A Case of Albers-Schonberg's Syndrome. |
Jin Su Choi, Sang Min Kim |
Department of Ophthalmology, Kyung Hee University, School of Medicine, Seoul, Korea. |
Albers - Schonberg 씨 증후군 1예 |
김상민(Sang Min Kim),최진수(Jin Soo Choi) |
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Abstract |
Albers-Schonberg's syndrome is rare familial disease. This syndrome is characterized by increasing roentgenographic density and bony fragility of all skeleton, optic nerve atrophy, nystagmus, anemia, because of abnormal bone growing and decrease hematopoietic system. The conservative theraphy is performed mainly. Anemia is improved by steroid theraphy, splenectomy, transfusion. Early optic canal decompression help to prevent optic nerve atrophy. The authors persented a 17 months old female with Albers-Schonberg's syndrome(malignant type), who was admitted because of epistaxis and growth retardation. |
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