KMID : 0358419940370050991
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Korean Journal of Obstetrics and Gynecology 1994 Volume.37 No. 5 p.991 ~ p.997
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A Case of Perinatal Lethal Osteogenesis Imperfecta
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Ȳ¹ÌÁ¤
¹Ú½Å±Ù/À̵¿Áø/Á¶»ó·¡/±è¿Ï¿µ/ÀÌÁ¾ÇÐ/±èÁ¾È/¹é¿ø¿µ
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Abstract
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Osteogenesis imperfecta is a heterogeneous group of inherited disoders characterized by bone fragility than is accompanied by other evidence of malfunction of connective tissue including abnormalities in teeth (dentinigenesis imperfecta), hearing
loss,
alterations in scleral hue, and evidence of soft tissue dysplasia One of these, the perinatal from on osteogenesis imperfecta type II is rare disorder characterized by small thoracic cavity, beaded rib, and hort, bowed limbs.
We recently experienced a case of osteogenesis imperfecta type II diagnosed in utero by ultrasonogram and confirmed by postnatal radiograph and autopsy.
We present the case with a brief review of the literatures.
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