aortic arch syndrome

大動脈弓症候群 AAS

WordNet

1. a passageway under a curved masonry construction; "they built a triumphal arch to memorialize their victory" (同)archway
2. (architecture) a masonry construction (usually curved) for spanning an opening and supporting the weight above it
3. a curved shape in the vertical plane that spans an opening
4. expert in skulduggery; "an arch criminal"
5. a curved bony structure supporting or enclosing organs (especially the inner sides of the feet)
6. (used of behavior or attitude) characteristic of those who treat others with condescension (同)condescending, patronizing, patronising
7. form an arch or curve; "her back arches"; "her hips curve nicely" (同)curve, arc
8. naughtily or annoyingly playful; "teasing and worrying with impish laughter"; "a wicked prank" (同)impish, implike, mischievous, pixilated, prankish, puckish, wicked
9. a pattern of symptoms indicative of some disease
10. a complex of concurrent things; "every word has a syndrome of meanings"
11. a continuous portion of a circle
12. constructed with or in the form of an arch or arches; "an arched passageway"

PrepTutorEJDIC

1. (建物の)『アーチ』,迫持(せりもち) / アーチ型の記念碑,弓形門 / 『弓形のもの』 / (足の)土踏まず / …'を'弓形にする / …‘に'アーチをかける / 弓形になる,弓状に伸びる
2. 《名詞の前にのみ用いて》おもな,主要な(chief) / (おもに女・子供が)ずるそうな,いたずらそうな(mischievous)
3. (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
4. 『弧』,円弧 / 電弧(2点間を弧状に流れる電流) / 弧を描く / 電弧を生じる
5. アーチ形の,弓形の / アーチのある
6. are (面積の単位)の変形

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/03/04 12:57:00」(JST)

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Takayasu's arteritis
Classification and external resources
Left anterior oblique angiographic image of Takayasu's arteritis showing areas of stenosis in multiple great vessels
ICD-10	M31.4
ICD-9	446.7
OMIM	207600
DiseasesDB	12879
MedlinePlus	001250
eMedicine	med/2232 ped/1956 neuro/361 radio/51
MeSH	D013625

Takayasu's disease (also known as "aortic arch syndrome", "nonspecific aortoarteritis" and the "pulseless disease") is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing affecting often young or middle-aged women of Asian descent. It mainly affects the aorta (the main blood vessel leaving the heart) and its branches, as well as the pulmonary arteries. Females are about 8-9 times more likely to be affected than males. Patients often notice the disease symptoms between 15 and 30 years of age. In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than Takayasu's arteritis. Takayasu's arteritis is similar to other forms of vasculitis, including giant cell arteritis. Due to obstruction of the main branches of the aorta, including the left common carotid artery, the brachiocephalic artery, and the left subclavian artery, Takayasu's arteritis can present as pulseless upper extremities (arms, hands, and wrists with weak or absent pulses on the physical examination) which may be why it is also commonly referred to as the "pulseless disease."

Pathophysiology

Axial T1-weighted post-gadolinium MRI in a patient with Takayasu arteritis showing thickened, enhancing aortic wall, consistent with large vessel vasculitis

Although its etiology is unknown, the condition is characterized by segmental and patchy granulomatous inflammation of the aorta and its major derivative branches. This inflammation leads to arterial stenosis, thrombosis, and aneurysms. There is also irregular fibrosis of the blood vessels due to chronic vasculitis leading to sometimes massive intimal fibrosis (fibrosis of the inner section of the blood vessels). Prominent narrowing due to inflammation, granuloma, and fibrosis is often seen in arterial studies such as Magnetic resonance angiography (MRA), computed tomography angiography (CTA), or arterial angiography (DSA).

Symptoms

it is a pulse less disease.pulses are absent,severe muscle pain, Some patients develop an initial "inflammatory phase" characterized by systemic illness with symptoms of malaise, fever, night sweats, weight loss, arthralgia, and fatigue. There is also often anemia and marked elevation of the ESR or C-reactive protein (nonspecific markers of inflammation). The initial "inflammatory phase" is often followed by a secondary "pulseless phase." The "pulseless phase" is characterized by vascular insufficiency from intimal narrowing of the vessels manifesting as arm or leg claudication, renal artery stenosis causing hypertension, and neurological manifestations due to decreased blood flow to the brain. Of note is the function of renal artery stenosis in causation of high blood pressure: Normally perfused kidneys produce proportionate amount of a substance called renin. Stenosis of the renal arteries, causes hypoperfusion (decreased blood flow) of the juxtaglomerular apparatus, resulting in exaggerated secretion of renin, and high blood levels of aldosterone, eventually leading to water and salt retention and high blood pressure. The neurological symptoms of the disease vary depending on the degree, and the nature of the blood vessel obstruction and can range from lightheadedness, to seizures in severe cases. One rare but important feature of the Takayasu's arteritis is ocular involvement in form of visual field defects, vision loss, or retinal hemorrhage Some patients with Takayasu's arteritis may present with only late vascular changes, without an antecedent systemic illness. In the late stage, weakness of the arterial walls may give rise to localized aneurysms. As with all aneurysms, possibility of rupture and vascular bleeding is existent and requires monitoring. Raynaud's phenomenon is commonly found in this disease, mainly due to decreased circulation of the blood to the arms.

Treatments

The great majority of patients with Takayasu’s arteritis respond to steroids such as prednisone. The usual starting dose is approximately 1 milligram per kilogram of the body weight per day (for most people, this is approximately 60 milligrams a day). Because of the significant side effects of long-term high–dose prednisone use, the starting dose is tapered over several weeks to a dose that the physician feels is tolerable for the patient. Along with prednisone inj.cyclophosmide for 6 months then convert to tab.azoron or tab.methotextrate for strenthing of arteries or to prevent cancer. supplementations like calcium,iron folic tablets can prescribe Surgical options may need to be explored for patients who do not respond to steroids. Re-perfusion of tissue can be achieved by large vessel reconstructive surgery such as bypass grafting. Grafting autologous tissue has the highest rates of success. Percutaneous transluminal coronary angioplasty (PTCA) is not as effective in the long term but has fewer risks.

History

The first case of Takayasu’s arteritis was described in 1908 by a Japanese ophthalmologist, Mikito Takayasu at the Annual Meeting of the Japan Ophthalmology Society. Takayasu described a peculiar "wreathlike" appearance of the blood vessels in the back of the eye (retina). Two Japanese physicians at the same meeting (Drs. Onishi and Kagoshima) also reported similar eye findings in patients whose wrist pulses were absent. It is now known that the blood vessel malformations that occur in the retina are an angiogenic response to the arterial narrowings in the neck, and that the absence of pulses noted in some patients occurs because of narrowings of the blood vessels to the arms. The eye findings described by Takayasu are rarely seen in patients from North America and British Columbia^{[citation needed]}.

References

External links

• Johns Hopkins Vasculitis Center Discusses Takayasu's Arteritis
• Takayasu's Arteritis Research Association
• 00389 at CHORUS
• -362086386 at GPnotebook

UpToDate Contents

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• 1. 胸部大動脈瘤の臨床的特徴および診断 clinical features and diagnosis of thoracic aortic aneurysm
• 2. 腹部大動脈瘤の疫学、危険因子、病因および自然経過 epidemiology risk factors pathogenesis and natural history of abdominal aortic aneurysm
• 3. 血管内腹部大動脈修復術の合併症 complications of endovascular abdominal aortic repair
• 4. 腹部大動脈瘤の血管内修復 endovascular repair of abdominal aortic aneurysm
• 5. 胸部大動脈の鈍的損傷の手術および血管内修復 surgical and endovascular repair of blunt thoracic aortic injury

English Journal

• Takayasu arteritis is characterised by disturbances of B cell homeostasis and responds to B cell depletion therapy with rituximab.

• Hoyer BF, Mumtaz IM, Loddenkemper K, Bruns A, Sengler C, Hermann KG, Maza S, Keitzer R, Burmester GR, Buttgereit F, Radbruch A, Hiepe F.SourceCorrespondence to Falk Hiepe, Department of Medicine, Clinic for Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin and German Rheumatism Research Center Berlin-Leibniz Institute, Charitéplatz 1, D-10117 Berlin, Germany; falk.hiepe@charite.de.
• Annals of the rheumatic diseases.Ann Rheum Dis.2012 Jan;71(1):75-9. Epub 2011 Sep 27.
• Introduction Takayasu arteritis (TA) is a large vessel vasculitis involving the aorta and its major branches. T cell-mediated autoimmunity is thought to play a major role in its pathogenesis, while the role of B cells is still unclear.METHODS: B cell subsets in the peripheral blood of 17 patients wi
• PMID 21953334

• Cardiovascular defects in a mouse model of HOXA1 syndrome.

• Makki N, Capecchi MR.AbstractCongenital heart disease is one of the most common human birth defects, yet many genes and pathways regulating heart development remain unknown. A recent study in humans revealed that mutations in a single Hox gene, HOXA1 (Athabascan Brainstem Dysgenesis Syndrome, Bosley-Salih-Alorainy Syndrome), can cause severe cardiovascular malformations, some of which are lethal without surgical intervention. Since the discovery of the human syndromes, there have been no reports of any Hox mouse mutants with cardiac defects, hampering studies to explore the developmental causes of the human disease. In this study, we identify severe cardiovascular malformations in a Hox mouse model, which mimic the congenital heart defects in HOXA1 syndrome patients. Hoxa1 null mice show defects such as interrupted aortic arch, aberrant subclavian artery and Tetralogy of Fallot, demonstrating that Hoxa1 is required for patterning of the great arteries and outflow tract of the heart. We show that during early embryogenesis, Hoxa1 is expressed in precursors of cardiac neural crest cells (NCCs), which populate the heart. We further demonstrate that Hoxa1 acts upstream of several genes, important for neural crest specification. Thus, our data allow us to suggest a model in which Hoxa1 regulates heart development through its influence on cardiac NCCs, providing insight into the mechanisms underlying the human disease.
• Human molecular genetics.Hum Mol Genet.2012 Jan 1;21(1):26-31. Epub 2011 Sep 22.
• Congenital heart disease is one of the most common human birth defects, yet many genes and pathways regulating heart development remain unknown. A recent study in humans revealed that mutations in a single Hox gene, HOXA1 (Athabascan Brainstem Dysgenesis Syndrome, Bosley-Salih-Alorainy Syndrome), ca
• PMID 21940751

Japanese Journal

• Fontan Completion Following Flow Adjustable Bilateral Pulmonary Artery Banding

• Yoshii Takeshi,Miyamoto Takashi,Inui Akitoshi,Tanaka Yuuki,Yoshitake Shuichi,Seki Mitsuru,Kobayashi Tomio
• International Heart Journal, 2014
• … We have performed bilateral pulmonary artery banding operations combined with planned percutaneous balloon dilatation at banding sites for patients with hypoplastic left heart syndrome and related anomalies. … Here, we report a case of Fontan completion in a patient who underwent aortic arch repair and a bidirectional Glenn procedure following flowadjustable bilateral pulmonary artery banding. …
• NAID 130004699230

• 右肺低形成・left pulmonary artery sling・bridging bronchus を合併した先天性気管狭窄症に対してスライド気管形成術を施行した1 例

• 大片 祐一,横井 暁子,尾藤 祐子,中尾 真,園田 真理,谷本 光隆,吉田 拓哉,長谷川 智巳,大嶋 義博,西島 栄治
• 日本小児外科学会雑誌 50(5), 890-894, 2014
• 右肺低形成・left pulmonary artery sling・bridging bronchus を合併した先天性気管狭窄症に対して大動脈前方でのスライド気管形成術を行い良好な経過を得たので報告する．症例は9 か月女児．上気道炎に伴い喘鳴が悪化し，胸部造影CT にて右肺低形成を伴う先天性気管狭窄症と診断され当科入院となった．受診時は軽度の狭窄音を認める程度であったが入院4 日目に持続する啼泣 …
• NAID 130004684299

• An Aberrant Subclavian Artery Exhibiting the Partial Steal Phenomenon in a Patient with VACTERL Association

• Budincevic Hrvoje,Starcevic Katarina,Bielen Ivan,Demarin Vida
• Internal Medicine 53(16), 1859-1861, 2014
• … CT angiography of the neck vessels and aortic arch confirmed an anomalous right subclavian artery -known as the lusorian artery. … Further studies are warranted in patients with VACTERL in order to identify possible links between the prevalence of an aberrant right subclavian artery (lusorian artery) and possible congenital subclavian steal syndrome or dysphagia lusoria. …
• NAID 130004678415

Related Links

• Aortic arch syndrome | definition of aortic arch syndrome by ...

aortic [a-or´tik] pertaining to the aorta. aortic arch syndrome any of a group of disorders adding to occlusion of the arteries arising from the aortic arch; such occlusion may be caused by atherosclerosis, arterial embolism, or other ...

• Aortic arch syndrome: MedlinePlus Medical Encyclopedia

Aortic arch syndrome refers to a group of signs and symptoms associated with structural problems in the arteries that branch off the aortic arch. The aortic arch is the top part of the main artery carrying blood away from ...

Related Pictures

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★リンクテーブル★

リンク元	「AAS」「大動脈弓症候群」
関連記事	「arc」「arch」「aortic」「syndrome」

「AAS」

　　[★]

• 大動脈弓症候群 aortic arch syndrome
• 同化男性ステロイド anabolic-androgenic steroids
• アレルギー性アスペルギルス副鼻腔炎 allergic Aspergillus sinusitis
• 環軸亜脱臼 環軸関節亜脱臼 atlantoaxial subluxation

「大動脈弓症候群」

　　[★]

英

aortic arch syndrome, AAS

関

Takayasu's arteritis

「arc」

　　[★]

• n.

• アーク、弧、電弧

関

AIDS-related complex、lymphadenopathy syndrome

　 　

「arch」

　　[★]

• n.

• アーチ

• adj.

• 弓状の

関

arcuate

　 　 　 　 　 　 　 　 　 　

「aortic」

　　[★]

• adj.

• 大動脈の

関

aorta、aortae、aortal

「syndrome」

　　[★]

• n.

• 症候群