WordNet

sound loudly and sonorously; "the bells rang" (同)peal
a toroidal shape; "a ring of ships in the harbor"; "a halo of smoke" (同)halo, annulus, doughnut, anchor ring
jewelry consisting of a circlet of precious metal (often set with jewels) worn on the finger; "she had rings on every finger"; "he noted that she wore a wedding band" (同)band
a characteristic sound; "it has the ring of sincerity"
a platform usually marked off by ropes in which contestants box or wrestle
the sound of a bell ringing; "the distinctive ring of the church bell"; "the ringing of the telephone"; "the tintinnabulation that so voluminously swells from the ringing and the dinging of the bells"--E. A. Poe (同)ringing, tintinnabulation
attach a ring to the foot of, in order to identify; "ring birds"; "band the geese to observe their migratory patterns" (同)band
make (bells) ring, often for the purposes of musical edification; "Ring the bells"; "My uncle rings every Sunday at the local church" (同)knell
wearing a wedding ring; lawfully married; "a ringed wife"- Tennyson
having colored rings around the body
gymnastic apparatus consisting of a pair of heavy metal circles (usually covered with leather) suspended by ropes; used for gymnastic exercises; "the rings require a strong upper body"
the giving of a ring as a token of engagement

PrepTutorEJDIC

『指輪』 / 『輪』,環;輪形,円形 / (円形の)サーカス演技場,公演場,競技場;(ボクシング・レスリングの)リング / 《複数形で》(体操の)つり輪 / (不法な目的で結託した)(…の)徒党,一味《+『of』+『名』》 / (原子の)環 / …を円形に取り囲む,円で囲む《+『about』(『around』,《英》『round』)+『名,』+『名』+『about』(『around』,《英》『round』)+『名』》 / 〈動物〉‘に'鼻輪(首輪,足輪)をはめる / (遊びで)…‘に'輪を投げる / 環状に動く,輪を描く
〈鐘・ベルなどが〉『鳴る』 / 《『ring』+『形』〈補〉》(…のように)聞こえる / (…を)ベルを鳴らして求める《+『for』+『名』》 / 〈音声・楽器などが〉鳴り響く;〈場所が〉(音などで)満ちる《+『with』+『名』》 / 耳鳴りがする / 〈鐘・ベルなど〉‘を'『鳴らす』 / 〈人〉‘を'(鐘などを)『鳴らして呼ぶ』;…‘を'鐘を鳴らして知らせる / 《英》…‘に'電話をかける《+『up』+『名』,+『名』+『up』》 / 《単数形で》(鐘・ベルなどの)『鳴る音,響き』《+『of』+『名』》 / 鐘(ベルなど)を鳴らすこと;ベル(など)が鳴ること / 《単数形で》(…の)固有の響き,調子,感じ《+『of』+『名』》 / 《話》電話をかけること
輪にある;環状の / 指輪をはめた

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/14 16:50:39」(JST)

wiki en

Kayser–Fleischer rings (KF rings) are dark rings that appear to encircle the iris of the eye. They are due to copper deposition in part of the cornea (Descemet's membrane) as a result of particular liver diseases.^[1] They are named after Dr. Bernhard Kayser and Dr. Bruno Fleischer, the German doctors who first described them in 1902 and 1903.^[2]^[3]^[4] Initially thought to be due to the accumulation of silver, they were first demonstrated to contain copper in 1934.^[5]

Detection

As Kayser–Fleischer rings do not cause any symptoms, it is common for them to be identified during investigations for other medical conditions. In certain situations, they are actively sought; in that case, the early stages may be detected by slit lamp examination before they become visible to the naked eye.^[1]

Appearance

The rings, which consist of copper deposits where the cornea meets the sclera, in Descemet's membrane, first appear as a crescent at the top of the cornea. Eventually, a second crescent forms below, at 6 o'clock, and ultimately completely encircles the cornea.^[1]^[6]

Associations

Kayser–Fleischer rings are a sign of Wilson's disease, which involves abnormal copper handling by the liver resulting in copper accumulation in the body and is characterised by abnormalities of the basal ganglia of the brain, liver cirrhosis, splenomegaly, involuntary movements, muscle rigidity, psychiatric disturbances, dystonia and dysphagia. The combination of neurological symptoms, a low blood ceruloplasmin level and KF rings is diagnostic of Wilson's disease.^[1]

Other causes of KF rings are cholestasis (obstruction of the bile ducts), primary biliary cirrhosis and "cryptogenic" cirrhosis (cirrhosis in which no cause can be identified).^[1]

References

^ ^a ^b ^c ^d ^e McDonnell G, Esmonde T (1999). "A homesick student". Postgrad Med J 75 (884): 375–8. PMC 1741256. PMID 10435182.
^ Kayser B (1902). "Über einen Fall von angeborener grünlicher Verfärbung des Cornea". Klin Monatsbl Augenheilk 40 (2): 22–25.
^ Fleischer B (1903). "Zwei weitere Fälle von grünlicher Verfärbung der Kornea". Klin Monatsbl Augenheilk 41 (1): 489–491.
^ synd/1758 at Who Named It?
^ Gerlach W, Rohrschneider W (1934). "Besteht das Pigment des Kayser-Fleischerschen Hornhautringes aus Silber?". Klin Wochenschr 13: 48–49. doi:10.1007/BF01799043.
^ -1684406263 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. ウィルソン病：診断のための検査 wilson disease diagnostic tests
2. ウィルソン病：臨床症状、診断、および自然史 wilson disease clinical manifestations diagnosis and natural history
3. Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis (primary biliary cirrhosis)
4. ウィルソン病：疫学および病因 wilson disease epidemiology and pathogenesis
5. ウィルソン病：治療および予後 wilson disease treatment and prognosis

English Journal

The sunflower cataract in Wilson's disease: pathognomonic sign or rare finding?

Langwińska-Wośko E1,2, Litwin T3, Dzieżyc K1, Członkowska A1,4.
Acta neurologica Belgica.Acta Neurol Belg.2015 Nov 17. [Epub ahead of print]
The presence of Kayser-Fleischer ring in patients with Wilson's disease (WD) is well documented and included in diagnostic algorithms; however, data about the occurrence of the second postulated ophthalmological sign of WD, sunflower cataract (SC), are limited and even conflicting. The aim of our st
PMID 26577266

Bilateral pallidal deep brain stimulation in idiopathic dystonic camptocormia.

Yadav R, Ansari AZ, Surathi P, Srinivas D1, Somanna S, Pal P.
Neurology India.Neurol India.2015 Nov-Dec;63(6):911-4.
INTRODUCTION: Camptocormia is seen with Parkinson's disease (PD), segmental or generalized dystonia, extensor myopathies, anterior horn cell disorders, and muscle disorders. In severe selected cases of PD with camptocormia and dystonic camptocormia, deep brain stimulation (DBS) has been tried with v
PMID 26588625

Sunflower cataract: do not forget Wilson's disease.

Litwin T1, Langwińska-Wośko E2, Dzieżyc K1, Członkowska A3.
Practical neurology.Pract Neurol.2015 Oct;15(5):385-6. doi: 10.1136/practneurol-2014-001056. Epub 2015 May 2.
A 41-year-old man with liver cirrhosis of unknown aetiology for 6 years was admitted to our department to confirm the diagnosis of Wilson's disease. He consulted an ophthalmologist who suspected the presence of a sunflower cataract and Kayser-Fleischer ring. At admission, his liver function tests
PMID 25935927

Japanese Journal

成人発症の劇症肝炎型 Wilson 病の1例

八辻賢,徳重克年,橋本悦子 [他],山本雅一,高崎健,染谷貴志,熊田博光,白鳥敬子
肝臓 = ACTA HEPATOLOGICA JAPONICA 46(2), 71-77, 2005-02-25
症例は25歳女性. 肝機能障害を指摘された既往はない. 原因不明の劇症肝炎を発症し当院紹介受診となった. 血漿交換を繰り返すも検査所見, 肝性脳症の改善なく, 生体肝移植を施行した. 術後, 肝組織銅含有量より原疾患はWilson病であると診断した. 我々の検索しえた限り, 25歳の本例は劇症肝炎で発症したWilson病としては本邦最高齢であり, 成人の原因不明の劇症肝炎でもWilson病は鑑別診 …
NAID 10015446088

水晶体後嚢にびまん性黄褐色混濁を認めた Wilson 病の1例

小松直樹,佐々木勇二,馬場高志,井上幸次
日本眼科紀要 = Folia ophthalmologica Japonica 55(9), 723-727, 2004-09-28
NAID 10014270461

Histopathological findings of liver in Wilson's disease. A case report and discussion of hepatic fibrogenesis.:—A CASE REPORT AND DISCUSSION OF HEPATIC FIBROGENESIS—

相沢共樹,諸星利男,国村利明,丸岡悦子,布野健一,黒木辰郎,神田実喜男,友安茂,鶴岡延熹
昭和医学会雑誌 52(4), 435-440, 1992
… An 18 year old man was admitted with liver dysfunction including Kayser-Fleischer ring, hypo-ceruloplasminemia and hypo-copperemia in the serum. …
NAID 130001826840

Related Pictures

★リンクテーブル★

リンク元	「ウィルソン病」「カイザー・フライシャー角膜輪」
拡張検索	「Kayser-Fleischer rings」
関連記事	「ring」

「ウィルソン病」

Kayser-Fleischer ring
	A Kayser-Fleischer ring in a 32-year-old patient who had longstanding speech difficulties and tremor.
Classification and external resources
ICD-10	H18.0
ICD-9	371.14
DiseasesDB	30056

　　[★]

英: Wilson disease (HIM) , Wilson's disease
同: ウイルソン病、(国試)Wilson病、(国試)肝レンズ核変性症 hepatolenticular degeneration、ウェストファル-シュトリュンペル病 Westphal-Struempell disease
関: カイザー・フライシャー角膜輪 Kayser-Fleischer ring, ATP7B、銅

遺伝形式

常染色体劣性遺伝　←　構造でなく機能を提供する蛋白だからね

疫学

20歳までに発症。早ければ3歳で発症(HIM.2450)。

病因

細胞内銅輸送蛋白の遺伝子異常(ATP-7B)(13q14.3)

病態

十二指腸上部で銅の吸収　→　血行性に肝臓に輸送される　→　胆汁内への銅への排泄が障害される　→　肝細胞内銅沈着　→　肝細胞細胞障害　→　肝硬変
肝臓内でアポセルロプラスミンに銅を結合する反応の異常(HIM.2450) ＆　セルロプラスミンの合成低下(出典不明。肝機能低下？) 　→　血清セルロプラスミン低下　→　(正常では血液中に存在する銅の90%がセルロプラスミンに結合しているので)血清銅低下 (HIM.2450)
血液中に入った銅はアルブミンと結合して存在　→　結合能弱くfreeの血清銅増加

　→　血行性に各臓器に移行・沈着　→　脳、角膜、腎臓、血球に障害 (IMD)

　→　尿中への銅排泄増加