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MCID: OPT032
MIFTS: 29

Optic Pathway Glioma

Categories: Rare diseases, Neuronal diseases, Endocrine diseases, Eye 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 53 59
Visual Pathway Glioma 73

Characteristics:

Orphanet epidemiological data:

59
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: 34
Orphanet: 59  
Rare neurological diseases
Rare endocrine diseases


External Ids:

Orphanet 59 ORPHA2086
ICD10 via Orphanet 34 D33.3
UMLS via Orphanet 74 C0796418
UMLS 73 C0796418
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Summaries for Optic Pathway Glioma

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NIH Rare Diseases : 53 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 Carboplatin and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and liver.

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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 27)
# Related Disease Score Top Affiliating Genes
1 legius syndrome 11.1
2 chromosome 17q11.2 deletion syndrome 11.1
3 glioma 10.7
4 neurofibromatosis, type i 10.5
5 neurofibromatosis, type iv, of riccardi 10.5
6 cerebritis 10.0
7 scoliosis 9.9
8 syringomyelia 9.9
9 retinitis 9.9
10 type i 9.9
11 diencephalic syndrome 9.7
12 hemolytic anemia 9.7
13 gigantism 9.7
14 ectropion 9.7
15 acromegaly 9.7
16 astrocytoma 9.7
17 gliosarcoma 9.7
18 rhabdomyosarcoma 9.7
19 orbit rhabdomyosarcoma 9.7
20 nevus, epidermal 9.7
21 obsessive-compulsive disorder 9.7
22 strabismus 9.7
23 pilocytic astrocytoma 9.7
24 retinitis pigmentosa 9.7
25 juvenile pilocytic astrocytoma 9.7
26 aging 9.7
27 leber congenital amaurosis 4 9.7

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 128)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Carboplatin Approved Phase 3,Phase 2,Phase 1 41575-94-4 10339178 498142 38904
2
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
3
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
4
Procarbazine Approved, Investigational Phase 3 671-16-9 4915
5
Dexamethasone Approved, Investigational, Vet_approved Phase 3,Phase 2,Phase 1 50-02-2 5743
6
Cyproheptadine Approved Phase 3,Phase 2 129-03-3 2913
7
Histamine Approved, Investigational Phase 3,Phase 2 75614-87-8, 51-45-6 774
8
Lomustine Approved, Investigational Phase 3 13010-47-4 3950
9
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
10
Ondansetron Approved Phase 3 99614-02-5 4595
11
Thioguanine Approved Phase 3 154-42-7 2723601
12 Dermatologic Agents Phase 3,Phase 2,Phase 1
13 Antimitotic Agents Phase 3,Phase 2
14 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Phase 1
15
Histamine Phosphate Phase 3,Phase 2 51-74-1 65513
16 Dexamethasone acetate Phase 3,Phase 2,Phase 1 1177-87-3
17 Alkylating Agents Phase 3,Phase 1
18 Serotonin Agents Phase 3,Phase 2
19 Serotonin Antagonists Phase 3,Phase 2
20 Gastrointestinal Agents Phase 3,Phase 2,Phase 1,Not Applicable
21 Neurotransmitter Agents Phase 3,Phase 2,Not Applicable
22 Histamine Antagonists Phase 3,Phase 2
23 Histamine H1 Antagonists Phase 3,Phase 2
24 Anti-Allergic Agents Phase 3,Phase 2
25 Emetics Phase 3
26 Peripheral Nervous System Agents Phase 3,Phase 1
27 Antiemetics Phase 3,Phase 1
28 Antipruritics Phase 3,Phase 2
29 BB 1101 Phase 3,Phase 2,Phase 1
30 Antimetabolites Phase 3
31 Autonomic Agents Phase 3,Phase 1
32 Antimetabolites, Antineoplastic Phase 3
33 Antineoplastic Agents, Alkylating Phase 3,Phase 1
34
Serotonin Investigational, Nutraceutical Phase 3,Phase 2 50-67-9 5202
35
Irinotecan Approved, Investigational Phase 2,Phase 1 97682-44-5, 100286-90-6 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
Vinblastine Approved Phase 2 865-21-4 13342 241903
39
Bevacizumab Approved, Investigational Phase 2 216974-75-3
40
Peginterferon alfa-2b Approved Phase 2 99210-65-8, 215647-85-1
41
Lenalidomide Approved Phase 2 191732-72-6 216326
42
Acetaminophen Approved Phase 2 103-90-2 1983
43
Diphenhydramine Approved, Investigational Phase 2 147-24-0, 58-73-1 3100
44
Promethazine Approved, Investigational Phase 2 60-87-7 4927
45
Crizotinib Approved Phase 1, Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
46
Trametinib Approved Phase 1, Phase 2 871700-17-3 11707110
47
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
48
Nicotinamide Approved, Investigational, Nutraceutical Phase 2 98-92-0 936
49
Niacin Approved, Investigational, Nutraceutical Phase 2 59-67-6 938
50
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037

Interventional clinical trials:

(show all 44)
# 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 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
5 Phase 3 Study of ANP Therapy vs. TMZ for Optic Pathway Glioma Withdrawn NCT01260103 Phase 3 Temozolomide;ANP Therapy
6 Antineoplaston Therapy in Treating Children With Visual Pathway Glioma Completed NCT00003477 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
7 Carboplatin in Patients With Progressive Gliomas Completed NCT00002749 Phase 2 carboplatin
8 Irinotecan in Treating Children With Refractory Solid Tumors Completed NCT00004078 Phase 2 irinotecan hydrochloride
9 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
10 Phase II Pegylated Interferon Recruiting NCT02343224 Phase 2 Pegylated interferon alpha-2b
11 Selumetinib in Treating Young Patients With Recurrent or Refractory Low Grade Glioma Recruiting NCT01089101 Phase 1, Phase 2 Selumetinib
12 Vinblastine +/- Bevacizumab in Children With Unresectable or Progressive Low Grade Glioma (LGG) Recruiting NCT02840409 Phase 2 Vinblastine;Bevacizumab
13 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
14 Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients With Central Nervous System Cancer or Leptomeningeal Cancer Active, not recruiting NCT00445965 Phase 2
15 Crizotinib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Anaplastic Large Cell Lymphoma Active, not recruiting NCT00939770 Phase 1, Phase 2 crizotinib
16 Intermittent Dosing Of Selumetinib In Childhood NF1 Associated Tumours Not yet recruiting NCT03326388 Phase 1, Phase 2 Selumetinib
17 Trametinib for Pediatric Neuro-oncology Patients With Refractory Tumor and Activation of the MAPK/ERK Pathway. Not yet recruiting NCT03363217 Phase 1, Phase 2 Trametinib
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 Hypotonia and Neurofibromatosis Type 1 (NF1) Glioma Recruiting NCT02584413 Gadolinium contrast
39 Natural History Study of Patients With Neurofibromatosis Type I Recruiting NCT00924196
40 Collecting and Storing Blood and Brain Tumor Tissue Samples From Children With Brain Tumors Active, not recruiting NCT00919750
41 Feasibility and Clinically Application of Magnetic Resonance Fingerprinting Suspended NCT02387840 Not Applicable
42 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
43 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452 Not Applicable
44 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:

41
Brain, T Cells, Liver, 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 85)
# Title Authors Year
1
Delineation of the visual pathway in paediatric optic pathway glioma patients using probabilistic tractography, and correlations with visual acuity. ( 29527480 )
2018
2
Insights into optic pathway glioma vision loss from mouse models of neurofibromatosis type 1. ( 29704429 )
2018
3
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
4
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
5
Obsessive-compulsive disorder after therapy for an optic pathway glioma. ( 29556577 )
2018
6
Neurofibromatosis type 1 and optic pathway glioma: Molecular interplay and therapeutic insights. ( 29049847 )
2018
7
Marked functional recovery and imaging response of refractory optic pathway glioma to BRAFV600E inhibitor therapy: a report of two cases. ( 29392423 )
2018
8
Evaluation of racial disparities in pediatric optic pathway glioma incidence: Results from the Surveillance, Epidemiology, and End Results Program, 2000-2014. ( 29684801 )
2018
9
Morning glory disc anomaly and ipsilateral sporadic optic pathway glioma. ( 29780905 )
2018
10
Optic pathway glioma of childhood. ( 28257299 )
2017
11
Dramatic clinical and radiographic response to BRAF inhibition in a patient with progressive disseminated optic pathway glioma refractory to MEK inhibition. ( 29040023 )
2017
12
Validation of an automated tractography method for the optic radiations as a biomarker of visual acuity in neurofibromatosis-associated optic pathway glioma. ( 28587872 )
2017
13
Clinical Presentation and Outcome of Patients With Optic Pathway Glioma. ( 28847625 )
2017
14
Optic Pathway Gliomas Secondary to Neurofibromatosis Type 1. ( 28941532 )
2017
15
Natural history of optic pathway gliomas in a cohort of unselected patients affected by Neurofibromatosis 1. ( 28577031 )
2017
16
Macrocephaly Is Not a Predictor of Optic Pathway Glioma Development or Treatment in Neurofibromatosis Type 1. ( 27581847 )
2016
17
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
18
High-Dose Intravenous Vitamin C Treatment of a Child with Neurofibromatosis Type 1 and Optic Pathway Glioma: A Case Report. ( 27773919 )
2016
19
Distinguishing optic pathway glioma and retinitis pigmentosa with visual field testing. ( 27316291 )
2016
20
Ophthalmologic Screening for Optic Pathway Glioma in Neurofibromatosis Type 1. ( 27858947 )
2016
21
Optic pathway glioma in children: 10A years of experience in a single institution. ( 27007263 )
2016
22
Cerebral granulomatous condition mimicking optic pathway glioma. ( 27648508 )
2016
23
Primary Gliosarcoma of the Optic Nerve: A Unique Adult Optic Pathway Glioma. ( 27792048 )
2016
24
Optic pathway glioma volume predicts retinal axon degeneration in neurofibromatosis type 1. ( 27815398 )
2016
25
No correlation between NF1 mutation position and risk of optic pathway glioma in 77 unrelated NF1 patients. ( 26969325 )
2016
26
Can we improve accuracy and reliability of MRI interpretation in children with optic pathway glioma? Proposal for a reproducible imaging classification. ( 26518314 )
2015
27
Reversible Growth Hormone Excess in Two Girls with Neurofibromatosis Type 1 and Optic Pathway Glioma. ( 26488470 )
2015
28
Risk of optic pathway glioma in children with neurofibromatosis type 1 and optic nerve tortuosity or nerve sheath thickening. ( 26294105 )
2015
29
Optic pathway glioma in children: does visual deficit correlate with radiology in focal exophytic lesions? ( 26277358 )
2015
30
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. ( 26098902 )
2015
31
Strabismus in patients with neurofibromatosis typeA 1-associated optic pathway glioma. ( 26486023 )
2015
32
Rare case of optic pathway glioma with extensive intra-ocular involvement in a child with neurofibromatosis type 1. ( 25624686 )
2015
33
Long-term visual outcome after chemotherapy for optic pathway glioma in children: Site and age are strongly predictive. ( 26280460 )
2015
34
Clinicopathological and molecular features of malignant optic pathway glioma in an adult. ( 25150758 )
2014
35
Single agent vinorelbine in pediatric patients with progressive optic pathway glioma. ( 25366366 )
2014
36
Immune hemolytic anemia with drug-induced antibodies to carboplatin and vincristine in a pediatric patient with an optic pathway glioma. ( 24860959 )
2014
37
Low-dose Cisplatin-Etoposide regimen for patients with optic pathway glioma: a report of four cases and literature review. ( 24272769 )
2014
38
Spontaneous Regression of a Massive Sporadic Chiasmal Optic Pathway Glioma. ( 25260914 )
2014
39
Gender as a disease modifier in neurofibromatosis type 1 optic pathway glioma. ( 24740685 )
2014
40
Visual function and optic pathway glioma: a critical response-reply. ( 23307229 )
2013
41
Functional outcome measures for NF1-associated optic pathway glioma clinical trials. ( 24249802 )
2013
42
Optic pathway glioma, scoliosis, Chiari type 1 malformation, and syringomyelia in a patient with neurofibromatosis type 1. ( 24174788 )
2013
43
Detection of tumor progression in optic pathway glioma with and without neurofibromatosis type 1. ( 24101736 )
2013
44
'Double Midbrain' Sign in Extensive Optic Pathway Glioma in Neurofibromatosis-1. ( 23943576 )
2013
45
Diencephalic syndrome as sign of tumor progression in a child with neurofibromatosis type 1 and optic pathway glioma: a case report. ( 23615855 )
2013
46
Visual function and optic pathway glioma: a critical response. ( 23307228 )
2013
47
Clustering of mutations in the 5' tertile of the NF1 gene in Slovakia patients with optic pathway glioma. ( 23906300 )
2013
48
Prognostic role for diffusion-weighted imaging of pediatric optic pathway glioma. ( 23673514 )
2013
49
Feasibility and comparison of visual acuity testing methods in children with neurofibromatosis type 1 and/or optic pathway gliomas. ( 23329671 )
2013
50
Optic pathway glioma as part of a constitutional mismatch-repair deficiency syndrome in a patient meeting the criteria for neurofibromatosis type 1. ( 22848017 )
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 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
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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