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In vivo corneal confocal microscopy of sub-basal nerve plexus in patients affected by Fabry disease

Poster Details


First Author: L.Primavera ITALY

Co Author(s): M. Fresina   P. Versura   M. Pellegrini   E. Campos           

Abstract Details

Purpose:

To investigate corneal sub-basal nerve plexus in patients with Fabry disease by laser scanning corneal confocal microscopy (CCM).

Setting:

All patients were evaluated at the Sant’Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy between May 2018 and September 2018.

Methods:

Twenty-five patients affected by Fabry disease and twenty-five, age-matched, healthy volunteers underwent examination with CCM. Three high quality images of the sub-basal nerve plexus of the cornea were used from each patient and control subject, with each of these analyzed by automated CCMetrics software (University of Manchester UK) and the average of these results considered. The parameters quantified by the software were: corneal nerve fiber density (CNFD), corneal nerve branch density (CNBD), corneal nerve fiber lenght (CNFL) and corneal total branch density (CTBD).

Results:

Values of CNFD (21.8 ± 7.5 vs 28.1 ± 5.5 fibers/mm2, P = 0.003), CNFL (12.8 ± 3.8 vs 15.7 ± 3.1mm/mm2, P = 0.004) and CTBD (43.6 ± 25.0 vs 57.0 ± 28.5 fibers/mm2, P = 0.046) were significantly reduced in patients compared to control subjects. Values of CNFD (27.4 ± 7.1 vs 20.1 ± 6.9 fibers/mm2; P = 0.034) and CNFL (15.6 ± 4.7 vs 12.0 ± 3.2 mm/mm2; P = 0.038) were significantly reduced in patient receiving enzyme replacement therapy (ERT) compared to patients not receiving ERT.

Conclusions:

CCM is a useful means to detect early small fibers neuropathy in patients affected by Fabry disease.

Financial Disclosure:

None

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