cystic medial degeneration

嚢胞性中膜変性？ CMD

WordNet

1. the process of declining from a higher to a lower level of effective power or vitality or essential quality (同)devolution
2. passing from a more complex to a simpler biological form (同)retrogression
3. dividing an animal into right and left halves (同)median
4. of or relating to or resembling a cyst
5. of or relating to a normal cyst (as the gallbladder or urinary bladder)
6. a closed sac that develops abnormally in some body structure

PrepTutorEJDIC

1. 退歩,退化,墜落
2. 中間の,中間に位置する / 平均の,並なみの
3. 胞嚢(ほうのう)性の
4. 胞嚢(ほうのう)
5. mediumの複数形

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/01/29 08:47:02」(JST)

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[Wiki en表示]

Familial thoracic aortic aneurysm
Classification and external resources
Micrograph showing cystic medial degeneration, the histologic correlate of familial thoracic aortic aneurysms. The image shows abundant basophilic ground substance in the tunica media (blue at top of image) and disruption of the elastic fibers. The tunica adventitia (yellow at bottom of image) with vaso vasorum is also seen. Movat's stain.
ICD-9	441.00
OMIM	607086
DiseasesDB	30073
eMedicine	emerg/28

Familial thoracic aortic aneurysm (also referred to as familial aortic dissection or cystic medial necrosis of aorta) is an autosomal dominant disorder of large arteries.

There is an association between familial thoracic aortic aneurysm, Marfan syndrome and massive baclofen overdose as well as other hereditary connective tissue disorders.

Presentation

A degenerative breakdown of collagen, elastin, and smooth muscle caused by aging contributes to weakening of the wall of the artery.

In the aorta, this can result in the formation of a fusiform aneurysm. There is also increased risk of aortic dissection.

Genetics

Types include:

Type	OMIM	Gene	Locus
AAT1	607086		11q23.3-q24
AAT4	132900	MYH11	16p
AAT6	611788	ACTA2	10q

Terminology

It is sometimes called "Erdheim cystic medial necrosis of aorta", after Jakob Erdheim.

The term "cystic medial degeneration" is sometimes used instead of "cystic medial necrosis", because necrosis is not always found.

References

See also

• Aneurysm

External links

• GeneReview/NCBI/NIH/UW entry on Thoracic Aortic Aneurysms and Aortic Dissections
• 1449852953 at GPnotebook

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 大動脈解離の臨床症状および診断 clinical manifestations and diagnosis of aortic dissection
• 2. マルファン症候群および関連疾患の遺伝学、臨床的特徴、および診断 genetics clinical features and diagnosis of marfan syndrome and related disorders
• 3. 胸部大動脈瘤の疫学、危険因子、病因、および自然史 epidemiology risk factors pathogenesis and natural history of thoracic aortic aneurysm
• 4. Extracranial carotid artery aneurysm
• 5. 特発性脳動脈および頚動脈の解離：臨床的特徴および診断 spontaneous cerebral and cervical artery dissection clinical features and diagnosis

English Journal

• Spontaneous rupture of an isolated iliac artery dissection in a young man because of cystic medial degeneration Erdheim-Gsell.

• Dueppers P1, Jankowiak S2, Schelzig H3, Wagenhäuser MU3, Oberhuber A3.
• Annals of vascular surgery.Ann Vasc Surg.2015 Apr;29(3):596.e11-3. doi: 10.1016/j.avsg.2014.10.041. Epub 2015 Jan 14.
• BACKGROUND: Rupture of isolated iliac artery dissections is a life-threatening event and has not been associated with cystic medial degeneration (CMD) Erdheim-Gsell.METHODS: A young man presented to our emergency unit in a critical hemodynamic condition. Computed tomography scan feigned the rupture
• PMID 25596406

• Massive carotid artery aneurysm presenting as an oropharyngeal swelling in a young woman.

• Chang KH1, Cotter J1, McGreal GT1.
• BMJ case reports.BMJ Case Rep.2015 Jan 30;2015. pii: bcr2014206810. doi: 10.1136/bcr-2014-206810.
• A 28-year-old woman presented to the emergency department with a 5-day history of a sore throat and a pharyngeal swelling that increased in size. The patient was previously investigated for Marfan's syndrome after episodes of bilateral spontaneous pneumothoraces but did not meet the criteria for dia
• PMID 25636627

• Identification of three particular morphological phenotypes in sporadic thoracic aortic aneurysm: phenotype III as sporadic thoracic aortic aneurysm biomarker in aged individuals.

• Balistreri CR1, Maresi E, Pisano C, Di Maggio FM, Vaccarino L, Caruso C, Lio D, Ruvolo G, Candore G.
• Rejuvenation research.Rejuvenation Res.2014 Apr;17(2):192-6. doi: 10.1089/rej.2013.1505. Epub 2014 Apr 8.
• Aging has a striking impact on the heart and the vascular system, and particularly on the large elastic arteries (i.e., aorta), resulting in a multitude of changes at different structural and functional levels. As result, medial degeneration (MD) occurs. A characteristic example of MD is sporadic th
• PMID 24066724

Japanese Journal

• Positive Correlation Between the Dysregulation of Transforming Growth Factor-β1 and Aneurysmal Pathological Changes in Patients With Marfan Syndrome

• Kim Koung Li,Yang Jeong Hoon,Song Sun-Hwa,Kim Ji Yeon,Jang Shin Yi,Kim Jeong-Min,Kim Jee-Ah,Sung Ki-Ick,Kim Young-Wook,Suh Yeon-Lim,Suh Wonhee,Kim Duk-Kyung
• Circulation Journal 77(4), 952-958, 2013
• … Background: Our goal was to investigate the correlation between the dysregulation of transforming growth factor-β1 (TGF-β1) and cystic medial degeneration in the aortic aneurysmal tissues of in Marfan syndrome (MFS) patients. …
• NAID 130003361588

• Aneurysm of the Internal Mammary Artery with Cystic Medial Degeneration

• Okura Yuji,Kawasaki Takashi,Hiura Tohru,Seki Hiroshi,Saito Hirofumi
• Internal Medicine 51(17), 2355-2359, 2012
• … Given that a rupture of the aneurysm could have been life-threatening, it was removed surgically, and found to possess a paper-thin arterial wall with cystic medial degeneration. …
• NAID 130002062367

• RECS1 is a Negative Regulator of Matrix Metalloproteinase-9 Production and Aged RECS1 Knockout Mice are Prone to Aortic Dilation(EXPERIMENTAL INVESTIGATION)

• Zhao Hanjun,Ito Akihiko,Sakai Naohiko,Matsuzawa Yuji,Yamashita Shizuya,Nojima Hiroshi
• Circulation journal : official journal of the Japanese Circulation Society 70(5), 615-624, 2006-04-20
• … Background RECS1 is a mechanical stress responsive gene and RECS1 knockout (KO) mice (older than 14 months) are prone to cystic medial degeneration (CMD). …
• NAID 110004720684

Related Links

• Familial thoracic aortic aneurysm - Wikipedia, the free encyclopedia

[edit] Terminology. It is sometimes called "Erdheim cystic medial necrosis of aorta ", after Jakob Erdheim. The term "cystic medial degeneration" is sometimes used instead of "cystic medial necrosis", because necrosis is not always found.

• Cystic medial necrosis: pathological findings and clinical implications.

Cystic medial necrosis: pathological findings and clinical implications. [Article in English, Portuguese]. Yuan SM, Jing H. Department of Cardiothoracic Surgery, Affiliated Hospital of Taishan Medical College, Taian 27100, Shandong Province, ...

Related Pictures

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★リンクテーブル★

リンク元	「嚢胞性中膜変性」「CMD」
関連記事	「degeneration」「medial」「media」「cystic」

「嚢胞性中膜変性」

　　[★]

英

cystic medial degeneration CMD

関

[[]]

[show details]

嚢胞性中膜変性 : 7 件
嚢胞性内側変性 : 1 件

「CMD」

　　[★]

• congenital muscular dystrophy, 先天性筋ジストロフィ症
• 嚢胞性中膜変性 cystic medial degeneration

「degeneration」

　　[★]

• n.

• 変性、変性症、逆行変性、縮退、退化

関

degeneracy、degenerate、degenerative、denaturation、denature、involution、retract、retrograde degeneration、retrograde degradation、unfolding

　 　 　

「medial」

　　[★]

• adj.

• 内側の、中心の、中央の、内側性の

関

center、central、centre、centro、centrum、hub、inner、inside、interior、medialis、medially、middle

　 　

「media」

　　[★]

• n.

関

culture、culture media、culture medium、culture solution、medium

　

「cystic」

　　[★]

• adj.

• 嚢胞性の