Xeroderma pigmentosum and keratoconus.

Documenta ophthalmologica. Advances in ophthalmology ·Vol. 64 ·No. 1 ·1986-12-30 ·Pages 97-103

Blanksma LJ, Donders PC, van Voorst Vader PC

Abstract

Two XP patients are presented. A corneal perforation in the left eye of the first patient necessitated an at-random transplantation à chaud. The graft was remarkably well tolerated, which is possibly explained by UV-light-induced suppression of the cellular immune response in the patient. The right eye of this patient and both eyes of the second patient had a keratoconus. Keratoconus in XP may be the result of disturbances in the cell differentiation and the function of epithelial cells and keratocytes, due to UV-light-induced deficient DNA repair synthesis.

MeSH Terms

Adult Child Corneal Diseases/pathology,surgery Corneal Transplantation Humans Keratoconus/etiology,pathology,surgery Male Xeroderma Pigmentosum/complications,pathology

Authors & Affiliations

3 authors, click to expand affiliations / ORCID

Blanksma L J

Donders P C

van Voorst Vader P C

References (14)

14 references, click to expand

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Article Info

Journal

Documenta ophthalmologica. Advances in ophthalmology

Abbr.

Doc Ophthalmol

ISSN

0012-4486

DOI

10.1007/BF00166690

Published

1986-12-30

Pages

97-103

Language

English

Region

Netherlands

NLM ID

0370667

Subset

External Links

PubMed source DOI: 10.1007/BF00166690 View journal details

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