MCID: CYS023
MIFTS: 11

Cystic Medial Necrosis of Aorta

Categories: Rare diseases

Aliases & Classifications for Cystic Medial Necrosis of Aorta

MalaCards integrated aliases for Cystic Medial Necrosis of Aorta:

Name: Cystic Medial Necrosis of Aorta 52
Erdheim Cystic Medial Necrosis of Aorta 52
Erdheim Disease 52

Classifications:



Summaries for Cystic Medial Necrosis of Aorta

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 229 Definition Familial aortic dissection is the term used to describe rupture of the aortic wall at the level of the media, resulting in the formation of a false channel and deviation of part of the aortic flux. Familial predisposition to thoracic aortic aneurysms and type A dissections (concerning the ascending aorta and/or the aortic arch) has been demonstrated in around 19% of patients presenting with thoracic aortic dissections and several loci have been identified so far (16p12.2-p13.13, 3p24-25). This predisposition is transmitted in an autosomal dominant manner. Visit the Orphanet disease page for more resources.

MalaCards based summary : Cystic Medial Necrosis of Aorta, also known as erdheim cystic medial necrosis of aorta, is related to aortic aneurysm, familial thoracic 1 and histiocytosis. Affiliated tissues include bone, brain and lung.

Related Diseases for Cystic Medial Necrosis of Aorta

Diseases related to Cystic Medial Necrosis of Aorta via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 aortic aneurysm, familial thoracic 1 11.7
2 histiocytosis 10.2
3 langerhans cell histiocytosis 10.1
4 non-langerhans-cell histiocytosis 10.0
5 diabetes insipidus 10.0
6 splenic artery aneurysm 9.8
7 adenoma 9.8
8 aneurysm 9.8
9 pituitary tumors 9.8

Graphical network of the top 20 diseases related to Cystic Medial Necrosis of Aorta:



Diseases related to Cystic Medial Necrosis of Aorta

Symptoms & Phenotypes for Cystic Medial Necrosis of Aorta

Drugs & Therapeutics for Cystic Medial Necrosis of Aorta

Search Clinical Trials , NIH Clinical Center for Cystic Medial Necrosis of Aorta

Genetic Tests for Cystic Medial Necrosis of Aorta

Anatomical Context for Cystic Medial Necrosis of Aorta

MalaCards organs/tissues related to Cystic Medial Necrosis of Aorta:

40
Bone, Brain, Lung, Pituitary

Publications for Cystic Medial Necrosis of Aorta

Articles related to Cystic Medial Necrosis of Aorta:

(show all 15)
# Title Authors PMID Year
1
In-flight incapacitation and cystic medial necrosis. 61
24024313 2013
2
[The rupture of splenic artery aneurysm in Erdheim disease]. 61
22352219 2012
3
Extracerebral subdural manifestation of Chester-Erdheim disease associated with a giant adenoma of the pituitary. 61
14531550 2003
4
[A case of Chester-Erdheim disease with unusual bone involvement]. 61
11677449 2001
5
Chester-Erdheim disease: a neoplastic disorder. 61
10492045 1999
6
Brain stem infiltration by mixed Langerhans cell histiocytosis and Chester-Erdheim disease: more than just an isolated case? 61
10398577 1999
7
Pathology of the central nervous system in Chester-Erdheim disease: report of three cases. 61
9370231 1997
8
Neuroradiologic aspects of Chester-Erdheim disease. 61
7611030 1995
9
[Three cases of familial dissecting aortic aneurysm]. 61
2246538 1990
10
Polymyositis associated with dissecting aneurysm of arteries and intracerebral hemorrhage. 61
3776535 1986
11
Immunocytochemical characterization of pulmonary histiocytosis X cells in lung biopsies. 61
3872076 1985
12
[Role of Chester-Erdheim disease in histiocytosis]. 61
6287637 1982
13
Lipid (cholesterol) granulomatosis (Chester-Erdheim disease) and congenital megacalices. 61
699452 1978
14
[Spontaneous rupture of the aorta with special reference to idiopathic cystic aortic medionecrosis (Gsell-Erdheim disease)]. 61
4223308 1965
15
[2 clinically diagnosed cases of idiopathic median necrosis of the aorta (Gsell-Erdheim disease)]. 61
14456070 1961

Variations for Cystic Medial Necrosis of Aorta

Expression for Cystic Medial Necrosis of Aorta

Search GEO for disease gene expression data for Cystic Medial Necrosis of Aorta.

Pathways for Cystic Medial Necrosis of Aorta

GO Terms for Cystic Medial Necrosis of Aorta

Sources for Cystic Medial Necrosis of Aorta

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....