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Best vitelliform macular dystrophy

Other Names for this Disease
  • Best disease
  • Best macular dystrophy
  • Macular degeneration, polymorphic vitelline
  • Vitelliform macular dystrophy type 2
  • VMD2
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Best vitelliform macular dystrophy is a genetic form of macular degeneration that usually begins in childhood or adolescence and slowly progresses to affect central vision.[1][2][3] Individuals typically retain normal peripheral vision and dark adaptation.[2][3] The age of onset and severity of vision loss are highly variable.[1][2][3] Best vitelliform macular dystrophy is an autosomal dominant condition which is associated with an abnormality in the BEST1 (VMD2) gene.[1][2] There is no specific treatment for vitelliform macular dystrophy at this time.[4] Low vision aids may be helpful.[3]
Last updated: 9/10/2010


  1. Best Vitelliform Macular Dystrophy. National Organization for Rare Diseases (NORD). 2007; Accessed 7/28/2009.
  2. Vitelliform macular dystrophy. Genetics Home Reference (GHR). 2008; Accessed 7/28/2009.
  3. MacDonald IM, Lee T. Best Vitelliform Macular Dystrophy. GeneReviews. 2009; Accessed 7/28/2009.
  4. Besch D, Zrenner E. Best disease. Orphanet. 2005; Accessed 7/28/2009.
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  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
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