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

Research output: Contribution to journalArticle

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 languageEnglish (US)
Pages (from-to)811-833
Number of pages23
JournalAmerican Journal of Ophthalmology
Volume73
Issue number6
StatePublished - 1972

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Mucopolysaccharidoses
Conjunctiva
Skin
Connective Tissue Cells
Glycolipids
Vacuoles
Lysosomes
Glycosaminoglycans
Electron Microscopy
Cell Membrane
Acids

ASJC Scopus subject areas

  • Ophthalmology

Cite this

The systemic mucopolysaccharidoses. Ultrastructural and histochemical studies of conjunctiva and skin. / Kenyon, Kenneth R.; Quigley, Harry A; Hussels, Irene E.; Wyllie, Robert G.; Goldberg, Morton F.

In: American Journal of Ophthalmology, Vol. 73, No. 6, 1972, p. 811-833.

Research output: Contribution to journalArticle

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