A Case of Albers-Schonberg's Syndrome.
- Author:
Jin Su CHOI
1
;
Sang Min KIM
Author Information
1. Department of Ophthalmology, Kyung Hee University, School of Medicine, Seoul, Korea.
- Publication Type:Case Report
- MeSH:
Anemia;
Atrophy;
Decompression;
Epistaxis;
Female;
Hematopoietic System;
Humans;
Infant;
Optic Nerve;
Skeleton;
Splenectomy
- From:Journal of the Korean Ophthalmological Society
1982;23(3):847-850
- CountryRepublic of Korea
- Language:Korean
-
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.
