chondroitin and Corneal-Opacity

Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chondroitin; Cornea; Corneal Opacity; Dermatan Sulfate; Fibroblasts; Galactose; Glucosamine; Glycosaminoglycans; Heparin; Humans; Hyaluronic Acid; Hyaluronoglucosaminidase; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Proteoglycans; Retinitis Pigmentosa; Skin; Staining and Labeling; Sulfatases; Testis

Topics: Carbohydrate Metabolism, Inborn Errors; Chemical Phenomena; Chemistry; Chondroitin; Corneal Opacity; Gangliosides; Glycosaminoglycans; Histocytochemistry; Humans; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Chemical Phenomena; Chemistry; Chondroitin; Corneal Opacity; Female; Glucuronidase; Glycosaminoglycans; Humans; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Amniocentesis; Animals; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Corneal Opacity; Culture Techniques; Dogs; Galactosidases; Genes, Recessive; Glycolipids; Glycosaminoglycans; Heparin; Humans; Intellectual Disability; Liver; Mucopolysaccharidoses; Prognosis; Retinitis Pigmentosa; Sex Chromosomes; Sulfates; Sulfur Isotopes

Topics: Age Factors; Atmospheric Pressure; Burns; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Connective Tissue; Corneal Opacity; Glomerular Filtration Rate; Glycosaminoglycans; Humans; Intellectual Disability; Lupus Erythematosus, Systemic; Mucopolysaccharidosis IV; Neoplasms; Retinitis Pigmentosa

To describe new affected individuals of Franceschetti's original pedigree of hereditary recurrent erosion and to classify a unique entity called Franceschetti corneal dystrophy.. Observational case series.. Slit-lamp examination of 10 affected individuals was conducted. Biomicroscopic examinations were supplemented by peripheral corneal biopsy in 1 affected patient with corneal haze. Tissue was processed for light and electron microscopy and immunohistochemistry was performed. DNA analysis was carried out in 12 affected and 3 nonaffected family members.. All affected individuals suffered from severe ocular pain in the first decade of life, attributable to recurrent corneal erosions. Six adult patients developed bilateral diffuse subepithelial opacifications in the central and paracentral cornea. The remaining 4 affected individuals had clear corneas in the pain-free stage of the disorder. Histologic and immunohistochemical examination of the peripheral cornea in a single patient showed a subepithelial, avascular pannus. There was negative staining with Congo red. DNA analysis excluded mutations in the transforming growth factor beta-induced (TGFBI) gene and in the tumor-associated calcium signal transducer 2 (TACSTD2) gene.. We have extended the pedigree of Franceschetti corneal dystrophy and elaborated its natural history on the basis of clinical examinations. A distinctive feature is the appearance of subepithelial opacities in adult life, accompanied by a decreased frequency of recurrent erosion attacks. Its clinical features appear to distinguish it from most other forms of dominantly inherited recurrent corneal erosion reported in the literature.

Topics: Adult; Aged; Aged, 80 and over; Antigens, Neoplasm; Biomarkers; Biopsy; Cadherins; Cell Adhesion Molecules; Child; Chondroitin; Claudins; Corneal Dystrophies, Hereditary; Corneal Opacity; Decorin; Dermatan Sulfate; DNA Mutational Analysis; Extracellular Matrix Proteins; Eye Pain; Female; Humans; Immunohistochemistry; Male; Pedigree; Recurrence; Transforming Growth Factor beta

A patient developed an acute onset of pseudophakic bullous keratopathy and a diffuse, gray, subepithelial corneal deposit ten days following surgery during which sodium chondroitin sulfate was used. The deposit, identified histologically as calcium, was noted throughout Bowman's layer. The absence of chronic pathologic features in the posterior cornea eliminated Fuchs' endothelial dystrophy as the cause of the acute corneal decompensation. This histopathologic confirmation of calcium deposition with one formulation of chondroitin sulfate, combined with similar conclusions from previous case reports, stimulated the reformulation of the product, which has been shown to be useful in clinical practice.

Topics: Aged; Calcinosis; Cataract Extraction; Chondroitin; Cornea; Corneal Diseases; Corneal Opacity; Corneal Transplantation; Female; Humans; Lenses, Intraocular; Postoperative Complications

Viscoat is a new viscoelastic solution of chondroitin sulfate and sodium hyaluronate dissolved in a phosphate buffer. Recent findings of postoperative corneal deposits in association with its use have been reported to Cilco. The corneal deposits are noted 18 to 48 hours postoperatively and are similar in appearance to calcific band keratopathy. The clinical characteristics and prognosis of this entity are reviewed. Possible methods of treatment and recommendations for prevention are discussed.

Topics: Aged; Aged, 80 and over; Cataract Extraction; Chondroitin; Chondroitin Sulfates; Corneal Opacity; Drug Combinations; Female; Humans; Hyaluronic Acid; Time Factors; Visual Acuity

Two male and two female sibs with an unusual form of spondyloepiphyseal dysplasia were reported. The main clinical features were low stature, moderate shortness of trunk and neck, abnormal span: height ratio, low-normal UBS: LBS ratio, and peripheral corneal punctate opacities only seen by the slitlamp. Normal mental status was present. Typical metachromatic granules were not seen either in bone-marrow cells or in peripheral blood cells. The X-ray picture showed spondylar and pelvic dysplasia. Qualitative rather than quantitative anomalies were shown in the urinary mucopolysaccharides, mostly involving chondroitin-6-sulfate. The genetic data are consistent with autosomal recessive inheritance.

Topics: Abnormalities, Multiple; Adolescent; Adult; Child; Child, Preschool; Chondroitin; Chondroitin Sulfates; Corneal Opacity; Female; Genes, Recessive; Humans; Male; Mucopolysaccharidosis IV; Pedigree; Time Factors

Topics: Blood Transfusion; Chondroitin; Corneal Opacity; Glycosaminoglycans; Humans; Mucopolysaccharidoses; Mucopolysaccharidosis I; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Sulfates; Syndrome

Topics: Biotransformation; Chondroitin; Corneal Opacity; Glucuronidase; Glycosaminoglycans; Glycoside Hydrolases; Hexosaminidases; Humans; Intellectual Disability; Lysosomes; Mucopolysaccharidoses; Retinitis Pigmentosa; Sulfatases

Topics: Adult; Basement Membrane; Biopsy; Chondroitin; Conjunctiva; Connective Tissue Cells; Cornea; Corneal Opacity; Cytoplasm; Epithelial Cells; Fibroblasts; Glycosaminoglycans; Histiocytes; Histocytochemistry; Humans; Inclusion Bodies; Joint Diseases; Male; Microscopy, Electron; Mucopolysaccharidoses; Phenotype; Plasma Cells; Retinitis Pigmentosa; Schwann Cells; Syndrome

Topics: Biopsy; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Conjunctiva; Corneal Dystrophies, Hereditary; Corneal Opacity; Endoplasmic Reticulum; Glycosaminoglycans; Golgi Apparatus; Humans; Hyalin; Inclusion Bodies; Intellectual Disability; Macula Lutea; Microscopy, Electron; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Plasma Cells; Retinitis Pigmentosa

Topics: Child; Child, Preschool; Chondroitin; Consanguinity; Corneal Opacity; Female; Glycosaminoglycans; Heparitin Sulfate; Humans; Male; Mucopolysaccharidoses; Syndrome

Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chondroitin; Conjunctiva; Cornea; Corneal Opacity; Electrophoresis; Fibroblasts; Glycosaminoglycans; Histocytochemistry; Humans; Hyaluronic Acid; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Skin; Syndrome; Uronic Acids

Topics: Adolescent; Adult; Carbazoles; Carbohydrate Metabolism, Inborn Errors; Child; Chondroitin; Corneal Opacity; Female; Glycosaminoglycans; Heparitin Sulfate; Humans; Intellectual Disability; Male; Methods; Mucopolysaccharidoses; Resorcinols; Retinitis Pigmentosa; Uronic Acids

Topics: Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Chromatography; Corneal Opacity; Electrophoresis; Female; Galactosamine; Glycosaminoglycans; Heparitin Sulfate; Hexosamines; Humans; Hyaluronic Acid; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Sulfates; Uronic Acids

Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Chromatography, Gas; Chromatography, Gel; Corneal Opacity; Glycosaminoglycans; Heparin; Hexosamines; Humans; Hydrolysis; Intellectual Disability; Methanol; Monosaccharides; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Uronic Acids

Catabolism of sulfated mucopolysaccharide by normal human fibroblasts in culture is progressively inhibited as the pH of the growth medium is raised from 6.8 to 8.0. The final cell density increases with the change in pH. The capacity to degrade mucopolysaccharide is rapidly restored by lowering the pH, and this reactivation does not require protein synthesis. Such pH dependence is not observed in cells from patients with genetic impairment of mucopolysaccharide degradation, such as the Hurler or Hunter syndromes. These results may have relevance not only to studies of mucopolysaccharide metabolism in cell culture, but also to the use of metachromasia as a genetic marker and to the observation that normal fibroblasts are released from contact inhibition of growth as the pH of the growth medium is raised.

Topics: Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chondroitin; Contact Inhibition; Corneal Opacity; Fibroblasts; Glycosaminoglycans; Humans; Hydrogen-Ion Concentration; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa; Skin; Sulfur Isotopes; Sulfuric Acids

Topics: Blood Chemical Analysis; Bone and Bones; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Corneal Opacity; Deafness; Diagnosis, Differential; Dwarfism; Facial Expression; Female; Glycosaminoglycans; Heart Diseases; Humans; Infant; Intellectual Disability; Male; Mucopolysaccharidosis I; Mucopolysaccharidosis IV; Radiography; Retinitis Pigmentosa

Topics: Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Corneal Opacity; Female; Glycosaminoglycans; Humans; Infant; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Corneal Opacity; Glycosaminoglycans; Humans; Intellectual Disability; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Alleles; Carbohydrate Metabolism, Inborn Errors; Chondroitin; Corneal Opacity; Female; Fibroblasts; Glycosaminoglycans; Homozygote; Humans; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Mutation; Phenotype; Retinitis Pigmentosa

Topics: Bone and Bones; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Corneal Opacity; Female; Glycosaminoglycans; Heparin; Humans; Male; Radiography; Retinitis Pigmentosa

Topics: Carbohydrate Metabolism, Inborn Errors; Chondroitin; Chromatography, Paper; Corneal Opacity; Creatinine; Electrocardiography; Female; Glycosaminoglycans; Hepatomegaly; Humans; Intellectual Disability; Middle Aged; Mucopolysaccharidoses; Radiography

Topics: Adolescent; Adult; Carbohydrate Metabolism, Inborn Errors; Child; Chondroitin; Corneal Opacity; Female; Galactosamine; Glycosaminoglycans; Hexosamines; Humans; Intelligence; Male; Methods; Mucopolysaccharidoses; Radiography; Retinitis Pigmentosa; Sulfates; Visual Acuity

Topics: Adolescent; Chondroitin; Corneal Opacity; Craniofacial Dysostosis; Diagnosis, Differential; Dwarfism; Encephalocele; Female; Humans; Mucopolysaccharidoses; Pneumoencephalography; Syndrome

Topics: Adolescent; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Chromatography; Corneal Opacity; Diagnosis, Differential; Female; Glycosaminoglycans; Heparin; Humans; Hyaluronic Acid; Infant; Intellectual Disability; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Adolescent; Bone and Bones; Carbohydrate Metabolism, Inborn Errors; Child; Chondroitin; Corneal Opacity; Female; Heparin; Humans; Hypertrichosis; Intellectual Disability; Joint Diseases; Male; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Adolescent; Adult; Carbohydrate Metabolism, Inborn Errors; Child; Child, Preschool; Chondroitin; Connective Tissue; Corneal Opacity; Ehlers-Danlos Syndrome; Female; Glycosaminoglycans; Humans; Infant; Infant, Newborn; Intellectual Disability; Male; Marfan Syndrome; Mucopolysaccharidoses; Mucopolysaccharidosis IV; Retinitis Pigmentosa

Topics: Atrophy; Child; Chondroitin; Chymotrypsin; Conjunctivitis; Corneal Opacity; Eyelids; Female; Glycosaminoglycans; Humans; Hyaluronic Acid; Hyaluronoglucosaminidase; Middle Aged; Mucous Membrane; Mucus

Topics: Adolescent; Adult; Chondroitin; Cornea; Corneal Opacity; Female; Glucosamine; Humans; Keratoconus; Male; Middle Aged; Polysaccharides

Topics: Animals; Chondroitin; Cornea; Corneal Opacity; Protective Agents; Rabbits; Research; Retinal Detachment; Sulfates; Sulfuric Acids; Surgical Procedures, Operative