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釜山에서 發見된 Progressive Muscular Dystrophy의 一家系

A Clan of Progressive Muscular Dystrophy Found in Pusan

최신의학 1959년 2권 7호 p.71 ~ 72
한기수,
소속 상세정보
한기수 (  ) - 부산의대 신경정신과

Abstract


The author found a clan of progressive muscular dystrophy, facioscapulohumeral type, in pusan. All of the patients reveal typical signs and progression of facioscapulohumeral type of progressive muscular dystrophy which are described in the text book of neurology. They begin to have muscular dystrophy in the facial muscles in early childhood and then in the scapular region, back an3 upperarms. But forearms are not involved.
The families of the clan have been trying to keep their illness secret with rather amazing success. The first ´. patient seen in the figure lived at Ungchun, Kyungnam. He was an only child. No one of the clan knows whether there was any patient before this first patient. They do only vaguely KnaN that the firat patient´s relatives may be living at Masan area, but do not exactly know their names and places.
The figure shows that, with one exception all patients have a child or children of muscular dystrophy as well as normal ones. The 39 year old male patient seen in the 3rd from the left in the 3rd row has 7 children, one boy 6 daughters, but none of them has developed dystrophy yet.
This clan remind us of Huntington´s chorea. According to Davenport and Muncy Huntington´s chorea in the United States can be traced back to the three brothers why imigrated to the United States in 17th Centry.
It will be very interesting and important from the academic as well as social view points to observe the future E development of this clan.

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