Biomaterial Database

[Corneal dystrophy and Ehlers-Danlos syndrome]. 1983

Y Pouliquen, and G Petroutsos, and D Papaioannou

A case of bilateral corneal dystrophy in a young 6-year-old patient with an Ehlers-Danlos syndrome is presented. Thin opaque lamellae of different densities were observed in the posterior corneal stroma, and there were some subepithelial opacities. An irregularity of Descemet's membrane was also noted. The classification of this dystrophy is discussed, as well as the possible relation between the dystrophy and diseases of the elastic connective tissue.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003094	Collagen	A polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of SKIN; CONNECTIVE TISSUE; and the organic substance of bones (BONE AND BONES) and teeth (TOOTH).	Avicon,Avitene,Collagen Felt,Collagen Fleece,Collagenfleece,Collastat,Dermodress,Microfibril Collagen Hemostat,Pangen,Zyderm,alpha-Collagen,Collagen Hemostat, Microfibril,alpha Collagen
D003238	Connective Tissue	Tissue that supports and binds other tissues. It consists of CONNECTIVE TISSUE CELLS embedded in a large amount of EXTRACELLULAR MATRIX.	Connective Tissues,Tissue, Connective,Tissues, Connective
D003317	Corneal Dystrophies, Hereditary	Bilateral hereditary disorders of the cornea, usually autosomal dominant, which may be present at birth but more frequently develop during adolescence and progress slowly throughout life. Central macular dystrophy is transmitted as an autosomal recessive defect.	Corneal Dystrophies,Granular Dystrophy, Corneal,Groenouw's Dystrophies,Macular Dystrophy, Corneal,Stromal Dystrophies, Corneal,Corneal Dystrophy,Corneal Dystrophy, Hereditary,Corneal Granular Dystrophies,Corneal Granular Dystrophy,Corneal Macular Dystrophies,Corneal Macular Dystrophy,Corneal Stromal Dystrophies,Corneal Stromal Dystrophy,Dystrophy, Corneal,Dystrophy, Corneal Granular,Dystrophy, Corneal Macular,Dystrophy, Corneal Stromal,Dystrophy, Hereditary Corneal,Groenouw Dystrophies,Groenouws Dystrophies,Hereditary Corneal Dystrophies,Hereditary Corneal Dystrophy,Stromal Dystrophy, Corneal
D003319	Corneal Stroma	The lamellated connective tissue constituting the thickest layer of the cornea between the Bowman and Descemet membranes.	Corneal Stromas,Stroma, Corneal,Stromas, Corneal
D003886	Descemet Membrane	A layer of the cornea. It is the basal lamina of the CORNEAL ENDOTHELIUM (from which it is secreted) separating it from the CORNEAL STROMA. It is a homogeneous structure composed of fine collagenous filaments, and slowly increases in thickness with age.	Lamina Elastica Posterior,Descemet's Membrane,Descemet Membranes,Descemets Membrane,Elastica Posterior, Lamina,Elastica Posteriors, Lamina,Lamina Elastica Posteriors,Membrane, Descemet,Membrane, Descemet's,Membranes, Descemet,Posterior, Lamina Elastica,Posteriors, Lamina Elastica
D004535	Ehlers-Danlos Syndrome	A heterogeneous group of autosomally inherited COLLAGEN DISEASES caused by defects in the synthesis or structure of FIBRILLAR COLLAGEN. There are numerous subtypes: classical, hypermobility, vascular, and others. Common clinical features include hyperextensible skin and joints, skin fragility and reduced wound healing capability.	Cutis Elastica,Ehlers Danlos Disease,Ehlers-Danlos Disease,Danlos Disease, Ehlers,Disease, Ehlers Danlos,Disease, Ehlers-Danlos,Ehlers Danlos Syndrome,Syndrome, Ehlers-Danlos
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man