MCID: PMS001
MIFTS: 63

Poems Syndrome malady

Rare diseases, Neuronal diseases, Blood diseases, Cancer diseases, Immune diseases, Skin diseases, Bone diseases categories

Summaries for Poems Syndrome

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44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 Poems syndrome is a rare blood disorder. it is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, and skin changes. signs and symptoms may include progressive sensorimotor polyneuropathy, enlarged liver and spleen, swollen lymph nodes, and darkening of the skin. further testing and evaluation may show plasma cell disorder and sclerotic bone lesions. many people with poems syndrome have diabetes (20-50%) and primary gonadal failure (55-70%). other general signs and symptoms may include increased body hair, thickening of the skin, whitening of the nails, fatigue, weakness, swelling of the ankles and legs, enlarged breast tissue, generalized aches and pains, fluid in the lungs, and vision changes. sign and symptoms tend to begin in a person's 40's to 50's. poems syndrome is a rapidly progressive disorder that can become life threatening. currently the exact cause of poems syndrome is unknown. researchers believe the syndrome involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body. last updated: 2/18/2013

MalaCards: Poems Syndrome, also known as osteosclerotic myeloma, is related to myeloma and thrombocytosis, and has symptoms including cortico-adrenal hypoplasia/insufficiency, renal disease/nephropathy and decreased body hair/axillar/pubic hairlessness. An important gene associated with Poems Syndrome is VEGFA (vascular endothelial growth factor A), and among its related pathways are HIF-1 signaling pathway and Nucleotide-binding domain, leucine rich repeat containing receptor (NLR) signaling pathways. The compounds muramyl tripeptide phosphatidylethanolamine and turpentine have been mentioned in the context of this disorder. Affiliated tissues include skin, bone and liver, and related mouse phenotypes are cardiovascular system and tumorigenesis.

Wikipedia:66 POEMS syndrome (also known as Crow?Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare... more...

Aliases & Classifications for Poems Syndrome

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Sources:
50Orphanet, 63UMLS, 9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 46Novoseek, 36MeSH, 59SNOMED-CT, 41NCIt, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
See all sources

Classifications:



Characteristics (Orphanet epidemiological data):

50
poems syndrome:
Prevalence: <1/1000000; Age of onset: Adulthood


Aliases & Descriptions:

poems syndrome 9 44 11 46 50 63
osteosclerotic myeloma 50 63
crow-fukase syndrome 44 50
polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes syndrome 44
polyneuropathy - endocrinopathy - plasma cell dyscrasia 50
polyneuropathy organomegaly 44
takatsuki syndrome 50
pep syndrome 50


External Ids:

Disease Ontology9 DOID:14039
MeSH36 D016878
NCIt41 C80303
MESH via Orphanet37 D016878
SNOMED-CT59 79268002
ICD10 via Orphanet27 D47.7
SNOMED-CT via Orphanet60 425657001, 79268002
UMLS via Orphanet64 C0085404, C1510415

Related Diseases for Poems Syndrome

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18GeneCards, 19GeneDecks
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Diseases related to Poems Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 147)
idRelated DiseaseScoreTop Affiliating Genes
1myeloma30.8IL1B, CST3, BGLAP, VEGFA, IL6
2thrombocytosis30.1IL6, IL1B
3kaposi's sarcoma30.1IL6, VEGFA
4arthritis30.0IL6, IL1B, BGLAP
5diabetes mellitus29.8BGLAP, VEGFA, IL6, CST3, IL1B
6rheumatoid arthritis29.7CCL11, IL6, CST3, VEGFA, BGLAP, IL1B
7polyneuropathy10.8
8hemangioma10.7
9castleman's disease10.6
10endotheliitis10.6
11glomeruloid hemangioma10.6
12hypertension10.6
13osteosclerotic myeloma10.5
14neuropathy10.5
15extramedullary plasmacytoma10.5
16multiple myeloma10.4
17demyelinating polyneuropathy10.3
18plasmacytoma10.3
19calciphylaxis10.3
20peripheral neuropathy10.3
21cryoglobulinemia10.2
22cerebritis10.2
23amyloidosis10.2
24osteosclerosis10.2
25chronic inflammatory demyelinating polyneuropathy10.2
26engraftment syndrome10.2
27hematopoietic stem cell transplantation10.2
28chronic polyneuropathy10.2
29pulmonary fibrosis10.2
30polycythemia vera10.1
31cystoid macular edema10.1
32alopecia10.1
33congestive heart failure10.1
34myocardial infarction10.1
35polycythemia10.1
36thyroiditis10.1
37vitamin b12 deficiency10.1
38central retinal vein occlusion10.1VEGFA
39diabetic macular edema10.1VEGFA
40degenerative disc disease10.1IL1B
41corneal ulcer10.1CCL11
42periodontal disease10.1IL6
43meningioma10.1CST3
44synovitis10.1IL6, IL1B
45euthyroid sick syndrome10.1IL1B, IL6
46chorioamnionitis10.1IL1B, IL6
47eye disease10.0IL1B, IL6
48adult respiratory distress syndrome10.0IL1B, IL6
49respiratory failure10.0IL1B, IL6
50newborn respiratory distress syndrome10.0IL1B, IL6

Graphical network of the top 20 diseases related to Poems Syndrome:



Diseases related to poems syndrome

Symptoms for Poems Syndrome

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50Orphanet
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Symptoms:

50 (show all 23)
  • cortico-adrenal hypoplasia/insufficiency
  • renal disease/nephropathy
  • decreased body hair/axillar/pubic hairlessness
  • hypotrichosis/atrichia/atrichiasis/scalp hairlessness
  • gynecomastia/breast/mammary gland enlargement/hyperplasia
  • agammaglobulinemia/hypogammaglobulinemia/b-cell deficiency
  • nerve conduction abnormality
  • hypothalamic-hypophyseal axis functional anomalies/hypopituitarism
  • lymphadenopathy/polyadenopathies
  • splenomegaly
  • hepatomegaly/liver enlargement (excluding storage disease)
  • irregular/in bands/reticular skin hyperpigmentation
  • hirsutism/hypertrichosis/increased body hair
  • arteriovenous malformations/vascular malformations (excluding port-wine stains)
  • tapered fingers
  • bone/osseous neoplasm/tumor/carcinoma/cancer
  • osteosclerosis/osteopetrosis/bone condensation
  • precocious menopause/secondary amenorrhea
  • late puberty/hypogonadism/hypogenitalism
  • insulin-independent/type 2 diabetes
  • hypothyroidy
  • lymphedema
  • thick skin/pachydermia/orange skin

Drugs & Therapeutics for Poems Syndrome

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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Drug clinical trials:

Search ClinicalTrials for Poems Syndrome

Search NIH Clinical Center for Poems Syndrome

Search CenterWatch for Poems Syndrome

Genetic Tests for Poems Syndrome

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Anatomical Context for Poems Syndrome

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34MalaCards
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MalaCards organs/tissues related to Poems Syndrome:

34
Skin, Bone, Liver, Breast, Lymph node, Testes, Spleen, Lung, Endothelial, Bone marrow, B cells, Heart, Thyroid, Myeloid, Kidney, Colon, Pituitary

Animal Models for Poems Syndrome or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Poems Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.3CST3, IL1B, IL6, VEGFA
2MP:00020068.0CST3, IL1B, IL6, VEGFA

Publications for Poems Syndrome

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53PubMed
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Articles related to Poems Syndrome:

(show top 50)    (show all 420)
idTitleAuthorsYear
1
The characteristics of ascites in patients with POEMS syndrome. (23811954)
2013
2
Outcomes of patients with POEMS syndrome treated initially with radiation. (23699599)
2013
3
POEMS syndrome and Castleman disease. (24006526)
2013
4
POEMS syndrome presenting with acute demyelinating polyneuropathy: increased terminal latency indices and uniform demyelination. (23812201)
2013
5
Glomeruloid hemangiomas in the absence of POEMS syndrome. (22443389)
2012
6
Hemangioma in POEMS syndrome: an atypical clinical and histological presentation. (21749469)
2012
7
POEMS syndrome with prominent acute axonal lesions. (21982617)
2012
8
Comparison of imaging methods in POEMS syndrome. (22580861)
2012
9
Mobilization of PBSCs in poor mobilizers with POEMS syndrome using G-CSF with plerixafor. (22609884)
2012
10
The clinical study of POEMS syndrome in China. (20424579)
2010
11
Crow-Fukase (POEMS) syndrome]. (20420180)
2010
12
POEMS syndrome, congestive heart failure and thalidomide -food for thought. (20823658)
2010
13
POEMS syndrome - a unique presentation of a rare paraneoplastic syndrome. (22798312)
2010
14
A case of POEMS syndrome with mixed hyaline vascular and plasma cell type Castleman's disease. (19670555)
2009
15
POEMS syndrome with an isolated lytic lesion and glomeruloid hemangiomas: report of one case]. (19701559)
2009
16
Bilateral non-simultaneous optic neuropathy and unilateral macular edema in a patient with POEMS syndrome. (19123170)
2009
17
POEMS syndrome: a case report. (19119489)
2008
18
Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. (17605964)
2007
19
A case of POEMS syndrome with cystoid macular edema. (15767082)
2005
20
Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. (15975949)
2005
21
Restricted use of Vlambda genes in POEMS syndrome. (15075094)
2004
22
Thalidomide in POEMS syndrome: case report. (15114600)
2004
23
POEMS syndrome, fibrinogen, and ischemic stroke: a critical point of view. (14732636)
2004
24
POEMS syndrome. (14715103)
2003
25
Crow-Fukase (POEMS) syndrome: a study of peripheral nerve biopsy in five new cases. (12904234)
2003
26
Long-term follow-up on a patient with incomplete POEMS syndrome undergoing high-dose therapy and autologous blood stem cell transplantation. (12360984)
2002
27
POEMS syndrome: a case report and discussion. (11378655)
2001
28
Glomeruloid haemangioma and POEMS syndrome. (11572909)
2001
29
Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. (9517610)
1998
30
Abnormal FSH hypersecretion as an endocrinological manifestation of POEMS syndrome. (9625458)
1998
31
Arterial obliteration in POEMS syndrome: possible role of vascular endothelial growth factor. (9558193)
1998
32
Long term melphalan-prednisolone chemotherapy for POEMS syndrome. (9328260)
1997
33
Myeloneuropathy in POEMS syndrome. (8941279)
1996
34
Acute arterial obliteration: a new feature of the POEMS syndrome? (8699962)
1996
35
Pro-inflammatory cytokines: a pathogenic key of POEMS syndrome]. (8881441)
1996
36
Amyotrophic lateral sclerosis associated with multiple myeloma, endocrinopathy and skin changes suggestive of a POEMS syndrome variant. (7595623)
1995
37
Cryoglobulinemia and other dysproteinemias, familial Mediterranean fever, and POEMS syndrome. (7718425)
1995
38
POEMS syndrome in a female Italian patient. A case report. (8539476)
1995
39
POEMS syndrome: clinical, pathological and immunological study of a case. (7708142)
1994
40
Alopecia as a rare cutaneous manifestation of POEMS syndrome. (7918001)
1994
41
Castleman disease in POEMS syndrome with elevated interleukin-6. (8499003)
1993
42
POEMS syndrome, osteosclerotic myeloma and Castleman's disease: a case report. (1953537)
1991
43
Renal involvement in POEMS syndrome. (1944731)
1991
44
POEMS syndrome with idiopathic flushing mimicking carcinoid syndrome. (2029023)
1991
45
POEMS syndrome: a rarely diagnosed multisystem disorder. (2163715)
1990
46
The endocrinopathy of POEMS syndrome. (2555917)
1989
47
Refractory ascites due to POEMS syndrome. (2535829)
1989
48
POEMS syndrome associated with polycythemia vera. (2537136)
1989
49
POEMS syndrome associated with cryoglobulinemia, lymphoma, multiple seborrheic keratosis, and ichthyosis. (2848065)
1988
50
Renal ultrasound in POEMS syndrome. (3920286)
1985

Variations for Poems Syndrome

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Expression for genes affiliated with Poems Syndrome

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Poems Syndrome

Search GEO for disease gene expression data for Poems Syndrome.

Pathways for genes affiliated with Poems Syndrome

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Sources:
51PathCards, 31KEGG, 56Reactome, 39NCBI BioSystems Database, 54QIAGEN, 61Thomson Reuters, 55R&D Systems, 13EMD Millipore
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Pathways related to Poems Syndrome according to GeneCards/GeneDecks:

(show all 37)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5VEGFA, IL6
2
Show member pathways
9.5IL1B, IL6
3
Show member pathways
Immune response MIF in innate immunity response61
9.5IL6, IL1B
4
Show member pathways
thioredoxin pathway39
9.5IL1B, IL6
59.5IL6, IL1B
6
Show member pathways
IL27-mediated signaling events39
9.5IL6, IL1B
7
Show member pathways
9.5IL6, IL1B
89.5IL1B, IL6
99.5IL6, IL1B
10
Show member pathways
9.5IL6, IL1B
11
Show member pathways
9.5IL1B, IL6
12
Show member pathways
9.5IL6, IL1B
139.5IL1B, IL6
149.5IL6, IL1B
159.5IL6, IL1B
169.5IL6, IL1B
179.5IL1B, IL6
189.5IL1B, IL6
199.5IL1B, IL6
209.5IL6, IL1B
219.5IL1B, IL6
229.5IL6, IL1B
239.5IL6, IL1B
249.4CCL11, VEGFA
259.4IL6, BGLAP
269.4IL6, BGLAP
279.3CCL11, IL6
289.1IL1B, VEGFA, IL6
299.1IL6, IL1B, VEGFA
309.0IL6, BGLAP, IL1B
31
Show member pathways
8.9IL1B, IL6, CCL11
32
Show member pathways
Transcription NF kB signaling pathway61
Immune response Toll like receptor TLR ligands and common TLR signaling pathway leading to cell proinflammatory response61
8.9IL1B, IL6, CCL11
338.9CCL11, IL6, IL1B
34
Show member pathways
8.9IL1B, CCL11, IL6
35
Show member pathways
8.9CCL11, IL6, IL1B
36
Show member pathways
8.9CCL11, IL6, IL1B
37
Show member pathways
8.5VEGFA, CCL11, IL6, IL1B

Compounds for genes affiliated with Poems Syndrome

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Sources:
46Novoseek, 30IUPHAR, 12DrugBank, 52PharmGKB, 62Tocris Bioscience, 25HMDB, 3BitterDB
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Compounds related to Poems Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 93)
idCompoundScoreTop Affiliating Genes
1muramyl tripeptide phosphatidylethanolamine469.9IL1B, IL6
2turpentine469.9IL1B, IL6
3tenidap46 3010.8IL1B, IL6
4anakinra46 1210.8IL1B, IL6
5taurine chloramine469.8IL1B, IL6
6vesnarinone469.7IL6, IL1B
7hbd2469.7IL1B, IL6
8mip 1alpha469.6IL1B, IL6
9pd 169316469.5VEGFA, BGLAP
10bucillamine469.5VEGFA, IL1B, IL6
11triptolide469.5IL6, VEGFA, IL1B
12gm-csf46 3010.5IL1B, IL6, VEGFA
13risedronate46 52 30 62 1213.5IL1B, BGLAP
14ns 39846 6210.5BGLAP, VEGFA, IL1B
15zoledronic acid469.4BGLAP, IL6, VEGFA
16thalidomide46 52 62 1212.4VEGFA, IL6, IL1B
17clodronate46 52 1211.4IL6, IL1B, BGLAP
18helenalin469.4IL1B, IL6
19curcumin469.4IL6, VEGFA, IL1B
20simvastatin46 52 62 30 25 1214.3BGLAP, VEGFA, IL6
21hydrocortisone46 3 62 1212.3IL1B, BGLAP, IL6
22groalpha469.3CCL11, IL6, IL1B
23budesonide46 52 1211.3BGLAP, IL1B, CCL11
24fluticasone propionate46 52 62 1212.3BGLAP, CCL11, IL1B
25poly(i-c)469.3IL6, CCL11, IL1B
26peptidoglycan46 3010.3IL6, IL1B, CCL11
27ccl346 3010.3CCL11, IL1B, IL6
28leukotriene b446 62 2511.2CCL11, IL6, IL1B
29troglitazone46 30 62 1212.2IL6, CCL11, IL1B
30herbimycin a46 6210.2IL1B, IL6, CCL11
31calcitriol46 62 25 1212.2BGLAP, IL1B, IL6
32endotoxin469.2IL1B, CCL11, IL6
33prednisolone46 30 1211.2BGLAP, IL6, CCL11
34pamidronate46 52 1211.1IL6, BGLAP
35prostaglandin469.1IL1B, IL6, CCL11
36infliximab46 52 1211.0VEGFA, IL1B, IL6, BGLAP
3715-deoxy-delta-12,14-prostaglandin j2468.9IL1B, VEGFA, CCL11, IL6
38hyaluronic acid46 259.9VEGFA, CCL11, IL6, IL1B
39ly294002468.9IL6, VEGFA, CCL11, IL1B
40actinomycin d468.8VEGFA, CCL11, IL1B, IL6
41genistein46 30 62 3 25 1213.8IL6, CCL11, IL1B, VEGFA
42histamine46 30 2510.8IL1B, BGLAP, CCL11, IL6
43methotrexate52 46 1210.7CST3, IL6, IL1B
44sb 20358046 629.4IL1B, IL6, CCL11, VEGFA, BGLAP
45pge2468.4IL6, CCL11, IL1B, VEGFA, BGLAP
46cyclosporin a46 30 6210.4CST3, IL6, BGLAP, CCL11
47alanine468.1CCL11, IL6, BGLAP, CST3
48n acetylcysteine468.1CST3, IL1B, IL6, CCL11, VEGFA
49dexamethasone46 52 30 1210.6VEGFA, CCL11, IL6, IL1B, CST3, BGLAP
50vegf467.6CST3, BGLAP, VEGFA, CCL11, IL6, IL1B

GO Terms for genes affiliated with Poems Syndrome

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17Gene Ontology
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Cellular components related to Poems Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.6IL1B, VEGFA
2extracellular spaceGO:0056157.0CST3, IL1B, IL6, CCL11, VEGFA, BGLAP
3extracellular regionGO:0055766.8BGLAP, VEGFA, CCL11, IL6, IL1B, CST3

Biological processes related to Poems Syndrome according to GeneCards/GeneDecks:

(show all 18)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of vascular endothelial growth factor receptor signaling pathwayGO:0309499.9IL1B, VEGFA
2positive regulation of cell divisionGO:0517819.9IL1B, VEGFA
3positive regulation of nitric oxide biosynthetic processGO:0454299.8IL1B, IL6
4positive regulation of interleukin-6 productionGO:0327559.8IL1B, IL6
5positive regulation of peptidyl-serine phosphorylationGO:0331389.8VEGFA, IL6
6positive regulation of T cell proliferationGO:0421029.8IL6, IL1B
7positive regulation of epithelial cell proliferationGO:0506799.7IL6, VEGFA
8negative regulation of cysteine-type endopeptidase activity involved in apoptotic processGO:0431549.7VEGFA, IL6
9response to mechanical stimulusGO:0096129.6IL6, BGLAP
10positive regulation of peptidyl-tyrosine phosphorylationGO:0507319.6VEGFA, IL6
11positive regulation of endothelial cell proliferationGO:0019389.6CCL11, VEGFA
12response to glucocorticoidGO:0513849.4IL6, BGLAP
13positive regulation of sequence-specific DNA binding transcription factor activityGO:0510919.4IL1B, IL6
14positive regulation of protein phosphorylationGO:0019349.3VEGFA, IL1B
15positive regulation of angiogenesisGO:0457669.3IL1B, CCL11, VEGFA
16regulation of cell shapeGO:0083609.2IL6, CCL11, VEGFA
17inflammatory responseGO:0069549.2CCL11, IL6, IL1B
18positive regulation of transcription from RNA polymerase II promoterGO:0459449.1IL1B, IL6, VEGFA

Molecular functions related to Poems Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051259.1IL1B, IL6, VEGFA

Products for genes affiliated with Poems Syndrome

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Sources for Poems Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet