All issues > Volume 31(8); 1988

Original Article

J Korean Pediatr Soc. 1988;31(8):1085-1090. Published online August 31, 1988.

Two Cases of Osteogenesis Imperfecta Congenita.

Soon Soen Lim¹, Byeung Ho Lee¹, Young Ha Kim¹, Moon Ki Joe¹

¹Department of Pediatrics, St. Columban fs Hospital, Mokpo, Korea

Received: October 30, 1987;  Accepted: March 16, 1988.

Abstract

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.

Keywords :Osteogenesis Imperfecta Congenita.