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Williams RJ Virgilio LA (1976)
An unusually large human eye with abnormal scleral collagenArch Ophthalmol, 94
Brunning RD (1970)
Morphologic alterations in nucleated blood and marrow cells in genetic disordersHum Pathol, 1
Horn RG Spicer SS (1967)
The Connective Tissue
Okada S MacBrinn MC (1969)
Generalized gangliosidosis: Impaired cleavage of galactose from a mucopolysaccharide and a glycoproteinScience, 163
Meyer K Hoffman P (1956)
Transglycosilation during the mixed digestion of hyaluronic acid and chondroitin sulfate by testicular hyaluronidaseJ Biol Chem, 219
McKusick VA (1972)
Heritable Disorders of Connective Tissue
Quintarelli G Scott JE (1964)
The chemical and histochemical properties of Alcian blue: I. The mechanism of Alcian blue stainingHistochemistry, 4
Garner A (1969)
Histochemistry of corneal macular dystrophyInvest Ophthalmol Vis Sci, 8
Meyer K Sero N (1965)
Variations in keratosulfateJ Biochem, 240
O'Brien JS Okada S (1968)
Generalized gangliosidosis: ?-Galactosidase deficiencyScience, 160
Abstract • To our knowledge, this is the first clinicopathologic report of a previously unrecognized maculopathy in which there is thickening and mucopolysaccharide deposition in the sclera subjacent to the macula. A healthy 50-year-old man had bilateral mottling of the retinal pigment epithelium (RPE) in the macular region. The left eye was enucleated because of a choroidal melanoma. Histopathologic examination disclosed a thickened sclera with abnormal collagen fibrils with diameters up to 5,800 A and compressed choroid. The overlying RPE had areas of hypopigmentation, hyperpigmentation, and hyperplasia and contained intracytoplasmic lipofuscin. References 1. Spicer SS, Horn RG, Leppi TJ: Histochemistry of connective tissue mucopolysaccharides , in Wagner BM, Smith DE (eds): The Connective Tissue . Baltimore, Williams & Wilkins Co, 1967, pp 251-303. 2. McKusick VA: Heritable Disorders of Connective Tissue , ed 4. St Louis, CV Mosby Co, 1972, pp 629-630. 3. Brunning RD: Morphologic alterations in nucleated blood and marrow cells in genetic disorders . Hum Pathol 1970;1:99-124.Crossref 4. Scott JE, Quintarelli G, Dellovo MC: The chemical and histochemical properties of Alcian blue: I. The mechanism of Alcian blue staining . Histochemistry 1964;4:73-85.Crossref 5. Garner A: Histochemistry of corneal macular dystrophy . Invest Ophthalmol Vis Sci 1969;8:475-483. 6. Sero N, Meyer K, Anderson B, et al: Variations in keratosulfate . J Biochem 1965;240:1005-1010. 7. Okada S, O'Brien JS: Generalized gangliosidosis: β-Galactosidase deficiency . Science 1968;160:1002-1004.Crossref 8. MacBrinn MC, Okada S, Ho MW, et al: Generalized gangliosidosis: Impaired cleavage of galactose from a mucopolysaccharide and a glycoprotein . Science 1969;163:946-947.Crossref 9. Hoffman P, Meyer K, Linker A: Transglycosilation during the mixed digestion of hyaluronic acid and chondroitin sulfate by testicular hyaluronidase . J Biol Chem 1956;219:653-663. 10. Howard GM: Obscure scleral thickening. Read before the Eastern Ophthalmic Pathology Society Meeting, Philadelphia, April 12, 1971. 11. Virgilio LA, Williams RJ, Klintworth GK: An unusually large human eye with abnormal scleral collagen . Arch Ophthalmol 1976;94:101-105.Crossref 12. Mann I: The Development of the Human Eye , ed 3. New York, Grune & Stratton Inc, 1969, pp 254-255. 13. Duke-Elder S: System of Ophthalmology . St Louis, CV Mosby Co, pt 1, 1913, vol 3, pp 162-163.
Archives of Ophthalmology – American Medical Association
Published: May 1, 1982
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