Li, Senmao, Siggel, Robert, Guo, Yongwei ORCID: 0000-0001-9195-0770, Loreck, Niklas, Rokohl, Alexander C., Kurschat, Christine and Heindl, Ludwig M. . Corneal densitometry: a potential indicator for early diagnosis of Fabry disease. Graefes Arch. Clin. Exp. Ophthalmol.. NEW YORK: SPRINGER. ISSN 1435-702X

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Abstract

Purpose To assess corneal densitometry in patients with Fabry disease (FD) and to compare corneal densitometry differences in FD patients to different corneal manifestations. Methods Ten participants (20 eyes) with FD and 10 age-matched healthy volunteers (20 eyes) were recruited. All participants were assessed by standardized ophthalmic examinations and the corneal densitometry analysis by Pentacam HR. Densitometry measurements were analyzed in standardized grayscale units. Results Seven patients developed conjunctival vessel tortuosity, cornea verticillata appeared in 6 patients, and two patients had Fabry cataract. Retinal vessel tortuosity occurred in 4 patients, and dilation of retinal vessels appeared in 3 patients, all symptoms occurred in both eyes. The first diagnosis of FD up to examination was 4.7 +/- 3.23 years, and first ERT up to examination was 2.6 +/- 2.27 years. The initial time to diagnosis was negatively related to the corneal densitometry value of the 0-2-mm (r = - 0.556, p = 0.011) and 2-6-mm (r = - 0.482, p = 0.032) zones in the posterior layer. FD group have significantly higher corneal densitometry in anterior 0-2-mm zone and 2-10-mm zone anterior and posterior layer than the control group (p <= 0.035, respectively). When divided into two groups by the existence of cornea verticillata, there was a statistically significant difference in the anterior layer, 6-10-mm zone (p = 0.031); in the central layer, 0-2 mm (p = 0.012), 2-6 mm (p = 0.001), 6-10 mm (p = 0.002), and total (p = 0.002); and in the posterior layer, 6-10 mm (p = 0.004) and total (p = 0.002). Conclusions FD patients show higher corneal densitometry, and corneal densitometry may have potential for early diagnosis and reminding progress of FD.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Li, SenmaoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Siggel, RobertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Guo, YongweiUNSPECIFIEDorcid.org/0000-0001-9195-0770UNSPECIFIED
Loreck, NiklasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rokohl, Alexander C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kurschat, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Heindl, Ludwig M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-310265
DOI: 10.1007/s00417-020-05027-6
Journal or Publication Title: Graefes Arch. Clin. Exp. Ophthalmol.
Publisher: SPRINGER
Place of Publication: NEW YORK
ISSN: 1435-702X
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
ENZYME REPLACEMENT THERAPY; OCULAR MANIFESTATIONS; MANAGEMENT; FEATURESMultiple languages
OphthalmologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/31026

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