Ultrastructural aspects of corneal fibrous tissue in the Scheie syndrome

An ultrastructural study of the corneal fibrous tissue was performed in a case of Scheie's syndrome. Mucopolysaccharidosis deposits in keratocytes were observed as electron-clear and electron-dense inclusions. Modifications of the extracellular space included modifications of lamellar collagen...

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Bibliographic Details
Published inVirchows Archiv. B, Cell pathology Vol. 27; no. 1; p. 63
Main Authors Tabone, E, Grimaud, J A, Peyrol, S, Grandperret, D, Durand, L
Format Journal Article
LanguageEnglish
Published Germany 01.12.1978
Subjects
Biopsy
Collagen
Cornea - pathology
Cornea - ultrastructure
Extracellular Space - ultrastructure
Humans
Inclusion Bodies - ultrastructure
Microscopy, Electron
Mucopolysaccharidoses - pathology
Mucopolysaccharidosis I - pathology
Proteoglycans
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Summary:An ultrastructural study of the corneal fibrous tissue was performed in a case of Scheie's syndrome. Mucopolysaccharidosis deposits in keratocytes were observed as electron-clear and electron-dense inclusions. Modifications of the extracellular space included modifications of lamellar collagen organization and local hypertrophy of collagen bundles; presence of microfibrillar dense material isolating large irregular collagen fibers; and presence of fibrous long spacing type collagen fibers. The significance of these changes is discussed. This special form of collagen organization is supposed to appear in a modified microenvironment, that is the presence of an abnormal concentration of proteoglycans.
DOI:10.1007/BF02888983