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MCID: OPT032
MIFTS: 29

Optic Pathway Glioma

Categories: Endocrine diseases, Eye diseases, Neuronal diseases, Rare diseases
Genes Tissues Related diseases Publications Drugs
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Aliases & Classifications for Optic Pathway Glioma

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MalaCards integrated aliases for Optic Pathway Glioma:

Name: Optic Pathway Glioma 54 60
Visual Pathway Glioma 74

Characteristics:

Orphanet epidemiological data:

60
optic pathway glioma
Inheritance: Not applicable; Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Neuronal diseases Endocrine diseases Eye diseases
See all MalaCards categories (disease lists)
ICD10: 35
Orphanet: 60  
Rare neurological diseases
Rare endocrine diseases


External Ids:

ICD10 via Orphanet 35 D33.3
UMLS via Orphanet 75 C0796418
Orphanet 60 ORPHA2086
UMLS 74 C0796418
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Summaries for Optic Pathway Glioma

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NIH Rare Diseases : 54 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 2086Disease definitionOptic pathway glioma (OPG) is a benign tumor that develop along the optic nerve (chiasm, tracts, and radiations) characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns. OPG are often linked to neurofibromatosis type 1 (NF1, see this term).Visit the Orphanet disease page for more resources.

MalaCards based summary : Optic Pathway Glioma, also known as visual pathway glioma, is related to legius syndrome and chromosome 17q11.2 deletion syndrome. An important gene associated with Optic Pathway Glioma is NF1 (Neurofibromin 1). The drugs Vincristine and Carboplatin have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and t cells.

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Related Diseases for Optic Pathway Glioma

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Diseases related to Optic Pathway Glioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 24)
# Related Disease Score Top Affiliating Genes
1 legius syndrome 11.3
2 chromosome 17q11.2 deletion syndrome 11.3
3 glioma 10.9
4 neurofibromatosis, type iv, of riccardi 10.7
5 neurofibromatosis, type i 10.7
6 strabismus 10.0
7 scoliosis 10.0
8 syringomyelia 10.0
9 nevus, epidermal 9.9
10 obsessive-compulsive disorder 9.9
11 retinitis pigmentosa 9.9
12 leber congenital amaurosis 4 9.9
13 hemolytic anemia 9.9
14 ectropion 9.9
15 acromegaly 9.9
16 astrocytoma 9.9
17 gliosarcoma 9.9
18 rhabdomyosarcoma 9.9
19 retinitis 9.9
20 juvenile pilocytic astrocytoma 9.9
21 mechanical strabismus 9.9
22 diencephalic syndrome 9.9
23 gigantism 9.9
24 pilocytic astrocytoma 9.9

Graphical network of the top 20 diseases related to Optic Pathway Glioma:



Diseases related to Optic Pathway Glioma
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Symptoms & Phenotypes for Optic Pathway Glioma

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Drugs & Therapeutics for Optic Pathway Glioma

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Drugs for Optic Pathway Glioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 134)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
2
Carboplatin Approved Phase 3,Phase 2,Phase 1 41575-94-4 38904 10339178 498142
3
Procarbazine Approved, Investigational Phase 3 671-16-9 4915
4
Lomustine Approved, Investigational Phase 3 13010-47-4 3950
5
Thioguanine Approved Phase 3 154-42-7 2723601
6
Ondansetron Approved Phase 3 99614-02-5 4595
7
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
8
Cisplatin Approved Phase 3 15663-27-1 441203 84093 2767
9
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 1177-87-3
10
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 50-02-2 5743
11
Cyproheptadine Approved Phase 3,Phase 2 129-03-3 2913
12
Histamine Approved, Investigational Phase 3,Phase 2 51-45-6 774
13 Antimitotic Agents Phase 3,Phase 2
14 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Phase 1
15 Dermatologic Agents Phase 3,Phase 2,Phase 1
16 Alkylating Agents Phase 3,Phase 1
17 Antimetabolites, Antineoplastic Phase 3
18 Antimetabolites Phase 3
19 Antineoplastic Agents, Alkylating Phase 3,Phase 1
20 Peripheral Nervous System Agents Phase 3,Phase 1
21 Emetics Phase 3
22 Autonomic Agents Phase 3,Phase 1
23 BB 1101 Phase 3,Phase 2,Phase 1
24 Antiemetics Phase 3,Phase 1
25 Gastrointestinal Agents Phase 3,Phase 2,Phase 1,Not Applicable
26
Histamine Phosphate Phase 3,Phase 2 51-74-1 65513
27 Anti-Allergic Agents Phase 3,Phase 2
28 Neurotransmitter Agents Phase 3,Phase 2,Not Applicable
29 Histamine H1 Antagonists Phase 3,Phase 2
30 Serotonin Agents Phase 3,Phase 2
31 Serotonin Antagonists Phase 3,Phase 2
32 Antipruritics Phase 3,Phase 2
33 Histamine Antagonists Phase 3,Phase 2
34
Serotonin Investigational, Nutraceutical Phase 3,Phase 2 50-67-9 5202
35
Irinotecan Approved, Investigational Phase 2,Phase 1 100286-90-6, 97682-44-5 60838
36
Megestrol acetate Approved, Investigational, Vet_approved Phase 2 595-33-5 11683
37
Iodine Approved, Investigational Phase 2,Phase 1 7553-56-2 807
38
Peginterferon alfa-2b Approved Phase 2 99210-65-8, 215647-85-1
39
Trametinib Approved Phase 1, Phase 2 871700-17-3 11707110
40
Bevacizumab Approved, Investigational Phase 2 216974-75-3
41
Vinblastine Approved Phase 2 865-21-4 13342 241903
42
Lenalidomide Approved Phase 2 191732-72-6 216326
43
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
44
Acetaminophen Approved Phase 2 103-90-2 1983
45
Promethazine Approved, Investigational Phase 2 60-87-7 4927
46
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
47 topoisomerase I inhibitors Phase 2,Phase 1
48 Topoisomerase Inhibitors Phase 2,Phase 1
49 Central Nervous System Stimulants Phase 2
50 Antineoplastic Agents, Hormonal Phase 2,Phase 1,Not Applicable

Interventional clinical trials:

(show all 45)
# Name Status NCT ID Phase Drugs
1 Carboplatin Plus Vincristine in Treating Children and Adolescents With Low Grade Glioma Unknown status NCT00003015 Phase 3 carboplatin;vincristine sulfate
2 Combination Chemotherapy in Treating Children With Progressive Brain Tumors Completed NCT00002944 Phase 3 carboplatin;lomustine;procarbazine hydrochloride;thioguanine;vincristine sulfate
3 Acupressure in Controlling Nausea in Young Patients Receiving Highly Emetogenic Chemotherapy Completed NCT01346267 Phase 3
4 Selumetinib Versus Carboplatin/Vincristine in Treating Patients With Newly Diagnosed or Previously Untreated NF1-Associated Low-Grade Glioma Not yet recruiting NCT03871257 Phase 3 Carboplatin;Selumetinib;Selumetinib Sulfate;Vincristine;Vincristine Sulfate
5 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
6 Phase 3 Study of ANP Therapy vs. TMZ for Optic Pathway Glioma Withdrawn NCT01260103 Phase 3 Temozolomide;ANP Therapy
7 Antineoplaston Therapy in Treating Children With Visual Pathway Glioma Completed NCT00003477 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
8 Carboplatin in Patients With Progressive Gliomas Completed NCT00002749 Phase 2 carboplatin
9 Irinotecan in Treating Children With Refractory Solid Tumors Completed NCT00004078 Phase 2 irinotecan hydrochloride
10 Cyproheptadine and Megestrol in Preventing Weight Loss in Children With Cachexia Caused By Cancer or Cancer Treatment Completed NCT00066248 Phase 2 cyproheptadine hydrochloride;megestrol acetate
11 Phase II Pegylated Interferon Recruiting NCT02343224 Phase 2 Pegylated interferon alpha-2b
12 Selumetinib in Treating Young Patients With Recurrent or Refractory Low Grade Glioma Recruiting NCT01089101 Phase 1, Phase 2 Selumetinib
13 Trametinib for Pediatric Neuro-oncology Patients With Refractory Tumor and Activation of the MAPK/ERK Pathway. Recruiting NCT03363217 Phase 1, Phase 2 Trametinib
14 Vinblastine +/- Bevacizumab in Children With Unresectable or Progressive Low Grade Glioma (LGG) Recruiting NCT02840409 Phase 2 Vinblastine;Bevacizumab
15 Low-Dose or High-Dose Lenalidomide in Treating Younger Patients With Recurrent, Refractory, or Progressive Pilocytic Astrocytoma or Optic Pathway Glioma Active, not recruiting NCT01553149 Phase 2 Lenalidomide
16 Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients With Central Nervous System Cancer or Leptomeningeal Cancer Active, not recruiting NCT00445965 Phase 2
17 Intermittent Dosing Of Selumetinib In Childhood NF1 Associated Tumours Not yet recruiting NCT03326388 Phase 1, Phase 2 Selumetinib
18 Sorafenib in Children and Young Adults With Recurrent or Progressive Low-Grade Astrocytomas Terminated NCT01338857 Phase 2 Sorafenib (Nexavar)
19 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1 verteporfin
20 Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma Completed NCT00994500 Phase 1 vorinostat;bortezomib
21 Pazopanib Hydrochloride in Treating Young Patients With Solid Tumors That Have Relapsed or Not Responded to Treatment Completed NCT00929903 Phase 1 pazopanib hydrochloride
22 AZD2171 in Treating Young Patients With Recurrent, Progressive, or Refractory Primary CNS Tumors Completed NCT00326664 Phase 1 Cediranib Maleate
23 Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas Completed NCT00101270 Phase 1 irinotecan hydrochloride;oxaliplatin
24 Flavopiridol in Treating Children With Relapsed or Refractory Solid Tumors or Lymphomas Completed NCT00012181 Phase 1 alvocidib
25 Imetelstat Sodium in Treating Young Patients With Refractory or Recurrent Solid Tumors or Lymphoma Completed NCT01273090 Phase 1 imetelstat sodium
26 PTC299 in Treating Young Patients With Refractory or Recurrent Primary Central Nervous System Tumors Completed NCT01158300 Phase 1 VEGF inhibitor PTC299
27 Enzastaurin in Treating Young Patients With Refractory Primary CNS Tumors Completed NCT00503724 Phase 1 enzastaurin hydrochloride
28 O6-benzylguanine and Carmustine in Treating Children With Refractory CNS Tumors Completed NCT00003765 Phase 1 O6-benzylguanine;carmustine
29 Colony-Stimulating Factors in Treating Children With Recurrent or Refractory Solid Tumors Completed NCT00003597 Phase 1 carboplatin;etoposide;ifosfamide
30 Monoclonal Antibody Therapy in Treating Patients With Leptomeningeal Cancer Completed NCT00003022 Phase 1
31 Entinostat in Treating Pediatric Patients With Recurrent or Refractory Solid Tumors Recruiting NCT02780804 Phase 1 Entinostat
32 Pomalidomide in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors Active, not recruiting NCT02415153 Phase 1 Pomalidomide
33 Busulfan, Melphalan, Topotecan Hydrochloride, and a Stem Cell Transplant in Treating Patients With Newly Diagnosed or Relapsed Solid Tumor Active, not recruiting NCT00638898 Phase 1 busulfan;melphalan;topotecan hydrochloride
34 Gamma-Secretase Inhibitor RO4929097 in Treating Young Patients With Relapsed or Refractory Solid Tumors, CNS Tumors, Lymphoma, or T-Cell Leukemia Terminated NCT01088763 Phase 1 gamma-secretase/Notch signalling pathway inhibitor RO4929097;dexamethasone
35 MT2004-30: Tomotherapy for Solid Tumors Terminated NCT00623077 Phase 1 busulfan;etoposide;ifosfamide;melphalan;thiotepa;Mesna
36 Talotrexin in Treating Young Patients With Recurrent Solid Tumors or Leukemia That is Recurrent or Does Not Respond to Treatment Withdrawn NCT00458744 Phase 1 talotrexin
37 Ophthalmological Screening and Follow-up of Optic Pathway Gliomas in Children With Neurofibromatosis Type 1. Unknown status NCT02078401
38 Collecting and Storing Tissue From Young Patients With Cancer Completed NCT00898755
39 Collecting and Storing Blood and Brain Tumor Tissue Samples From Children With Brain Tumors Completed NCT00919750
40 Hypotonia and Neurofibromatosis Type 1 (NF1) Glioma Recruiting NCT02584413 Not Applicable Gadolinium contrast
41 Natural History Study of Patients With Neurofibromatosis Type I Recruiting NCT00924196
42 Feasibility and Clinically Application of Magnetic Resonance Fingerprinting Suspended NCT02387840 Not Applicable
43 18F-FDOPA PET/CT or PET/MRI in Measuring Tumors in Patients With Newly-Diagnosed or Recurrent Gliomas Terminated NCT02175745 Not Applicable 18F-fluoro-dihydroxyphenylalanine
44 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452 Not Applicable
45 Studying Cerebrospinal Fluid Proteins and Angiogenesis Proteins in Young Patients With Newly Diagnosed Central Nervous System Tumors Withdrawn NCT00897858

Search NIH Clinical Center for Optic Pathway Glioma

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Genetic Tests for Optic Pathway Glioma

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Anatomical Context for Optic Pathway Glioma

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MalaCards organs/tissues related to Optic Pathway Glioma:

42
Brain, Liver, T Cells, Testes
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Publications for Optic Pathway Glioma

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Articles related to Optic Pathway Glioma:

(show top 50) (show all 96)
# Title Authors Year
1
Long-term visual acuity in patients with optic pathway glioma treated during childhood with up-front BB-SFOP chemotherapy-Analysis of a French pediatric historical cohort. ( 30849081 )
2019
2
Newly-diagnosed Optic Pathway Glioma During Pregnancy: A Case Report. ( 30878749 )
2019
3
Commentary: Identification of Mutation Regions on NF1 Responsible for High- and Low-Risk Development of Optic Pathway Glioma in Neurofibromatosis Type I. ( 30881378 )
2019
4
Manifestations and Treatment of Adult-onset Symptomatic Optic Pathway Glioma in Neurofibromatosis Type 1. ( 30711963 )
2019
5
Prevalence of Strabismus Among Children With Neurofibromatosis Type 1 Disease With and Without Optic Pathway Glioma. ( 30371910 )
2019
6
Delineation of the visual pathway in paediatric optic pathway glioma patients using probabilistic tractography, and correlations with visual acuity. ( 29527480 )
2018
7
Insights into optic pathway glioma vision loss from mouse models of neurofibromatosis type 1. ( 29704429 )
2018
8
Visual outcomes after chemotherapy for optic pathway glioma in children with and without neurofibromatosis type 1: results of the International Society of Paediatric Oncology (SIOP) Low-Grade Glioma 2004 trial UK cohort. ( 29343527 )
2018
9
Endovascular glue embolization of a radiation-induced lenticulostriate artery pseudoaneurysm in a pediatric patient with optic pathway glioma: Case report and review of literature. ( 29848144 )
2018
10
Obsessive-compulsive disorder after therapy for an optic pathway glioma. ( 29556577 )
2018
11
Neurofibromatosis type 1 and optic pathway glioma: Molecular interplay and therapeutic insights. ( 29049847 )
2018
12
Marked functional recovery and imaging response of refractory optic pathway glioma to BRAFV600E inhibitor therapy: a report of two cases. ( 29392423 )
2018
13
Evaluation of racial disparities in pediatric optic pathway glioma incidence: Results from the Surveillance, Epidemiology, and End Results Program, 2000-2014. ( 29684801 )
2018
14
Morning glory disc anomaly and ipsilateral sporadic optic pathway glioma. ( 29780905 )
2018
15
Use of Optical Coherence Tomography to Detect Retinal Nerve Fiber Loss in Children With Optic Pathway Glioma. ( 30619059 )
2018
16
Correlation of peripapillary retinal nerve fibre layer thickness with visual acuity in paediatric patients affected by optic pathway glioma. ( 30284379 )
2018
17
Identification of Mutation Regions on NF1 Responsible for High- and Low-Risk Development of Optic Pathway Glioma in Neurofibromatosis Type I. ( 30087692 )
2018
18
Regression after subtotal resection of an optic pathway glioma in an adult without adjuvant therapy: case report. ( 29999469 )
2018
19
Vascular stenosis in a child with visual pathway glioma treated with bevacizumab: a case report and review of literature. ( 29249070 )
2018
20
Optic pathway glioma of childhood. ( 28257299 )
2017
21
Dramatic clinical and radiographic response to BRAF inhibition in a patient with progressive disseminated optic pathway glioma refractory to MEK inhibition. ( 29040023 )
2017
22
Validation of an automated tractography method for the optic radiations as a biomarker of visual acuity in neurofibromatosis-associated optic pathway glioma. ( 28587872 )
2017
23
Clinical Presentation and Outcome of Patients With Optic Pathway Glioma. ( 28847625 )
2017
24
Macrocephaly Is Not a Predictor of Optic Pathway Glioma Development or Treatment in Neurofibromatosis Type 1. ( 27581847 )
2016
25
Optic Pathway Glioma and Cerebral Focal Abnormal Signal Intensity in Patients with Neurofibromatosis Type 1: Characteristics, Treatment Choices and Follow-up in 134 Affected Individuals and a Brief Review of the Literature. ( 27466519 )
2016
26
High-Dose Intravenous Vitamin C Treatment of a Child with Neurofibromatosis Type 1 and Optic Pathway Glioma: A Case Report. ( 27773919 )
2016
27
Distinguishing optic pathway glioma and retinitis pigmentosa with visual field testing. ( 27316291 )
2016
28
Ophthalmologic Screening for Optic Pathway Glioma in Neurofibromatosis Type 1. ( 27858947 )
2016
29
Optic pathway glioma in children: 10A years of experience in a single institution. ( 27007263 )
2016
30
Cerebral granulomatous condition mimicking optic pathway glioma. ( 27648508 )
2016
31
Primary Gliosarcoma of the Optic Nerve: A Unique Adult Optic Pathway Glioma. ( 27792048 )
2016
32
Optic pathway glioma volume predicts retinal axon degeneration in neurofibromatosis type 1. ( 27815398 )
2016
33
No correlation between NF1 mutation position and risk of optic pathway glioma in 77 unrelated NF1 patients. ( 26969325 )
2016
34
Tram-track-like calcification in adult optic pathway glioma. ( 30459973 )
2016
35
Can we improve accuracy and reliability of MRI interpretation in children with optic pathway glioma? Proposal for a reproducible imaging classification. ( 26518314 )
2015
36
Reversible Growth Hormone Excess in Two Girls with Neurofibromatosis Type 1 and Optic Pathway Glioma. ( 26488470 )
2015
37
Risk of optic pathway glioma in children with neurofibromatosis type 1 and optic nerve tortuosity or nerve sheath thickening. ( 26294105 )
2015
38
Optic pathway glioma in children: does visual deficit correlate with radiology in focal exophytic lesions? ( 26277358 )
2015
39
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. ( 26098902 )
2015
40
Strabismus in patients with neurofibromatosis typeA 1-associated optic pathway glioma. ( 26486023 )
2015
41
Rare case of optic pathway glioma with extensive intra-ocular involvement in a child with neurofibromatosis type 1. ( 25624686 )
2015
42
Long-term visual outcome after chemotherapy for optic pathway glioma in children: Site and age are strongly predictive. ( 26280460 )
2015
43
Clinicopathological and molecular features of malignant optic pathway glioma in an adult. ( 25150758 )
2014
44
Single agent vinorelbine in pediatric patients with progressive optic pathway glioma. ( 25366366 )
2014
45
Immune hemolytic anemia with drug-induced antibodies to carboplatin and vincristine in a pediatric patient with an optic pathway glioma. ( 24860959 )
2014
46
Low-dose Cisplatin-Etoposide regimen for patients with optic pathway glioma: a report of four cases and literature review. ( 24272769 )
2014
47
Spontaneous Regression of a Massive Sporadic Chiasmal Optic Pathway Glioma. ( 25260914 )
2014
48
Gender as a disease modifier in neurofibromatosis type 1 optic pathway glioma. ( 24740685 )
2014
49
Visual function and optic pathway glioma: a critical response-reply. ( 23307229 )
2013
50
Functional outcome measures for NF1-associated optic pathway glioma clinical trials. ( 24249802 )
2013
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Variations for Optic Pathway Glioma

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Expression for Optic Pathway Glioma

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Search GEO for disease gene expression data for Optic Pathway Glioma.
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Pathways for Optic Pathway Glioma

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GO Terms for Optic Pathway Glioma

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Sources for Optic Pathway Glioma

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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