macrophage Activation Syndrome

Genetic Tests for Macrophage Activation Syndrome

Anatomical Context for Macrophage Activation Syndrome

MalaCards organs/tissues related to Macrophage Activation Syndrome:

41
Bone, Liver, Bone Marrow, T Cells, B Cells, Monocytes, Placenta

Publications for Macrophage Activation Syndrome

Articles related to Macrophage Activation Syndrome:

(show top 50) (show all 346)
# Title Authors Year
1
Adult-Onset Still Disease and Macrophage Activation Syndrome Following Chikungunya and Hepatitis E Coinfection. ( 29485548 )
2018
2
Unopposed IL-18 signaling leads to severe TLR9-induced macrophage activation syndrome in mice. ( 29295842 )
2018
3
H-ferritin and proinflammatory cytokines are increased in the bone marrow of patients affected by macrophage activation syndrome. ( 28960260 )
2018
4
Autoinflammatory diseases: Free IL-18 causes macrophage activation syndrome. ( 29386593 )
2018
5
Interleukin-18 diagnostically distinguishes and pathogenically promotes human and murine macrophage activation syndrome. ( 29326099 )
2018
6
Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus. ( 29451069 )
2018
7
Novel UNC13D intronic variant disrupting a NFI_B enhancer in a patient with recurrent macrophage activation syndrome and systemic juvenile idiopathic arthritis. ( 29409136 )
2018
8
Hypofibrinogenemia Is Associated With Poor Outcome and Secondary Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome in Pediatric Severe Sepsis. ( 29470247 )
2018
9
Macrophage activation syndrome associated with griscelli syndrome type 2: case report and review of literature. ( 29875956 )
2018
10
Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. ( 29481673 )
2018
11
Macrophage Activation Syndrome in Patients Affected by Adult-onset Still Disease: Analysis of Survival Rates and Predictive Factors in the Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale Cohort. ( 29657144 )
2018
12
Systemic lupus erythematosus presenting to haematology with pancytopenia and features of macrophage activation syndrome. ( 29467122 )
2018
13
Macrophage Activation Syndrome (MAS) in a Recently Released Prisoner with Systemic Lupus Erythematosus (SLE). ( 29930239 )
2018
14
Features, Treatment and Outcomes of Macrophage Activation Syndrome in Childhood-onset Systemic Lupus Erythematosus. ( 29342508 )
2018
15
Systemic juvenile idiopathic arthritis and macrophage activation syndrome: update on pathogenesis and treatment. ( 29870499 )
2018
16
Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center. ( 29976044 )
2018
17
Macrophage Activation Syndrome Associated With Adult Onset Still's Disease. ( 29938438 )
2018
18
Are the 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome applicable to patients with adult-onset Still's disease? ( 30051293 )
2018
19
Comparison of Different Diagnostic Guidelines for the Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: Single Centre Experience. ( 30431724 )
2018
20
No preventive or therapeutic efficacy of infliximab against macrophage activation syndrome due to systemic juvenile idiopathic arthritis. ( 30406708 )
2018
21
Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia. ( 30061130 )
2018
22
Visceral Leishmaniasis Associated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage in a Lupus Patient. ( 30387429 )
2018
23
Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus. ( 30356389 )
2018
24
The link between Kawasaki disease shock syndrome and macrophage activation syndrome in terms of organ dysfunction observed in children with systemic inflammatory response syndrome. ( 30253701 )
2018
25
Kawasaki disease shock syndrome and macrophage activation syndrome. ( 30257616 )
2018
26
A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus. ( 30480003 )
2018
27
Plasma exchange successfully treated macrophage activation syndrome in rheumatoid factor-positive polyarticular juvenile idiopathic arthritis with co-existing pneumonia. ( 28328098 )
2018
28
Macrophage activation syndrome in systemic lupus erythematosus: a multicenter, case-control study in China. ( 28409239 )
2018
29
Anakinra treatment in macrophage activation syndrome: a single center experience and systemic review of literature. ( 29663156 )
2018
30
Interferon-γ-Mediated Immunopathology Potentiated by Toll-Like Receptor 9 Activation in a Murine Model of Macrophage Activation Syndrome. ( 30073799 )
2018
31
Thrombocytopaenia in Systemic Juvenile Idiopathic Arthritis: Not Always Macrophage Activation Syndrome! ( 30178590 )
2018
32
Macrophage activation syndrome: early diagnosis is key. ( 30214327 )
2018
33
Rapamycin as an Adjunctive Therapy for NLRC4 Associated Macrophage Activation Syndrome. ( 30319625 )
2018
34
Macrophage activation syndrome in a patient with axial spondyloarthritis on adalimumab. ( 30535729 )
2018
35
The clinical utility of splenic fluorodeoxyglucose uptake for diagnosis and prognosis in patients with macrophage activation syndrome. ( 28834911 )
2017
36
Refractory adult-onset Still disease complicated by macrophage activation syndrome and acute myocarditis: A case report treated with high doses (8a88mg/kg/d) of anakinra. ( 28614216 )
2017
37
Novel use of rituximab in macrophage activation syndrome secondary to systemic lupus erythematosus. ( 28827301 )
2017
38
Development and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome. ( 28807357 )
2017
39
Macrophage Activation Syndrome in Paediatric Rheumatic Diseases. ( 28588173 )
2017
40
Biologic therapy modifies clinical and laboratory features of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. ( 28499329 )
2017
41
Characteristic elevation of soluble TNF receptor IIA :A I ratio in macrophage activation syndrome with systemic juvenile idiopathic arthritis. ( 28815559 )
2017
42
Macrophage activation syndrome as a complication of juvenile rheumatoid arthritis. ( 29077164 )
2017
43
Macrophage activation syndrome associated with systemic lupus erythematosus treated successfully with the combination of steroid pulse, immunoglobulin and tacrolimus. ( 29099692 )
2017
44
Advances in immunopathogenesis of macrophage activation syndrome during rheumatic inflammatory diseases: toward new therapeutic targets? ( 28837367 )
2017
45
Role of plasma exchange, leukocytapheresis, and plasma diafiltration in management of refractory macrophage activation syndrome. ( 28730667 )
2017
46
Macrophage Activation Syndrome. ( 28598057 )
2017
47
Influenza B Virus Triggering Macrophage Activation Syndrome in an Infant. ( 29279647 )
2017
48
Macrophage Activation Syndrome: A Report of Two Cases and a Literature Review. ( 29209549 )
2017
49
Application of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease. ( 28412707 )
2017
50
Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: A study of 103 episodes in 89 adult patients. ( 28483541 )
2017

Variations for Macrophage Activation Syndrome

Expression for Macrophage Activation Syndrome

Search GEO for disease gene expression data for Macrophage Activation Syndrome.

Pathways for Macrophage Activation Syndrome

GO Terms for Macrophage Activation Syndrome

Cellular components related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endosome GO:0005768 9.56 HMGB1 PRF1 RAB27A UNC13D
2 extracellular region GO:0005576 9.23 ANGPT1 ANGPT2 CD163 HMGB1 PRF1 RAB27A
3 exocytic vesicle GO:0070382 9.16 RAB27A UNC13D
4 Weibel-Palade body GO:0033093 8.96 RAB27A UNC13D

Biological processes related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

(show all 23)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.74 ANGPT1 HMGB1 TNF
2 leukocyte migration GO:0050900 9.72 ANGPT1 ANGPT2 TNF
3 inflammatory response GO:0006954 9.71 CD163 HMGB1 NLRC4 TNF
4 defense response to virus GO:0051607 9.7 IRF5 PRF1 UNC13D
5 positive regulation of apoptotic process GO:0043065 9.67 HMGB1 IRF5 NLRC4 TNF
6 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.61 HMGB1 TNF
7 positive regulation of cell adhesion GO:0045785 9.61 ANGPT1 TNF
8 positive regulation of blood vessel endothelial cell migration GO:0043536 9.6 ANGPT1 HMGB1
9 positive chemotaxis GO:0050918 9.59 ANGPT1 HMGB1
10 positive regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043280 9.58 HMGB1 TNF
11 negative regulation of blood vessel endothelial cell migration GO:0043537 9.58 ANGPT2 HMGB1
12 positive regulation of phagocytosis GO:0050766 9.56 RAB27A TNF
13 positive regulation of interferon-beta production GO:0032728 9.54 HMGB1 IRF5
14 positive regulation of interleukin-12 production GO:0032735 9.51 HMGB1 IRF5
15 activation of innate immune response GO:0002218 9.49 HMGB1 NLRC4
16 positive regulation of exocytosis GO:0045921 9.48 RAB27A UNC13D
17 regulation of I-kappaB kinase/NF-kappaB signaling GO:0043122 9.46 ANGPT1 TNF
18 positive regulation of interferon-alpha production GO:0032727 9.43 HMGB1 IRF5
19 natural killer cell degranulation GO:0043320 9.32 RAB27A UNC13D
20 negative regulation of cytokine secretion involved in immune response GO:0002740 9.26 ANGPT1 TNF
21 positive regulation of regulated secretory pathway GO:1903307 9.16 RAB27A UNC13D
22 glomerulus vasculature development GO:0072012 8.96 ANGPT1 ANGPT2
23 Tie signaling pathway GO:0048014 8.62 ANGPT1 ANGPT2

Molecular functions related to Macrophage Activation Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 receptor tyrosine kinase binding GO:0030971 8.96 ANGPT1 ANGPT2
2 transcription regulatory region DNA binding GO:0044212 8.8 HMGB1 IRF5 TNF

Sources for Macrophage Activation Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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