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NR. 2/2009
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Choroba Fabry’ego –
zmiany w morfologii rogówki obserwowane za pomocą mikroskopii
konfokalnej
Microstructural Changes in Cornea of Patients with Fabry’s
Disease
Urszula Kołodziejska1,
Mariusz Kłopotowski2, Anna M. Ambroziak1,
Lidia Chojnowska2, Jacek P. Szaflik1
1 Katedra i Klinika Okulistyki II Wydziału
Lekarskiego Warszawskiego Uniwersytetu Medycznego
Samodzielny Publiczny Kliniczny Szpital Okulistyczny w Warszawie
Kierownik: prof. dr hab. n. med. Jerzy Szaflik
2 I Klinika Choroby Wieńcowej Instytutu Kardiologii
im. Prymasa Tysiąclecia Stefana Kardynała Wyszyńskiego
w Warszawie
Kierownik: prof. dr hab. n. med. Witold Rużyłło |
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| Summary: |
Purpose: To
identify and describe the microstructural changes in
cornea of patients with Fabry disease.
Method: Four patients (eight eyes) suffering from
Fabry disease underwent in vivo confocal microscopy of
the cornea by both scanning slit and laser scanning
confocal microscopy systems.
Bright intracellular inclusions were visualized in basal
epithelium and in some cases deposits were observed in
Bowman’s layer. Interestingly, the changes were also
found in the eyes in which no changes were visible by
slit lamp examination. The images obtained by both
confocal microscopes were comparable, though the laser
system provided better contrast of the images of the
diseased corneal layers.
Conclusions: The microstructural alterations of
the cornea in Fabry disease can be visualized by means
of in vivo confocal microscopy also in patients in who
neither the typical cornea verticillata lines nor other
ocular changes are present in slit lamp biomicroscopy
and fundus examination. This feature may possibly
facilitate the early clinical diagnosis in doubtful
cases before molecular analyses. |
| Key words: |
Fabry’s disease, cornea
verticillata, confocal microscopy. |
| Słowa kluczowe: |
choroba Fabry’ego,
keratopatia wirowata, mikroskopia konfokalna. |
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