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Biochemical and Histological Analysis of 'Recurrent' Macular Corneal Dystrophy

Biochemical and Histological Analysis of 'Recurrent' Macular Corneal Dystrophy Abstract • A patient with macular corneal dystrophy who had a successful 6-mm corneal transplant 23 years ago underwent a second keratoplasty for marked irregular astigmatism. The excised button, which contained the original graft and a rim of host cornea, was divided into several portions. One portion was examined histologically, another portion was incubated in organ culture with radioactive precursors and the biosynthetically labeled products characterized, and a third portion was used for cell culture and karotype analysis. The results indicated that host stromacytes had not invaded the graft and that graft stromacytes had synthesized normal proteoglycans. Furthermore, although there was excessive synthesis of abnormal proteoglycan by host stromacytes and accumulation of this material in the host cornea, minor amounts of this material actually accumulated in the graft cornea, possibly contributing to the astigmatism. A large transpant that would leave a minimum of host corneal tissue may be conducive to a longer-term good result in patients with macular corneal dystrophy. References 1. Klintworth GL, Vogel FS: Macular corneal dystrophy, an inherited acid mucopolysaccharide storage disease of the corneal fibroblast . Am J Pathol 1964;45:565-576. 2. Gardner A: Histochemistry of corneal macular dystrophy . Invest Ophthalmol Vis Sci 1969;8:475-483. 3. Newsome DA, Foidart JM, Hassell JR, et al: Detection of specific collagen types in normal and keratoconus corneas . Invest Ophthalmol Vis Sci 1981;20:738-750. 4. Axelsson I, Heinegard D: Fractionatian of proteoglycans from bovine corneal stroma . Biochem J 1975;145:491-500. 5. Hassell JR, Newsome DA, Hascall VC: Characterization and biosynthesis of proteoglycans of corneal stroma from rhesus monkey . J Biol Chem 1979;254:12346-12354. 6. Hassell JR, Newsome DA, Krachmer JH, et al: Macular corneal dystrophy: Failure to synthesize a mature keratin sulfate proteoglycan . Proc Natl Acad Sci USA 1980;77:3705-3709.Crossref 7. Klintworth GK, Smith CF: Macular corneal dystrophy, studies on sulfated glycosaminoglycans in corneal explant and in confluent stromal cell cultures . Am J Pathol 1977;89:167-181. 8. Hassell JR, Newsome DA, Nakazawa K, et al: Deposits in macular corneal dystrophy contain abnormal proteoglycans . Assoc Res Vision Ophthalmol 1981;20:115. 9. Kaufman HE, Lorenzetti DWC: Macular and lattice dystrophies and their recurrences after keratoplasty . Trans Am Acad Ophthalmol Otolaryngol 1967;71:112-118. 10. Herman SJ, Hughes WF: Recurrence of hereditary corneal dystrophy following keratoplasty . Am J Ophthalmol 1973;75:689-694. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

Biochemical and Histological Analysis of 'Recurrent' Macular Corneal Dystrophy

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Publisher
American Medical Association
Copyright
Copyright © 1982 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1982.01030040103019
Publisher site
See Article on Publisher Site

Abstract

Abstract • A patient with macular corneal dystrophy who had a successful 6-mm corneal transplant 23 years ago underwent a second keratoplasty for marked irregular astigmatism. The excised button, which contained the original graft and a rim of host cornea, was divided into several portions. One portion was examined histologically, another portion was incubated in organ culture with radioactive precursors and the biosynthetically labeled products characterized, and a third portion was used for cell culture and karotype analysis. The results indicated that host stromacytes had not invaded the graft and that graft stromacytes had synthesized normal proteoglycans. Furthermore, although there was excessive synthesis of abnormal proteoglycan by host stromacytes and accumulation of this material in the host cornea, minor amounts of this material actually accumulated in the graft cornea, possibly contributing to the astigmatism. A large transpant that would leave a minimum of host corneal tissue may be conducive to a longer-term good result in patients with macular corneal dystrophy. References 1. Klintworth GL, Vogel FS: Macular corneal dystrophy, an inherited acid mucopolysaccharide storage disease of the corneal fibroblast . Am J Pathol 1964;45:565-576. 2. Gardner A: Histochemistry of corneal macular dystrophy . Invest Ophthalmol Vis Sci 1969;8:475-483. 3. Newsome DA, Foidart JM, Hassell JR, et al: Detection of specific collagen types in normal and keratoconus corneas . Invest Ophthalmol Vis Sci 1981;20:738-750. 4. Axelsson I, Heinegard D: Fractionatian of proteoglycans from bovine corneal stroma . Biochem J 1975;145:491-500. 5. Hassell JR, Newsome DA, Hascall VC: Characterization and biosynthesis of proteoglycans of corneal stroma from rhesus monkey . J Biol Chem 1979;254:12346-12354. 6. Hassell JR, Newsome DA, Krachmer JH, et al: Macular corneal dystrophy: Failure to synthesize a mature keratin sulfate proteoglycan . Proc Natl Acad Sci USA 1980;77:3705-3709.Crossref 7. Klintworth GK, Smith CF: Macular corneal dystrophy, studies on sulfated glycosaminoglycans in corneal explant and in confluent stromal cell cultures . Am J Pathol 1977;89:167-181. 8. Hassell JR, Newsome DA, Nakazawa K, et al: Deposits in macular corneal dystrophy contain abnormal proteoglycans . Assoc Res Vision Ophthalmol 1981;20:115. 9. Kaufman HE, Lorenzetti DWC: Macular and lattice dystrophies and their recurrences after keratoplasty . Trans Am Acad Ophthalmol Otolaryngol 1967;71:112-118. 10. Herman SJ, Hughes WF: Recurrence of hereditary corneal dystrophy following keratoplasty . Am J Ophthalmol 1973;75:689-694.

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Jul 1, 1982

References