MCID: PRV003
MIFTS: 52

Perivascular Epithelioid Cell Tumor malady

Cancer diseases category

Summaries for Perivascular Epithelioid Cell Tumor

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63Wikipedia, 32MalaCards
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Wikipedia:63 In oncology, PEComa, also PEC tumour and perivascular epithelioid cell tumour, is a family of... more...

MalaCards: Perivascular Epithelioid Cell Tumor, also known as neoplasm with perivascular epithelioid cell differentiation, is related to lymphangioleiomyomatosis and angiomyolipoma. An important gene associated with Perivascular Epithelioid Cell Tumor is ACTC1 (actin, alpha, cardiac muscle 1), and among its related pathways are Inhibition of TSC complex formation by PKB and Glioblastoma Multiforme. The compounds paclitaxel and monochloroacetic acid have been mentioned in the context of this disorder. Affiliated tissues include uterus, liver and colon, and related mouse phenotypes are pigmentation and muscle.

Aliases & Classifications for Perivascular Epithelioid Cell Tumor

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8Disease Ontology, 10DISEASES, 60UMLS, 39NCIt, 56SNOMED-CT, 34MeSH
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases


Aliases & Descriptions:

perivascular epithelioid cell tumor 8 10
neoplasm with perivascular epithelioid cell differentiation 8
perivascular epithelioid cell neoplasms 60
pecoma 8


External Ids:

Disease Ontology8 DOID:2643
NCIt39 C38150
SNOMED-CT56 388601000
MeSH34 D054973

Related Diseases for Perivascular Epithelioid Cell Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Perivascular Epithelioid Cell Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 180)
idRelated DiseaseScoreTop Affiliating Genes
1lymphangioleiomyomatosis30.8DES, TSC1, TSC2
2angiomyolipoma30.7TSC2, PMEL, VIM, CCL26, S100A1, MLANA
3pancreatitis30.6KITLG
4neuroblastoma30.4SYP
5angiosarcoma30.4VIM, MUC1, ACTC1
6acute leukemia30.4TSC2, KIT, KITLG, NTRK3
7leukemia30.3TFE3, NTRK3, CDKN2A, KITLG, KIT
8tuberous sclerosis30.3VIM, CCL26, S100B, RHEB, SYP, TSC1
9smooth muscle tumor30.3VIM, CALD1, S100B, S100A1, DES, KIT
10leiomyoma30.3VIM, CALD1, S100B, S100A1, DES, KIT
11gastrointestinal stromal tumor30.3ACTC1, KIT, VIM, S100B, S100A1, DES
12kidney angiomyolipoma30.1TSC1, TSC2
13epithelioid leiomyosarcoma30.1VIM, DES, ACTC1
14leiomyosarcoma30.1CALD1, S100B, S100A1, MLANA, DES, MUC1
15melanoma30.0PMEL, VIM, S100B, S100A1, MLANA, DES
16endometriosis29.9ACTC1
17liposarcoma29.9VIM, DES
18clear cell sarcoma29.9S100B, KIT
19sarcoma29.8VIM, CALD1, S100B, S100A1, TFE3, MLANA
20perivascular tumor11.3
21uterus perivascular epithelioid cell tumor10.9
22hepatitis10.5
23appendicitis10.3
24hepatitis a10.3
25benign perivascular tumor10.3
26cervicitis10.3
27diverticulitis10.3
28lymphoblastic leukemia10.3
29mesenchymal cell neoplasm10.3
30prostatitis10.3
31kidney cancer10.0TFE3
32hemangioma10.0ACTC1
33esophagitis10.0CCL26
34renal oncocytoma10.0S100A1
35von hippel-lindau disease10.0TSC2
36atopic dermatitis10.0CCL26
37fasciitis10.0ACTC1
38pneumothorax10.0TSC1
39uterine carcinosarcoma10.0KIT
40histiocytosis10.0S100B
41infantile myofibromatosis10.0DES
42sertoli-leydig cell tumor10.0MUC1
43pituitary adenoma10.0SYP
44carney triad10.0KIT
45pancreatic islet cell tumors10.0SYP
46melanotic neuroectodermal tumor10.0SYP
47cystadenocarcinoma10.0MUC1
48tuberous sclerosis complex10.0TSC1, TSC2
49oxyphilic adenoma10.0TFE3, S100A1
50adenomatoid tumor10.0VIM

Graphical network of the top 20 diseases related to Perivascular Epithelioid Cell Tumor:



Diseases related to perivascular epithelioid cell tumor

Clinical Features for Perivascular Epithelioid Cell Tumor

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Drugs & Therapeutics for Perivascular Epithelioid Cell Tumor

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Genetic Tests for Perivascular Epithelioid Cell Tumor

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Anatomical Context for Perivascular Epithelioid Cell Tumor

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32MalaCards
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MalaCards organs/tissues related to Perivascular Epithelioid Cell Tumor:

32
Uterus, Liver, Colon, Kidney, Cervix, Pancreas, Lung, Lymph node, Prostate, Skin, Smooth muscle, Heart, Appendix

Animal Models for Perivascular Epithelioid Cell Tumor or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Perivascular Epithelioid Cell Tumor:

36 (show all 15)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00011869.6PMEL, TFE3, MLANA
2MP:00053699.6RHEB, CDKN2A, ACTC1, TSC1
3MP:00020069.1MUC1, CDKN2A, KITLG, KIT, TSC1, TSC2
4MP:00053709.0MUC1, CDKN2A, KITLG, KIT, TSC1, TSC2
5MP:00107718.9TSC2, PMEL, S100B, TFE3, CDKN2A, KITLG
6MP:00053768.7PMEL, RHEB, DES, KITLG, ACTC1, TSC1
7MP:00053808.6RHEB, CDKN2A, KITLG, KIT, TSC1, TSC2
8MP:00030128.5PMEL, MLANA, SYP, NTRK3, MUC1, CDKN2A
9MP:00053918.4PMEL, VIM, TFE3, NTRK3, MUC1, CDKN2A
10MP:00053868.2VIM, S100B, S100A1, DES, NTRK3, CDKN2A
11MP:00053858.1VIM, S100A1, RHEB, DES, NTRK3, KITLG
12MP:00028738.1S100B, S100A1, TFE3, RHEB, SYP, NTRK3
13MP:00036317.8VIM, RHEB, SYP, NTRK3, CDKN2A, KITLG
14MP:00053787.5VIM, CALD1, TFE3, RHEB, NTRK3, CDKN2A
15MP:00107687.3DES, RHEB, TFE3, CALD1, VIM, NTRK3

Publications for Perivascular Epithelioid Cell Tumor

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50PubMed
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Articles related to Perivascular Epithelioid Cell Tumor:

(show top 50)    (show all 120)
idTitleAuthorsYear
1
Perivascular epithelioid cell tumor of the rectum: report of a case and review of the literature. (24410788)
2014
2
Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case. (23515754)
2013
3
Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: a case report and review of literature. (23587410)
2013
4
Hepatic perivascular epithelioid cell tumor: a case report and review of the literature. (23774542)
2013
5
Primary perivascular epithelioid cell tumor in the rectum: a case report and review of the literature. (23484779)
2013
6
Malignant perivascular epithelioid cell tumor: a case report of a cutaneous tumor on the cheek of a male patient. (24124856)
2013
7
Multiple Perivascular Epithelioid Cell Tumors: Clear Cell Tumor of the Lung Accompanied by Angiomyolipoma of the Liver. (23603639)
2013
8
Malignant perivascular epithelioid cell tumor of the esophagus. (22957287)
2012
9
A renal epithelioid angiomyolipoma/perivascular epithelioid cell tumor with TFE3 gene break visualized by FISH. (23224603)
2012
10
HMB-45 negative clear cell perivascular epithelioid cell tumor of the skin. (22507471)
2012
11
Pigmented perivascular epithelioid cell tumor (PEComa) of the kidney: a case report and review of the literature. (23136579)
2012
12
Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features--analysis of 7 cases and review of the literature. (22278345)
2012
13
Malignant perivascular epithelioid cell tumor (PEComa) of the uterus: serial imaging with F-18 FDG PET/CT for surveillance of recurrence and evaluation of response to therapy. (21368597)
2011
14
Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs. (22126387)
2011
15
A patient presenting with a perivascular epithelioid cell tumor in the broad ligament: a case report. (21846376)
2011
16
Perivascular epithelioid cell tumor of the bladder. (21334671)
2011
17
Adrenal perivascular epithelioid cell tumor: a case report with discussion of differential diagnoses. (21466369)
2011
18
Fertility-sparing operation for recurrence of uterine cervical perivascular epithelioid cell tumor. (21139828)
2010
19
Perivascular epithelioid cell tumor of abdominal origin. (20471562)
2010
20
Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. (20048174)
2010
21
Molecular pathology of lymphangioleiomyomatosis and other perivascular epithelioid cell tumors. (20073603)
2010
22
Perivascular epithelioid cell tumor of the heart in a child. (20085497)
2010
23
Perivascular epithelioid cell tumor (PEComa) of the ascending colon: the implication of IFN-I+2b treatment. (21218021)
2010
24
Ocular perivascular epithelioid cell tumor: report of 2 cases with distinct clinical presentations. (20236689)
2010
25
Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report. (19399284)
2009
26
Perivascular epithelioid cell tumor (PEComa) of the pancreas: immunoelectron microscopy and review of the literature. (19712133)
2009
27
Perivascular epithelioid cell tumor. (19391667)
2009
28
Laparoscopic radical excision of primary round ligament perivascular epithelioid cell tumor mimicking leiomyoma. (19835808)
2009
29
Perivascular epithelioid cell tumor (PEComa) of the urinary bladder: report of 3 cases and review of the literature. (18971775)
2009
30
Perivascular epithelioid cell tumor of the nasal cavity with TFE3 expression. (19788626)
2009
31
Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus. (19322738)
2009
32
Perivascular epithelioid cell tumor of the retroperitoneum in a young woman resulting in an abdominal chyloma. (18213505)
2009
33
Perivascular epithelioid cell tumor of the cecum: a case report and review of literatures. (19719977)
2009
34
Growing perivascular epithelioid cell tumor of the liver studied with contrast-enhanced ultrasonography and magnetic resonance imaging. (19023006)
2008
35
Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor. (18205275)
2008
36
Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity. (18712670)
2008
37
Perivascular epithelioid cell tumor of the pericardium: a case report. (18402815)
2008
38
Metastatic perivascular epithelioid cell tumor of the colon in a child. (18493218)
2008
39
Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report. (17455882)
2007
40
Perivascular epithelioid cell tumor of the uterus: report of three cases. (17439750)
2007
41
Malignant perivascular epithelioid cell tumor of the colon: report of a case with molecular analysis. (16398680)
2006
42
Comparative genomic hybridization study of perivascular epithelioid cell tumor: molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm. (16647959)
2006
43
Perivascular epithelioid cell tumor of the uterus: a case report. (17285843)
2006
44
Correction: Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. (15869711)
2005
45
Perivascular epithelioid cell tumor (PEComa) with perirenal manifestation. (15948750)
2005
46
Perivascular epithelioid cell tumor of the orbit: a case report and review of the literature. (15803216)
2005
47
Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus. (16344678)
2005
48
Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament. (15361222)
2004
49
Malignant perivascular epithelioid cell tumor involving the prostate. (12562263)
2003
50
Perivascular epithelioid cell tumor ('PEComa') of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. (11756764)
2002

Genetic Variations for Perivascular Epithelioid Cell Tumor

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Expression for genes affiliated with Perivascular Epithelioid Cell Tumor

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Perivascular Epithelioid Cell Tumor

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Pathways for genes affiliated with Perivascular Epithelioid Cell Tumor

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53Reactome, 51QIAGEN, 37NCBI BioSystems Database, 12EMD Millipore, 52R&D Systems, 4Cell Signaling Technology, 29KEGG
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Pathways related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.1TSC1, TSC2
29.8CDKN2A, TSC1, TSC2
39.6TSC2, TSC1, RHEB
4
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9.6TSC2, TSC1, RHEB
5
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9.6RHEB, TSC1, TSC2
6
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9.6TSC2, KIT, KITLG, S100B
7
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9.5NTRK3, RHEB, KITLG
89.4NTRK3, RHEB, TSC2, TSC1
99.3ACTC1, DES, CALD1, VIM
10
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9.3ACTC1, DES, VIM, CALD1
11
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9.2CCL26, NTRK3, KITLG, KIT, TSC1, TSC2
129.2RHEB, KITLG, TSC1, TSC2, KIT
13
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9.1TSC1, VIM, NTRK3, TSC2, KIT, KITLG
14
Hide members
8.7RHEB, VIM, CCL26, DES, NTRK3, TSC1

Compounds for genes affiliated with Perivascular Epithelioid Cell Tumor

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44Novoseek, 49PharmGKB, 11DrugBank, 28IUPHAR, 24HMDB, 59Tocris Bioscience
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Compounds related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 78)
idCompoundScoreTop Affiliating Genes
1paclitaxel44 49 1112.1CDKN2A, S100B
2monochloroacetic acid4410.1VIM, KITLG, KIT
3eosin4410.1MUC1, S100B, VIM
4orcein4410.0VIM, S100B, DES, ACTC1
52-methoxyethanol4410.0ACTC1, S100A1, VIM
6creatinine4410.0SYP, CDKN2A, KITLG, ACTC1
7dexamethasone44 49 28 1112.9KITLG, CDKN2A, S100B
8glycogen44 2410.9SYP, KIT, ACTC1, TSC1, TSC2
9acetylcholine44 49 28 11 2413.9SYP, S100A1
10guanosine44 11 2411.8VIM, CDKN2A, TSC1, TSC2
11s 100449.6ACTC1, MUC1, SYP, DES, S100B, VIM
12reticulin449.6VIM, S100B, DES, SYP, MUC1, ACTC1
13toluidine449.6VIM, S100A1, DES, KITLG, KIT
14agarose449.5S100B, MUC1, CDKN2A, KITLG, KIT
15cytarabine44 49 1111.5KIT, KITLG, CDKN2A, MUC1, VIM
16actomyosin449.5VIM, CALD1, S100A1, ACTC1
17adriamycin449.5VIM, DES, MUC1, CDKN2A
18imatinib44 49 1111.5S100B, DES, NTRK3, KITLG, KIT, ACTC1
19ethanol44 49 11 2412.5S100B, DES, SYP, MUC1, KITLG
20steroid449.4MLANA, DES, SYP, CDKN2A, ACTC1, TSC2
21gold449.4DES, SYP, MUC1, CDKN2A, KITLG, KIT
22bromodeoxyuridine449.4VIM, S100B, DES, KITLG
23methotrexate44 49 1111.4VIM, MUC1, CDKN2A, KITLG, KIT
24silver449.4PMEL, S100A1, DES, SYP, CDKN2A
25formaldehyde44 2410.4VIM, S100B, DES, SYP, MUC1, KIT
26estradiol44 11 2411.3KITLG, MUC1, SYP, DES, VIM
27mucicarmine449.3ACTC1, MUC1, SYP, DES, S100A1, S100B
28b 723449.3VIM, S100B, S100A1, DES, SYP, MUC1
29actinomycin d449.3VIM, DES, MUC1, CDKN2A, KITLG, KIT
30cyclic amp44 2410.2VIM, S100B, DES, SYP, MUC1, CDKN2A
31lactate449.1VIM, S100B, MLANA, DES, KITLG, KIT
32biotin44 11 2411.1VIM, S100A1, DES, MUC1, CDKN2A, KITLG
33rapamycin449.1VIM, CCL26, RHEB, KITLG, KIT, TSC1
34leucine449.1VIM, S100B, TFE3, SYP, CDKN2A, KIT
35iron44 2410.1VIM, S100B, S100A1, DES, CDKN2A, KITLG
36oxygen44 2410.0CCL26, S100B, DES, MUC1, CDKN2A, KITLG
37threonine448.9S100B, DES, NTRK3, MUC1, CDKN2A, KIT
38doxorubicin44 49 1110.9VIM, DES, CDKN2A, KITLG, KIT
39nitric oxide44 11 2410.8S100B, S100A1, DES, SYP, MUC1, CDKN2A
40glucose448.8TFE3, RHEB, MUC1, CDKN2A, KITLG, ACTC1
41progesterone44 59 28 11 2412.7VIM, S100B, S100A1, DES, SYP, MUC1
42cysteine448.7VIM, CCL26, S100B, S100A1, DES, SYP
43hematoxylin448.4S100A1, S100B, VIM, MLANA, DES, SYP
44testosterone44 59 11 2411.3VIM, S100B, DES, SYP, NTRK3, MUC1
45tyrosine448.3SYP, DES, S100A1, S100B, NTRK3, MUC1
46retinoic acid44 249.2VIM, S100B, S100A1, DES, SYP, NTRK3
47vegf448.2VIM, S100B, DES, SYP, NTRK3, MUC1
48serine448.1VIM, S100B, RHEB, DES, SYP, MUC1
49calcium44 49 11 2410.9CCL26, CALD1, S100B, S100A1, DES, SYP
50paraffin447.7TSC2, TSC1, ACTC1, KIT, KITLG, CDKN2A

GO Terms for genes affiliated with Perivascular Epithelioid Cell Tumor

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16Gene Ontology
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Cellular components related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1TSC1-TSC2 complexGO:03359610.1TSC1, TSC2
2intermediate filamentGO:0058829.8VIM, DES, SYNM
3protein complexGO:0432349.3S100A1, SYP, CDKN2A, TSC1
4cytoplasmGO:0057377.5VIM, S100B, TFE3, NTRK3, TSC1, ACTC1

Biological processes related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of cell sizeGO:04579210.1TSC2, TSC1
2muscle filament slidingGO:0300499.7VIM, DES, ACTC1
3germ cell programmed cell deathGO:0352349.7KIT, KITLG
4cell cycle arrestGO:0070509.3TSC2, TSC1, CDKN2A, RHEB

Molecular functions related to Perivascular Epithelioid Cell Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1structural constituent of cytoskeletonGO:0052009.8SYNM, DES, VIM
2p53 bindingGO:0020399.7CDKN2A, MUC1, NTRK3
3S100 protein bindingGO:0445489.6S100A1, S100B
4identical protein bindingGO:0428029.1VIM, S100B, S100A1, DES, SYP
5protein bindingGO:0055156.7TSC2, PMEL, VIM, S100B, S100A1, MLANA

Products for genes affiliated with Perivascular Epithelioid Cell Tumor

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Sources for Perivascular Epithelioid Cell Tumor

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet