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

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

doi:10.1097/00003226-198605020-00006

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

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

University Administration

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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 language	English (US)
Pages (from-to)	107-114
Number of pages	8
Journal	Cornea
Volume	5
Issue number	2
DOIs	https://doi.org/10.1097/00003226-198605020-00006
State	Published - Jan 1 1986

ASJC Scopus subject areas

Ophthalmology

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10.1097/00003226-198605020-00006

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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.",

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T1 - Light and electron microscopic studies of the cornea in systemic mucopolysaccharidosis, Type I-HS

AU - Kameen, A.

AU - Maumenee, I. H.

AU - Green, W. R.

PY - 1986/1/1

Y1 - 1986/1/1

N2 - 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.

AB - 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.

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EP - 114

JO - Cornea

JF - Cornea

IS - 2

ER -

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

Abstract

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