J Korean Ophthalmol Soc.  1982 Sep;23(3):847-850.

A Case of Albers-Schonberg's Syndrome

Affiliations
  • 1Department of Ophthalmology, Kyung Hee University, School of Medicine, Seoul, Korea.

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.


MeSH Terms

Anemia
Atrophy
Decompression
Epistaxis
Female
Hematopoietic System
Humans
Infant
Optic Nerve
Skeleton
Splenectomy
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