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KMID : 0378019730160010167
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New Medical Journal
1973 Volume.16 No. 1 p.167 ~ p.171
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A case of Letterer-Siwe´s Disease
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鄭東星/Chung, Dong Sung
金亨晳/邊永壽/李英淳/Kim, Hyung Seok/Byun, Young Soo/Lee, Young Soon
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Abstract
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Letterer-Siwe´s disease is originated from some reticuloendothelial system with Hand Schuller-Chri￢stian´s disease and eosinophilic granuloma, the causes are not known yet exactly and the clinical manifestations are various but the pathological changes are so similar in each other that are very interesting in orthopedic fields. A case of Letterer-Siwe´s disease is experienced and reported. A 15 months old Korean girl was complained tenderness on thoracolumbar vertebra with progressive kyphotic change following high fever and diarrhea. Two to three days prior to admission, purpura was begining to be appeared on her lower abdomen. The roentgenological study were revealed clear round, radiolucent bony destructions in the skull and marked similar osteoporotic or radiolucent changes in postero-lateral aspect of the right 8th rib with expansion of the cortex but lung was clear. The vertebral bodies of the 10th, 12th thoracic and 1st lumbar were flattened, and various sized and irregular radiolucent cystic lesions were noted in the both iliac bones and medullary region of the right upper femur.
The biopsy was showed in microscopically well differentiated, proliferated and many 15-25i sized histiocytes, eosinophilic rich cytoplasm without lipid vacuoles. Consequently the diagnosis of Letterer￢Siwe´s disease was conformed by microscopic examination.
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