.: OKULISTYKA :.

Wydanie 2/2009
str. 101

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

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.

Słowa kluczowe: choroba Fabry'ego, keratopatia wirowata, mikroskopia konfokalna.

Keywords: Fabry's disease, cornea verticillata, confocal microscopy.

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