| 특징적인 임상소견을 보인 뇌회형맥락망막위축 1예 |
|
김진영, 이하범, 박성표 |
|
Department of Ophthalmology, Kangdong Sacred Heart Hospital, Hallym Medical University, Seoul, Korea |
| Gyrate Atrophy of Choroids and Retina Showing Typical Clinical Characteristics |
|
Jin Young Kim, Ha Bum Lee, Sung Pyo Park |
|
|
| Abstract |
Purpose
We report a rare case of a Gyrate Atrophy, in which the patient's level of serum ornithine was high with typical fundus findings. Methods: A 24-years old male patient presented with nyctalopia. We examined the Fundus examination, Fluorescein Angiography (FA), Visual field & electroretinogram. Amino acid analysis was used to measure the level of serum and urine amino acids. Results: The exact mechanism of chorioretinal atrophy in hyper-ornithinemia is not known. Fundus examination of both eyes revealed sharply demaracated areas of choroids and retinal atrophy in gyrate shape and involving the midperiphery with the macula not affected. Amino Acid analysis revealed a high serum and urine ornithine level (623Ռmol/L, 14,666Ռmol/g) with the normal range being 48∼195Ռmol/L, 20∼80Ռmol/g. (Korean J Optom Vis Sci 2008;7(1):23-29) |
| Key Words:
Gyrate atrophy, Ornithine, Ornithine aminotransferase |
|
PDF Links
Download Citation
