제 II형 골형성 부전증(2례 부검보고)
Osteogenesis Imperfecta Type II (Two Autopsy Cases)

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김우호; 이병두; 지제근
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서울대학교 의과대학
Seoul J Med 1982;23(4):501-508
Osteogenesis imperfecta is one of common hereditary
anomalies, but its spectrum appears quite broad.
There are many clinical and pathological features
which suggest heterogeneity of this disease.
We report two cases of osteogenesis imperfecta
that we thought best fit for type II. Both cases
were born after gestational periods of 30 and 40
weeks, and died immediately after birth. Both clinical
and radiological findings were compatible with those
of osteogenesis imperfecta in both cases.
Major histologic findings were alteration of both
endochondral and intramembranous ossification. And
interestingly osteoclasts were abundant in the surface
01 lamellar bone and medul1ary spaces as wel1 in
both cases.
We reviewed the literatures on histologic features
and discussed on recently proposed classification of
osteogenesis imperfecta.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 23 No.4 (1982)
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