Light and electron microscopic studies of the cornea in systemic mucopolysaccharidosis, Type I-HS

A. Kameen, I. H. Maumenee, W. R. Green

Research output: Contribution to journalArticlepeer-review

Abstract

Corneal graft specimens were obtained from two patients with Type I Hurler/Scheie systemic mucopolysaccharidosis. Light and transmission electron microscopy were performed. Numerous vacuoles containing a predominance of fibrillogranular and some multimembranous materials were noted in corneal epithelial cells, histiocytes, keratocytes, and endothelial cells. The clinical presentation and enzyme deficiency responsible for the syndrome are reviewed. Case reports and possible genetic mechanisms are presented.

Original languageEnglish (US)
Pages (from-to)107-114
Number of pages8
JournalCornea
Volume5
Issue number2
DOIs
StatePublished - Jan 1 1986

ASJC Scopus subject areas

  • Ophthalmology

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