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NR 1-3/2005
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Zanik girlandowaty
naczyniówki i siatkówki u dzieci
Gyrate atrophy of the
choroid and retina in children
Bronisława Koraszewska-Matuszewska,
Małgorzata Korzekwa, Elżbieta Samochowiec-Donocik
Z Katedry i Kliniki Okulistyki Dziecięcej ¦l±skiej Akademii
Medycznej w Katowicach
Kierownik: prof. dr hab. n. med. Bronisława
Koraszewska-Matuszewska |
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| Summary: |
The aim: the
history and clinical findings of 3.5 and 5.5-years old
siblings with gyrate atrophy (GA) of the choroid and
retina are presented.
Patients and method: Siblings: a girl at the age
3.5 years and her brother 5.5 years were examined.
Clinical and biochemical findings were performed.
Results: The best corrected visual acuity of both
girl and boy was below normal values. Myopia of middle
degree and myopic astigmatism was presented in both eyes
of siblings. On fundus examination sharply defined
bizarre shaped atrophic areas of peripheral choroid and
retina were seen in both eyes of children. Fluorescein
angiography showed hyperfluorescence in the area of
gyrate atrophy.
The boy’s plasma ornithine level was increased to
974.950 umol/L and the girl’s to 1007.188 umol/L. The
concentration of ornithine, lysine and arginine in the
urea of these patients was high.
Conclusion: Early cilnical and biochemical
diagnosis of gyrate atrophy of the choroid and retina in
children is very important, because low protein diet
with elimination of arginine can reduce the progression
of this severe disease. |
| Słowa kluczowe: |
zanik girlandowaty
naczyniówki i siatkówki, hiperornitynemia, dzieci,
rodzeństwo. |
| Key words: |
gyrate atrophy of choroid,
retina, hyperornithinemia, children, siblings. |
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