PMP
MCID: PSD007
MIFTS: 62

Pseudomyxoma Peritonei (PMP) malady

Cancer diseases category

Summaries for Pseudomyxoma Peritonei

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42NIH Rare Diseases, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 Pseudomyxoma peritonei is a tumor that most often begins in the appendix.  as the tumor grows, tumor cells may spread into the peritoneal cavity and land on other nearby organs (such as the ovaries or intestines), sometimes forming additional tumors on the surface of these organs.  pseudomyxoma peritonei is extremely unlikely to grow into these organs (to become invasive) or spread to more distant parts of the body (metastasize); therefore, it is not usually thought of as a malignant tumor.  it is rare, occurring in approximately two out of a million people, and women are affected two to three times more frequently than men.  the first symptoms of pseudomyxoma peritonei are an increase in the size of the belly, appendicitis, or the finding of a mass on the ovary during medical imaging tests.   last updated: 9/27/2011

MalaCards: Pseudomyxoma Peritonei, also known as PMP, is related to peritonitis and adenocarcinoma, and has symptoms including estomach/gastric neoplasm/tumor/carcinoma/cancer, peritoneal diseases and anomalies of the abdominal wall. An important gene associated with Pseudomyxoma Peritonei is MUC2 (mucin 2, oligomeric mucus/gel-forming), and among its related pathways are Asparagine N-linked glycosylation and Mucin expression in CF via IL-6, IL-17 signaling pathways. The compounds estrogen and butyrate have been mentioned in the context of this disorder. Affiliated tissues include appendix, ovary and colon, and related mouse phenotypes are tumorigenesis and homeostasis/metabolism.

Wikipedia:63 Pseudomyxoma peritonea is a clinical condition caused by cancerous cells (mucinous adenocarcinoma) that... more...

Aliases & Classifications for Pseudomyxoma Peritonei

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8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 39NCIt, 34MeSH, 56SNOMED-CT, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
pseudomyxoma peritonei:
Age of onset: Adulthood; Age of death: Any age


Aliases & Descriptions:

pseudomyxoma peritonei 8 42 10 44 48 60
pmp 42 48
syndrome of pseudomyxoma peritonei 42
gelatinous ascites 48
mucinous ascites 8
adenomucinosis 48


External Ids:

Disease Ontology8 DOID:3559
NCIt39 C3345
MeSH34 D011553
MESH via Orphanet35 D011553
ICD10 via Orphanet26 C78.6
SNOMED-CT via Orphanet57 112679004, 307601000
UMLS via Orphanet61 C0033822

Related Diseases for Pseudomyxoma Peritonei

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17GeneCards, 18GeneDecks
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Diseases related to Pseudomyxoma Peritonei via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 185)
idRelated DiseaseScoreTop Affiliating Genes
1peritonitis31.3F2
2adenocarcinoma31.2TP53, CEACAM5, CEACAM7, KRT20, KRT7, KRAS
3cystadenocarcinoma31.1CEACAM5, MUC5AC, MUC2
4mucinous cystadenocarcinoma31.0CEACAM7, KRT20, KRT7
5cystadenoma30.8KRT7, KRAS
6endometriosis30.7CDH2, PSG2
7hepatitis30.4KRAS, F2
8papillary carcinoma30.4KRT20, KRT7, CDH1, MUC2
9stomach cancer30.4KRAS, APC
10adenoma30.4TP53, CEACAM5, CEACAM7, KRT20, KRT7, KRAS
11pancreatitis30.2KRAS
12intestinal neoplasm30.2APC
13cervical cancer30.2TP53, CEACAM7, KRAS, PSG2
14colon adenocarcinoma30.2CEACAM5, CEACAM7, KRT20, KRAS, CDH1, MUC5AC
15ovarian cancer30.2TP53, KRT7, KRAS, CDH1, CDH2, PSG2
16malignant mesothelioma30.2CEACAM5, KRT20, KRT7, CDH1, CDH2, VIM
17lung adenocarcinoma30.2TP53, CEACAM5, CEACAM7, KRT20, KRT7, KRAS
18colorectal cancer30.2TP53, CEACAM5, CEACAM7, KRT20, KRT7, KRAS
19breast cancer30.2TP53, CEACAM5, CEACAM7, KRT7, KRAS, CDX2
20melanoma29.9TP53, CEACAM7, KRAS, CDX2, CDH1, CDH2
21mucinous adenocarcinoma10.8
22mucocele of appendix10.7
23mature teratoma10.6
24appendiceal neoplasm10.6
25appendix adenocarcinoma10.6
26cystic teratoma10.6
27ovarian cystic teratoma10.5
28appendix mucinous cystadenocarcinoma10.4
29ovarian cystadenocarcinoma10.4
30ovarian mucinous cystadenocarcinoma10.3
31intestinal obstruction10.3
32ovarian mucinous neoplasm10.3
33neurofibromatosis10.2
34acute pancreatitis10.2
35malignant peritoneal mesothelioma10.2
36colloid carcinoma of the pancreas10.2
37melioidosis10.2
38acromegaly10.2
39appendicitis10.2
40infertility10.2
41cecum adenocarcinoma10.2
42renal pelvis adenocarcinoma10.2
43colon mucinous adenocarcinoma10.2
44abdominal tuberculosis10.2
45bile duct carcinoma10.2
46cervicitis10.2
47dermatomyositis10.2
48hyperglycemia10.2
49mature teratoma of the ovary10.2
50ovarian cyst10.2

Graphical network of the top 20 diseases related to Pseudomyxoma Peritonei:



Diseases related to pseudomyxoma peritonei

Clinical Features for Pseudomyxoma Peritonei

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48Orphanet
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Symptoms:

48 (show all 19)
  • estomach/gastric neoplasm/tumor/carcinoma/cancer
  • peritoneal diseases
  • anomalies of the abdominal wall
  • lymphadenopathy/polyadenopathies
  • pancreatic/pancreas neoplasm/tumor/carcinoma/cancer
  • constipation
  • pollakiuria/polyuria/dysuria/anuria/acute urine retention/oliguria
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • early death/lethality
  • ascitis
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • herniae
  • acute abdominal pain/colic
  • intestinal obstruction/ileus
  • colon neoplasm/tumor/carcinoma/cancer
  • inflammatory intestinal disease
  • ovary/fallopian tube neoplasm/tumor/carcinoma/cancer (excl. teratoma/germinoma)
  • digestive neoplasm/tumor/carcinoma/cancer
  • nausea/vomiting/regurgitation/merycism/hyperemesis

Drugs & Therapeutics for Pseudomyxoma Peritonei

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Pseudomyxoma Peritonei

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Anatomical Context for Pseudomyxoma Peritonei

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32MalaCards
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MalaCards organs/tissues related to Pseudomyxoma Peritonei:

32
Appendix, Ovary, Colon, Testes, Small intestine, Pancreas, Lung, Breast, Spleen, Skin, Liver, Kidney

Animal Models for Pseudomyxoma Peritonei or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Pseudomyxoma Peritonei:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.8APC, MUC2, CDH1, CDX2, KRAS, TP53
2MP:00053768.6MUC2, MUC5AC, CDH2, CDX2, F2
3MP:00053818.4TP53, APC, MUC2, MUC5AC, CDH1, CDX2
4MP:00053808.1VIM, APC, CDH2, CDH1, CDX2, F2
5MP:00053857.9VIM, APC, MUC2, CDH2, CDH1, CDX2
6MP:00053787.8APC, MUC2, CDH2, CDH1, CDX2, F2
7MP:00053847.4VIM, APC, MUC2, CDH2, CDH1, CDX2

Publications for Pseudomyxoma Peritonei

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50PubMed
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Articles related to Pseudomyxoma Peritonei:

(show top 50)    (show all 491)
idTitleAuthorsYear
1
Female patients with pseudomyxoma peritonei: a single-institution clinicopathologic study of 35 cases. (23949422)
2014
2
Kras mutations and p53 overexpression in pseudomyxoma peritonei: association with phenotype and prognosis. (23199549)
2013
3
Treatment of pseudomyxoma peritonei with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC): a single center experience. (24193289)
2013
4
Feasibility of radical cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei of appendiceal origin. (23963027)
2013
5
Pulmonary giant cell carcinoma associated with pseudomyxoma peritonei. (23207264)
2012
6
Recurrent Incisional Hernia due to Pseudomyxoma Peritonei. (21629816)
2011
7
On or in the liver? Two cases with diffuse pseudomyxoma peritonei and synchronous hepatic pathology. (21249511)
2011
8
Pseudomyxoma peritonei: disseminated peritoneal adenomucinosis variant. (22696701)
2011
9
Application of advanced multimodality care to pseudomyxoma peritonei patient: report of first patient treated at a tertiary center. (22693376)
2010
10
Laparoscopic mucin removal in patients with pseudomyxoma peritonei. (19660216)
2009
11
Management of appendiceal pseudomyxoma peritonei diagnosed during pregnancy. (19454019)
2009
12
Blood transfusion therapy for massive hemorrhage associated with scheduled peritonectomy for pseudomyxoma peritonei and peritoneal dissemination]. (19364003)
2009
13
One of the most difficult management problems in surgery is pseudomyxoma peritonei. Foreword. (18590841)
2008
14
Ovarian mature teratomas with mucinous epithelial neoplasms: morphologic heterogeneity and association with pseudomyxoma peritonei. (18344868)
2008
15
Successful pregnancy in a patient with pseudomyxoma peritonei following in-vitro fertilization using donor eggs. (19562054)
2008
16
Cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei: need to establish a multi-institutional registry. (18376211)
2008
17
Pseudomyxoma peritonei presenting with femoral hernias and peritonitis. (17508254)
2007
18
Intraperitoneal hyperthermia in the management of pseudomyxoma peritonei. (17419229)
2007
19
Intraoperative hyperthermic intrathoracic chemotherapy for pleural extension of pseudomyxoma peritonei. (17418746)
2007
20
Toxicity and mortality of cytoreduction and intraoperative hyperthermic intraperitoneal chemotherapy in pseudomyxoma peritonei--a report of 103 procedures. (16303281)
2006
21
Cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei from appendiceal mucinous neoplasms. (16838392)
2006
22
Pseudomyxoma peritonei from mucinous adenocarcinoma of the urachus. (17050882)
2006
23
Recurrence of pseudomyxoma peritonei within the inguinal canal. (16457146)
2005
24
Mucinous tumors arising in ovarian mature cystic teratomas: relationship to the clinical syndrome of pseudomyxoma peritonei. (12717249)
2003
25
Image of the month: pseudomyxoma peritonei. (12806604)
2003
26
An Italian Multicentric Phase II study on peritonectomy and intra peritoneal hyperthermic perfusion (IPHP) to treat patients with pseudomyxoma peritonei. (16767904)
2003
27
Pseudomyxoma peritonei. (11854707)
2002
28
Typical clinical and radiographic findings in a patient with longstanding malignant pseudomyxoma peritonei secondary to a mucinous adenocarcinoma. (12453339)
2002
29
Cytoreductive surgery and perioperative intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei syndrome. (11693816)
2001
30
Extraperitoneal presentation of pseudomyxoma peritonei as a scrotal mass: case report and review of the literature. (17322685)
2000
31
The coexistence of low-grade mucinous neoplasms of the appendix and appendiceal diverticula: a possible role in the pathogenesis of pseudomyxoma peritonei. (10824920)
2000
32
Immunohistochemical evidence supporting the appendiceal origin of pseudomyxoma peritonei in women. (8986525)
1997
33
Pulmonary metastases in pseudomyxoma peritonei syndrome. (9386716)
1997
34
Mucinous adenocarcinoma arising in a giant urachal cyst associated with pseudomyxoma peritonei and stromal osseous metaplasia. (9234391)
1997
35
Columnar cells in smears from pseudomyxoma peritonei. (9067115)
1997
36
Failed treatment of pseudomyxoma peritonei (PMP) with intraperitoneal interferon alpha. (9005155)
1996
37
Synchronous mucinous tumors of the appendix and the ovary associated with pseudomyxoma peritonei. A clinicopathologic study of six cases with comparative analysis of c-Ki-ras mutations. (8651354)
1996
38
Pseudomyxoma peritonei. (8686628)
1996
39
Appendiceal mucinous cystadenoma associated with pseudomyxoma peritonei and multicystic peritoneal mesothelioma: report of a case. (8520171)
1995
40
Pseudomyxoma peritonei. A cancer whose biology is characterized by a redistribution phenomenon. (8129480)
1994
41
A role for dextran in pseudomyxoma peritonei? (1376399)
1992
42
Pseudomyxoma peritonei. A report of three cases. (2561123)
1989
43
Pseudomyxoma peritonei presenting as a scrotal mass. (3352057)
1988
44
Pseudomyxoma peritonei--benign or malignant. (3758624)
1986
45
Biochemical identification of the mucus of pseudomyxoma peritonei as the basis for mucolytic treatment. (2430456)
1986
46
Successful treatment of pseudomyxoma peritonei of ovarian origin with cis-platinum, doxorubicin, and cyclophosphamide. (4054721)
1985
47
Adenomyoma of the pylorus presenting as intestinal obstruction with pseudomyxoma peritonei--a case report. (7344587)
1981
48
Histogenesis of pseudomyxoma peritonei. Review of 9 cases. (840463)
1977
49
Pseudomyxoma peritonei. (13655140)
1959
50
On the etiology of pseudomyxoma peritonei ex appendicitide. (13605911)
1958

Genetic Variations for Pseudomyxoma Peritonei

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Expression for genes affiliated with Pseudomyxoma Peritonei

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pseudomyxoma Peritonei

Search GEO for disease gene expression data for Pseudomyxoma Peritonei.

Pathways for genes affiliated with Pseudomyxoma Peritonei

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53Reactome, 12EMD Millipore, 29KEGG, 37NCBI BioSystems Database, 51QIAGEN, 4Cell Signaling Technology
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Compounds for genes affiliated with Pseudomyxoma Peritonei

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44Novoseek, 2BitterDB, 24HMDB, 11DrugBank, 49PharmGKB, 59Tocris Bioscience, 28IUPHAR
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Compounds related to Pseudomyxoma Peritonei according to GeneCards/GeneDecks:

(show top 50)    (show all 61)
idCompoundScoreTop Affiliating Genes
1estrogen4410.5CEACAM7
2butyrate4410.2CDX2, CEACAM7
3aristolochic acid44 210.9VIM, CDH1, TP53
45-methylcytosine44 2410.9TP53, CDH1, APC
5dicoumarol449.8MUC5AC, F2, TP53
6sulindac44 1110.7TP53, CDH1, APC
7o6-methylguanine449.7APC, CDH1, KRAS, TP53
8nocodazole44 1110.6VIM, APC, CDH1, TP53
9adriamycin449.5CEACAM5, PSG2, VIM
10vitamin d449.4KRAS, CDX2, CDH1, APC, VIM
11leucovorin44 49 1111.4CEACAM5, KRAS, PSG2
12sb 20358044 5910.3VIM, MUC2, MUC5AC, CDH1, TP53
13p002449.2PSG2, CDH1, KRAS, CEACAM5
14carbohydrates449.2MUC2, MUC5AC, CDH1, CEACAM5
15proline449.2VIM, MUC2, MUC5AC, CDH1, TP53
16irinotecan44 49 1111.2CDH1, KRAS, CEACAM5, TP53
17cytosine44 2410.2CEACAM5, CDH1, PSG2, APC
18sialic acid449.2CEACAM5, KRT7, CDH2
19formaldehyde44 2410.1VIM, PSG2, CDH1, KRT7, KRT20
20retinoid449.1VIM, MUC5AC, CDH1, KRT7, TP53
21hydroxyapatite449.1CEACAM5, CDH1, VIM
22methotrexate44 49 1111.1VIM, MUC2, MUC5AC, CDH1, F2, KRT7
23oxaliplatin44 49 1111.0TP53, CEACAM5, KRAS
24latex448.9CEACAM5, CDH1, CDH2, PSG2
25gefitinib44 49 1110.8VIM, CDH1, KRAS, CEACAM7, CEACAM5
26crcs448.7APC, MUC2, CDH1, KRAS, CEACAM5, TP53
27hyaluronic acid44 249.7CEACAM5, KRT20, F2, CDH1, VIM
28lactate448.7VIM, PSG2, CDH1, F2, CEACAM5
29phosphatidylinositol448.6APC, MUC5AC, CDH1, KRAS, CEACAM5
30mucicarmine448.4VIM, KRT7, KRT20, CEACAM7, CEACAM5
31polysaccharide448.4MUC6, MUC5AC, CDH2, KRT7, CEACAM5
32doxorubicin44 49 1110.4VIM, PSG2, CDH1, KRT20, CEACAM5, TP53
335-aza-2deoxycytidine448.3VIM, APC, MUC2, CDH2, CDH1, CDX2
34oligonucleotide448.2VIM, APC, MUC2, MUC6, MUC5AC, CDH2
35thymidine44 249.1PSG2, CDX2, CEACAM7, CEACAM5, TP53
36testosterone44 59 11 2411.1VIM, APC, MUC5AC, CDH2, CDH1, KRT7
37cysteine448.0VIM, MUC2, MUC5AC, CDH2, CDH1, CDX2
38glycogen44 249.0VIM, APC, PSG2, CDH2, CDH1, KRT7
39serine448.0VIM, APC, MUC2, MUC5AC, CDH2, CDH1
40glucose447.9APC, PSG2, MUC2, MUC5AC, CDH2, KRT7
41hematoxylin447.8CDH1, KRT7, KRT20, CEACAM5, MUC5AC, MUC2
425fluorouracil447.8PSG2, MUC2, MUC6, MUC5AC, CDH1, KRAS
43lipid447.7VIM, APC, PSG2, CDH2, KRAS, KRT7
44retinoic acid44 248.5VIM, APC, MUC2, MUC5AC, CDH2, CDH1
45b 723447.4VIM, PSG2, CDH2, CDH1, KRT7, KRT20
46vegf447.3VIM, PSG2, CDH2, CDH1, KRAS, KRT7
47tyrosine447.1APC, MUC5AC, CDH2, CDH1, CDX2, KRAS
48calcium44 49 11 2410.0APC, MUC2, MUC6, MUC5AC, CDH2, CDH1
49paraffin445.9CDH2, CDH1, CDX2, KRAS, KRT7, KRT20
50progesterone44 59 28 11 249.9VIM, APC, MUC2, MUC6, MUC5AC, CDH2

GO Terms for genes affiliated with Pseudomyxoma Peritonei

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16Gene Ontology
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Cellular components related to Pseudomyxoma Peritonei according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1catenin complexGO:0163429.4CDH1, CDH2
2cell-cell adherens junctionGO:0059139.2APC, CDH2, CDH1
3intermediate filamentGO:0058829.2KRT20, KRT7, VIM
4Golgi lumenGO:0057969.1F2, MUC5B, MUC5AC, MUC6, MUC2

Biological processes related to Pseudomyxoma Peritonei according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1intermediate filament organizationGO:0451099.7KRT20, VIM
2cellular component disassembly involved in execution phase of apoptosisGO:0069219.6VIM, APC, CDH1
3O-glycan processingGO:0162669.5MUC5B, MUC5AC, MUC6, MUC2
4striated muscle cell differentiationGO:0511469.4KRAS, CDH2
5post-translational protein modificationGO:0436879.2MUC2, MUC6, MUC5AC, MUC5B, F2
6cellular protein metabolic processGO:0442679.0MUC2, MUC6, MUC5AC, MUC5B, F2

Molecular functions related to Pseudomyxoma Peritonei according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1gamma-catenin bindingGO:0452959.4CDH1, CDH2, APC
2extracellular matrix structural constituentGO:0052019.3MUC6, MUC5AC, MUC5B
3beta-catenin bindingGO:0080139.2CDH1, CDH2, APC
4protein bindingGO:0055156.6APC, MUC2, MUC5B, CDH2, CDH1, MCCC1

Products for genes affiliated with Pseudomyxoma Peritonei

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Sources for Pseudomyxoma Peritonei

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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