Corneal thickness in nephropathic cystinosis

Barrett Katz, Ronald B. Melles, Jerry A. Schneider, Narsing A. Rao

Research output: Contribution to journalArticle

12 Scopus citations

Abstract

Cystinosis is a rare autosomal recessive metabolic disorder in which non-protein cystine accumulates within cellular lysosomes owing to a defect in lysosomal cystine transport. The pathognomonic ocular manifestation of cystinosis is the deposition of distinctive iridescent crystals in the cornea, not associated with any inflammatory response or recognised change in corneal function. We measured corneal thickness in nine patients with infantile nephropathic cystinosis. We also studied a corneal button from one of these patients who underwent corneal transplantation. All nine patients had increased corneal thickness in comparison with an age matched control population. Electron microscopy analysis of the cystinotic button revealed structural changes of both epithelium and endothelium layers. Increased corneal thickness in patients with nephropathic cystinosis may reflect subclinical corneal oedema.

Original languageEnglish (US)
Pages (from-to)665-668
Number of pages4
JournalBritish Journal of Ophthalmology
Volume73
Issue number8
DOIs
StatePublished - Jan 1 1989

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ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this

Katz, B., Melles, R. B., Schneider, J. A., & Rao, N. A. (1989). Corneal thickness in nephropathic cystinosis. British Journal of Ophthalmology, 73(8), 665-668. https://doi.org/10.1136/bjo.73.8.665