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進行性 筋萎縮症 一例

A case with Progressive Muscular Dystrophy

소아과 1964년 7권 4호 p.39 ~ 41
李尙柱, 李丙玧, 李庸一,
소속 상세정보
李尙柱 (  ) - 韓一病院 小兒科
李丙玧 (  ) - 韓一病院 小兒科
李庸一 (  ) - 韓一病院 小兒科

Abstract


A twelve years and five months old boy was admitted to the Hanil Hospital on August 17, 1964 because of limping and muscular weakness.
He was normal full term spontaneous delivery. No family incidence of this disease was detectable. Father was afflicted with gonorrhea or other venereal disease several times and mother has contracted pulmonary tuberculosis four years ago.
The patient was somewhat undernourished and underdeveloped. He showed the sign of Gowers. who described the movements necessary for persons with this disease to rise from a sitting position on the floor to a standing position.
His tendon relexes were markedly hypoactive especially the knee jerks were absent.
Laboratory examination revealed that the increasein 24 hours urinary creatine excretion and decreased excretion of creatinine.
Muscle biopsy from the left calf revealed skeletal muscle showing groups of muscle bundles showed irregular bundles with atrophic degenerated sarcoplasm.
On roentgenogram dorsal spine was scohot;. to the
right side.

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