Journal of the Korean Pediatric Society 1988;31(8):1085-1090.
Published online August 31, 1988.
Two Cases of Osteogenesis Imperfecta Congenita.
Soon Soen Lim, Byeung Ho Lee, Young Ha Kim, Moon Ki Joe
Department of Pediatrics, St. Columban fs Hospital, Mokpo, Korea
선천성 골형성부전증 2례
임순선, 이병호, 김영하, 조문기
성골롬반 병원 소아과
Received: 30 October 1987   • Accepted: 16 March 1988
Osteogenesis imperfecta is an inherited disorder of connective tissue that affects the skeleton, ligament, skin, sclerae and dentin. It is thought to be characterized by the abnormal maturation of collagen in both mineralized and nonmineralized tissues. The three major clinical criteria are osteoporosis with abnormal fragility of the skeleton, blue sclerae and dentinogenesis imperfecta. The presence of two of these abnormalities confirms the diagnosis. We have experienced two cases of osteogenesis imperfecta congenita. The diagnosis was established by clinical and radiographic findings. A brief review of literature was made.
Key Words: Osteogenesis Imperfecta Congenita.

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