Wilson disease

出典: meddic

ウィルソン病

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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/06/19 11:33:33」(JST)

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英文文献

  • Reactive Arrays of Colorimetric Sensors for Metabolite and Steroid Identification.
  • Batres G, Jones T, Johnke H, Wilson M, Holmes AE, Sikich S.
  • Journal of sensor technology.J Sens Technol.2014 Dec 31;4(1). pii: 43398.
  • The work described herein examines a rapid mix-and-measure method called DETECHIP suitable for screening of steroids and metabolites. The addition of steroids and metabolites to reactive arrays of colorimetric sensors generated characteristic color "fingerprints" that were used to identify the analy
  • PMID 25019034
  • The nasal and sinus microbiome in health and disease.
  • Wilson MT1, Hamilos DL.
  • Current allergy and asthma reports.Curr Allergy Asthma Rep.2014 Dec;14(12):485. doi: 10.1007/s11882-014-0485-x.
  • There has been great interest in unraveling the complex inter-relationships between microbes and humans as they relate to human health and disease. This review will focus on recent advances in the appreciation and understanding of these relationships in terms of the upper respiratory tract, specific
  • PMID 25342392
  • Anaemia from a patient perspective in inflammatory bowel disease: results from the European Federation of Crohn's and Ulcerative Colitis Association's online survey.
  • Danese S1, Hoffman C, Vel S, Greco M, Szabo H, Wilson B, Avedano L.
  • European journal of gastroenterology & hepatology.Eur J Gastroenterol Hepatol.2014 Dec;26(12):1385-91. doi: 10.1097/MEG.0000000000000200.
  • OBJECTIVE: Anaemia is frequently seen in inflammatory bowel disease (IBD) and needs appropriate treatment. This online questionnaire investigated the patients' perceptions of anaemia: symptoms, prevalence, treatment and impact on quality of life.METHODS: Patients participated in this survey in 2012.
  • PMID 25264983

和文文献

  • 肝疾患 (特集 生活習慣病と認知機能 : 認知症の予防を見据えた生活習慣病の治療を目指して) -- (予防と治療)
  • 銅代謝とウイルソン病 (特集 金属代謝と肝疾患)
  • Biochemical Staging of the Chronic Hepatic Lesions of Wilson Disease
  • GOTO HIDEMI,HAYASHI HISAO,MIZUTANI NAOKI,KUMADA TAKASHI,TOYODA HIDENORI,YANO MOTOYOSHI,WAKUSAWA SHINYA,UEYAMA JUN,TATSUMI YASUAKI,HATTORI AI,HAYASHI KAZUHIKO,KATANO YOSHIAKI
  • Nagoya Journal of Medical Science 76(1-2), 139-148, 2014
  • NAID 120005385258

関連リンク

Wilson Disease Definition Wilson disease, or WD, is a rare inherited disorder that causes excess copper to accumulate in the body. It is also known as hepatolenticular degeneration. Steadily increasing amounts of copper circulating ...
Wilson disease is a disorder of copper metabolism that can present with hepatic, neurologic, or psychiatric disturbances, or a combination of these, in individuals ranging from age three years to over 50 years; symptoms vary among ...

関連画像

homesick student Wilson's disease (hepatolenticularFigure 1Kayser-Fleischer ring.jpgWilson's Diseasewilson disease


★リンクテーブル★
リンク元ウィルソン病」「ウェストファル-シュトリュンペル病
拡張検索Wilson disease gene」「medicine for Wilson disease」「anti-Wilson disease
関連記事Wilson」「disease」「diseased

ウィルソン病」

  [★]

Wilson disease (HIM) , Wilson's disease
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
カイザー・フライシャー角膜輪 Kayser-Fleischer ring, ATP7B

遺伝形式

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

症候

治療

  • 食事療法:銅制限食(貝、甲殻類、レバー、ナッツ類、チョコレート、キノコ類、豆類は銅を多く含むので避ける)
  • 薬物療法:銅キレート薬((D-ペニシラミン)の投与。トリエンチン

参考

  • 1. WILSON DISEASE - OMIM
[display]http://omim.org/entry/277900
  • 2. [charged] Pathogenesis and clinical manifestations of Wilson disease - uptodate [1]
  • 3. [charged] Treatment of Wilson disease - uptodate [2]

国試



ウェストファル-シュトリュンペル病」

  [★]

Westphal-Struempell disease
ウィルソン病 Wilson disease


Wilson disease gene」

  [★] ウィルソン病遺伝子


medicine for Wilson disease」

  [★] ウィルソン病治療薬


anti-Wilson disease」

  [★] ウィルソン病治療薬


Wilson」

  [★] ウィルソン

WordNet   license wordnet

「Scottish ornithologist in the United States (1766-1813)」
Alexander Wilson

WordNet   license wordnet

「Scottish physicist who invented the cloud chamber (1869-1959)」
Charles Thomson Rees Wilson

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「United States entomologist who has generalized from social insects to other animals including humans (born in 1929)」
E. O. Wilson, Edward Osborne Wilson

WordNet   license wordnet

「United States literary critic (1895-1972)」
Edmund Wilson

WordNet   license wordnet

「author of the first novel by an African American that was published in the United States (1808-1870)」
Harriet Wilson

WordNet   license wordnet

「American Revolutionary leader who was one of the signers of the Declaration of Independence (1742-1798)」
James Wilson

WordNet   license wordnet

「Canadian geophysicist who was a pioneer in the study of plate tectonics (1908-1993)」
John Tuzo Wilson

WordNet   license wordnet

「United States physicist honored for his work on cosmic microwave radiation (born in 1918)」
Robert Woodrow Wilson

WordNet   license wordnet

「English writer of novels and short stories (1913-1991)」
Sir Angus Wilson, Angus Frank Johnstone Wilson

WordNet   license wordnet

「28th President of the United States; led the United States in World War I and secured the formation of the League of Nations (1856-1924)」
Woodrow Wilson, Thomas Woodrow Wilson, President Wilson

WordNet   license wordnet

「a peak in the San Juan mountains of Colorado (14,246 feet high)」
Mount Wilson

PrepTutorEJDIC   license prepejdic

「ウィルソン(Thomas Woodrow Wilson;1856‐1924;米国第28代大統領1913‐21)」


disease」

  [★]

  • n.
疾患illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)
特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)
  • something that is very wrong with people's attitudes, way of life or with society.
ailailmentdisease entitydisorderillillnessmaladysicksickness
  • 注意
diseaseillnessdisorder


WordNet   license wordnet

「an impairment of health or a condition of abnormal functioning」

PrepTutorEJDIC   license prepejdic

「(体の)『病気』,疾患 / (精神・道徳などの)病気,病弊」

PrepTutorEJDIC   license prepejdic

「女性の話術芸人 =diseur」


diseased」

  [★]

WordNet   license wordnet

「caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes"」
morbid, pathologic, pathological

PrepTutorEJDIC   license prepejdic

「病気にかかった / 病的な,不健全な(morbid)」




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