Dense peripheral corneal clouding in Scheie syndrome

C. G. Summers; Chester B Whitley; E. J. Holland; R. L. Purple; W. Krivit

doi:10.1097/00003226-199405000-00016

Dense peripheral corneal clouding in Scheie syndrome

C. G. Summers, Chester B Whitley, E. J. Holland, R. L. Purple, W. Krivit

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Abstract

A 28-year-old woman with Scheie syndrome (MPS I-S) presented with the unusual feature of extremely dense peripheral corneal clouding, allowing maintenance of good central visual acuity. Characteristic systemic features, an abnormal electroretinogram result, and absent α-L-iduronidase activity confirmed the diagnosis despite the unusual corneal pattern of clouding.

Original language	English (US)
Pages (from-to)	277-279
Number of pages	3
Journal	Cornea
Volume	13
Issue number	3
DOIs	https://doi.org/10.1097/00003226-199405000-00016
State	Published - Jan 1 1994

Keywords

Corneal clouding
Mucopolysaccharidosis
Retinal degeneration
Scheie syndrome

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title = "Dense peripheral corneal clouding in Scheie syndrome",

abstract = "A 28-year-old woman with Scheie syndrome (MPS I-S) presented with the unusual feature of extremely dense peripheral corneal clouding, allowing maintenance of good central visual acuity. Characteristic systemic features, an abnormal electroretinogram result, and absent α-L-iduronidase activity confirmed the diagnosis despite the unusual corneal pattern of clouding.",

keywords = "Corneal clouding, Mucopolysaccharidosis, Retinal degeneration, Scheie syndrome",

author = "Summers, {C. G.} and Whitley, {Chester B} and Holland, {E. J.} and Purple, {R. L.} and W. Krivit",

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AU - Whitley, Chester B

AU - Holland, E. J.

AU - Purple, R. L.

AU - Krivit, W.

PY - 1994/1/1

Y1 - 1994/1/1

N2 - A 28-year-old woman with Scheie syndrome (MPS I-S) presented with the unusual feature of extremely dense peripheral corneal clouding, allowing maintenance of good central visual acuity. Characteristic systemic features, an abnormal electroretinogram result, and absent α-L-iduronidase activity confirmed the diagnosis despite the unusual corneal pattern of clouding.

AB - A 28-year-old woman with Scheie syndrome (MPS I-S) presented with the unusual feature of extremely dense peripheral corneal clouding, allowing maintenance of good central visual acuity. Characteristic systemic features, an abnormal electroretinogram result, and absent α-L-iduronidase activity confirmed the diagnosis despite the unusual corneal pattern of clouding.

KW - Corneal clouding

KW - Mucopolysaccharidosis

KW - Retinal degeneration

KW - Scheie syndrome

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ER -

Dense peripheral corneal clouding in Scheie syndrome

Abstract

Keywords

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