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다발성 골단 성장 부전증 Multiple Epiphyseal dysplasis

대한정형외과학회지 1972년 7권 3호 p.347 ~ 350
김창수, 이기린,
소속 상세정보
김창수 (  ) 
가톨릭의과대학 정형외과학교실

이기린 (  ) 
가톨릭의과대학 정형외과학교실

Abstract


Two cases of multiple epiphyseal dysplasia in a.3 years old boy and :a 10 years. old girl are reported.
Since Fairbank´s description Of the disease in 1935, it has been a well recognized entity which is a rare congenital developmental error characterized by mottling or irregularity in density and outline of several of the developing epiphyses, and dwarfism.
Many reports indicate a definite hereditary and familiar distribution, particularly one series in which there were 10 cases in a single family, But in this report parent and siblings, all of whom were examined clinically, and roentgenographically showed no demonstrable stigmata of any growth disturbance.
As the child develops, a subnormal growth rate becomes more obvious. The vertebrae are usually not affected so that dwarfing is confined to the extremity.
The process rarely comes to attention of the physician until the child begin to walk, and then it is noted that one has a waddling gait and short stubby digits of the hands and feet. Intelligence is unaffected. Prognosis as too life expectancy and range of activity is good.

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