Ultrastructural and histochemical studies of a newly recognized form of systemic mucopolysaccharidosis (Maroteaux-Lamy syndrome, mild phenotype)

Harry A. Quigley; Kenneth R. Kenyon

doi:10.1016/0002-9394(74)90383-3

Ultrastructural and histochemical studies of a newly recognized form of systemic mucopolysaccharidosis (Maroteaux-Lamy syndrome, mild phenotype)

Harry A. Quigley, Kenneth R. Kenyon

School of Medicine

Research output: Contribution to journal › Article › peer-review

17 Scopus citations

Abstract

A 31-year old man with corneal clouding and stiff joints, who may represent a newly recognized genetic form of systemic mucopolysaccharidosis type VI (MaroteauxLamy syndrome), was studied by histochemistry and electron microscopy. Connective tissue cells of cornea, conjunctiva, and skin had vacuoles containing acid mucopolysaccharide. This change was most marked in the cornea. Plasma cells with Russell bodies frequently occurred in the conjunctival stroma.

Original language	English (US)
Pages (from-to)	809-818
Number of pages	10
Journal	American journal of ophthalmology
Volume	77
Issue number	6
DOIs	https://doi.org/10.1016/0002-9394(74)90383-3
State	Published - Jun 1974

ASJC Scopus subject areas

Ophthalmology

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10.1016/0002-9394(74)90383-3

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title = "Ultrastructural and histochemical studies of a newly recognized form of systemic mucopolysaccharidosis (Maroteaux-Lamy syndrome, mild phenotype)",

abstract = "A 31-year old man with corneal clouding and stiff joints, who may represent a newly recognized genetic form of systemic mucopolysaccharidosis type VI (MaroteauxLamy syndrome), was studied by histochemistry and electron microscopy. Connective tissue cells of cornea, conjunctiva, and skin had vacuoles containing acid mucopolysaccharide. This change was most marked in the cornea. Plasma cells with Russell bodies frequently occurred in the conjunctival stroma.",

author = "Quigley, {Harry A.} and Kenyon, {Kenneth R.}",

note = "Funding Information: From the Eye Pathology Laboratory, Wilmer Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland. This study was supported in part by grants from The Seeing Eye, Inc., Morristown, New Jersey (Dr. Quigley) and from Fight For Sight, Inc., New York City (Dr. Kenyon). Copyright: Copyright 2017 Elsevier B.V., All rights reserved.",

year = "1974",

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AU - Kenyon, Kenneth R.

N1 - Funding Information: From the Eye Pathology Laboratory, Wilmer Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland. This study was supported in part by grants from The Seeing Eye, Inc., Morristown, New Jersey (Dr. Quigley) and from Fight For Sight, Inc., New York City (Dr. Kenyon). Copyright: Copyright 2017 Elsevier B.V., All rights reserved.

PY - 1974/6

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AB - A 31-year old man with corneal clouding and stiff joints, who may represent a newly recognized genetic form of systemic mucopolysaccharidosis type VI (MaroteauxLamy syndrome), was studied by histochemistry and electron microscopy. Connective tissue cells of cornea, conjunctiva, and skin had vacuoles containing acid mucopolysaccharide. This change was most marked in the cornea. Plasma cells with Russell bodies frequently occurred in the conjunctival stroma.

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Ultrastructural and histochemical studies of a newly recognized form of systemic mucopolysaccharidosis (Maroteaux-Lamy syndrome, mild phenotype)

Abstract

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