WordNet

it would be sensible; "youd best stay at home"
the supreme effort one can make; "they did their best"
the person who is most outstanding or excellent; someone who tops all others; "he could beat the best of them" (同)topper
(superlative of `good'
in a most excellent way or manner; "he played best after a couple of martinis"
an impairment of health or a condition of abnormal functioning
caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological
Canadian physiologist (born in the United States) who assisted F. G. Banting in research leading to the discovery of insulin (1899-1978) (同)C. H. Best, Charles Herbert Best

PrepTutorEJDIC

(『good』の最上級;比較級は『better』)『いちばんよい』,最もよい,最良の / (『well』の最上級;比較級は『better』)『健康状態が最良の』(healthiest) / 最大の,大部分の / 《話》(反語的に)きわめてひどい,徹底した / (『well』の最上級;比較級は『better』)『いちばんよく』,最もよく,最も / 《通例過去分詞と結合して形容詞を作って》最もよく / 『最上』,最善,最良;最もすぐれた人々(物) / (…の)『最もよいもの』(部分)《+『of』+『名』》
(体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
女性の話術芸人 =diseur
病気にかかった / 病的な,不健全な(morbid)
berylliumの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/26 23:12:36」(JST)

wiki en

Vitelliform macular dystrophy or vitelliform dystrophy is a genetic eye disorder that can cause progressive vision loss. This disorder affects the retina, specifically cells in a small area near the center of the retina called the macula. The macula is responsible for sharp central vision, which is needed for detailed tasks such as reading, driving, and recognizing faces.

Diagnosis

Vitelliform macular dystrophy causes a fatty yellow pigment (lipofuscin) to build up in cells underlying the macula. Over time, the abnormal accumulation of this substance can damage cells that are critical for clear central vision. As a result, people with this disorder often lose their central vision and may experience blurry or distorted vision. Vitelliform macular dystrophy does not affect side (peripheral) vision or the ability to see at night.

Researchers have described two forms of vitelliform macular dystrophy with similar features. The early-onset form (known as Best disease) usually appears in childhood; however, the onset of symptoms and the severity of vision loss vary widely. The adult-onset form begins later, usually in middle age, and tends to cause relatively mild vision loss. The two forms of vitelliform macular dystrophy each have characteristic changes in the macula that can be detected during an eye examination.

Pathophysiology

Mutations in the RDS and VMD2 genes cause vitelliform macular dystrophy. Mutations in the VMD2 gene are responsible for Best disease. Changes in either the VMD2 or RDS gene can cause the adult-onset form of vitelliform macular dystrophy; however, fewer than a quarter of cases result from mutations in these two genes. In most cases, the cause of the adult-onset form is unknown.

The VMD2 gene provides instructions for making a protein called bestrophin. Although its exact function is uncertain, this protein likely acts as a channel that controls the movement of negatively charged chlorine atoms (chloride ions) into or out of cells in the retina. Mutations in the VMD2 gene probably lead to the production of an abnormally shaped channel that cannot regulate the flow of chloride. Researchers have not determined how these malfunctioning channels are related to the buildup of lipofuscin in the macula and progressive vision loss.

The RDS gene provides instructions for making a protein called peripherin. This protein is essential for the normal function of light-sensing (photoreceptor) cells in the retina. Mutations in the RDS gene disrupt the structures in these cells that contain light-sensing pigments, leading to vision loss. It is unclear why RDS mutations affect only central vision in people with adult-onset vitelliform macular dystrophy.

Inheritance

Best disease, the early-onset form of vitelliform macular dystrophy, has an autosomal dominant pattern of inheritance.

Best disease is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person has one parent with the condition.

The inheritance pattern of adult-onset vitelliform macular dystrophy is uncertain. Some studies have suggested that it may be inherited in an autosomal dominant pattern. Many affected people, however, have no history of the disorder in their family and only a small number of affected families have been reported.

External links

VIDEO - Why Test for Best? Dr. Eric Brinton of the University of Wisconsin-Madison School of Medicine and Public Health.
www.bestdisease.net
Vitelliform macular dystrophy at NLM Genetics Home Reference
Best's disease - eMedicine.com
GeneReviews/NCBI/NIH/UW entry on Best Vitelliform Macular Dystrophy

UpToDate Contents

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Milk, milk products, and disease free health: an updated overview.

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Japanese Journal

器質性視覚障害と心因性視覚障害を合併した1例

大平明彦
東京女子医科大学雑誌 82(E1), E178-E182, 2012-01-31
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水野大
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Examination of validity of fall risk assessment items for screening high fall risk elderly among the healthy community-dwelling Japanese population

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Archives of Gerontology and Geriatrics 53(1), e41-e45, 2011-07
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NAID 120002661939

Related Pictures

★リンクテーブル★

リンク元	「黄斑疾患」「網膜変性」「ベスト病」
関連記事	「disease」「best」「BEST」「Be」

「黄斑疾患」

Vitelliform macular dystrophy
Classification and external resources
OMIM	608161 153700
DiseasesDB	34190 31278

　　[★]

SOP.147

中心性漿液性脈絡網膜症
嚢胞様黄斑浮腫
加齢黄斑変性：網膜上皮の萎縮をきたすdry typeと出血・浮腫・網膜色素上皮剥離を伴うwet typeがある。黄斑下に血管線維性増殖組織(脈絡膜新生血管網)がみられ、浮腫の周囲には硬性白斑を伴う。脈絡膜新生血管に対し、光感受性物質とレーザーを組み合わせた光力学療法や抗VEGF抗体(ベバシズマブ)による治療がある。
黄斑円孔：網膜硝子体界面の加齢変化のため、中心窩を中心に放射状に網膜が接線方向に牽引され、中心窩が挙上され嚢腫ができ前壁が消失して中心窩に円孔が形成される。治療は硝子体手術による円孔の閉鎖である。
近視性黄斑萎縮
網膜硝子体界面黄斑変性症
薬物毒性黄斑変性症
遺伝性黄斑変性症
黄斑ジストロフィ