The systemic mucopolysaccharidoses. Ultrastructural and histochemical studies of conjunctiva and skin

Kenneth R. Kenyon; Harry A. Quigley; Irene E. Hussels; Robert G. Wyllie; Morton F. Goldberg

doi:10.1016/0002-9394(72)90449-7

The systemic mucopolysaccharidoses. Ultrastructural and histochemical studies of conjunctiva and skin

Kenneth R. Kenyon, Harry A. Quigley, Irene E. Hussels, Robert G. Wyllie, Morton F. Goldberg

School of Medicine

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

47 Scopus citations

Abstract

Conjunctiva and skin from nine patients representing each of the six systemic mucopolysaccharidoses were examined by histochemistry and electron microscopy. Connective tissue cells consistently exhibited abnormal vacuolation by single-membrane-limited cytoplasmic bodies. These vacuoles, which contained predominantly fibrillogranular and occasionally membranous lamellar inclusions, are thought to represent lysosomes engorged with excessive accumulation of acid mucopolysaccharide and glycolipid, respectively. Among all specimens, the extent of vacuolation was more variable in conjunctiva than in skin. The extent of cellular abnormality in the conjunctiva did not consistently parallel to degree of corneal opacification.

Original language	English (US)
Pages (from-to)	811-833
Number of pages	23
Journal	American journal of ophthalmology
Volume	73
Issue number	6
DOIs	https://doi.org/10.1016/0002-9394(72)90449-7
State	Published - Jun 1972

ASJC Scopus subject areas

Ophthalmology

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abstract = "Conjunctiva and skin from nine patients representing each of the six systemic mucopolysaccharidoses were examined by histochemistry and electron microscopy. Connective tissue cells consistently exhibited abnormal vacuolation by single-membrane-limited cytoplasmic bodies. These vacuoles, which contained predominantly fibrillogranular and occasionally membranous lamellar inclusions, are thought to represent lysosomes engorged with excessive accumulation of acid mucopolysaccharide and glycolipid, respectively. Among all specimens, the extent of vacuolation was more variable in conjunctiva than in skin. The extent of cellular abnormality in the conjunctiva did not consistently parallel to degree of corneal opacification.",

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AU - Wyllie, Robert G.

AU - Goldberg, Morton F.

PY - 1972/6

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N2 - Conjunctiva and skin from nine patients representing each of the six systemic mucopolysaccharidoses were examined by histochemistry and electron microscopy. Connective tissue cells consistently exhibited abnormal vacuolation by single-membrane-limited cytoplasmic bodies. These vacuoles, which contained predominantly fibrillogranular and occasionally membranous lamellar inclusions, are thought to represent lysosomes engorged with excessive accumulation of acid mucopolysaccharide and glycolipid, respectively. Among all specimens, the extent of vacuolation was more variable in conjunctiva than in skin. The extent of cellular abnormality in the conjunctiva did not consistently parallel to degree of corneal opacification.

AB - Conjunctiva and skin from nine patients representing each of the six systemic mucopolysaccharidoses were examined by histochemistry and electron microscopy. Connective tissue cells consistently exhibited abnormal vacuolation by single-membrane-limited cytoplasmic bodies. These vacuoles, which contained predominantly fibrillogranular and occasionally membranous lamellar inclusions, are thought to represent lysosomes engorged with excessive accumulation of acid mucopolysaccharide and glycolipid, respectively. Among all specimens, the extent of vacuolation was more variable in conjunctiva than in skin. The extent of cellular abnormality in the conjunctiva did not consistently parallel to degree of corneal opacification.

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The systemic mucopolysaccharidoses. Ultrastructural and histochemical studies of conjunctiva and skin

Abstract

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