{"orpha":293375,"name":"Grayson-Wilbrandt corneal dystrophy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["GWCD"],"definition":"Grayson-Wilbrandt corneal dystrophy (GWCD) is an extremely rare form of corneal dystrophy characterized by variable patterns of opacification in the Bowman layer of the cornea which extend anteriorly into the epithelium with decreased to normal visual acuity.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-293375/","orphanet_url":"https://www.orpha.net/en/disease/detail/293375","names":{"fr":{"name":"Dystrophie cornéenne de Grayson-Wilbrandt","synonyms":["GWCD"],"definition":"La dystrophie cornéenne de Grayson-Wildbrandt (GWCD) est une forme extrêmement rare de dystrophie cornéenne caractérisée par différents types d'opacification de la membrane de Bowman allant jusqu'à la partie antérieure de l'épithélium, avec une acuité visuelle réduite ou normale"},"de":{"name":"Grayson-Wilbrandt-Hornhautdystrophie","synonyms":["GWCD"],"definition":"Die Grayson-Wilbrandt-Hornhautdystrophie (GWCD) ist eine extrem seltene Form der Hornhautdystrophie und gekennzeichnet durch unterschiedlich gemusterte Trübungen der Bowman-Schicht der Kornea, die sich nach vorn in das Epithel ausweiten. Die Sehschärfe ist eingeschränkt oder bleibt normal."},"es":{"name":"Distrofia corneal de Grayson-Wilbrandt","synonyms":["GWCD"],"definition":"La distrofia corneal de Grayson-Wilbrandt es una forma muy rara de distrofia corneal, caracterizada por patrones variables de opacificación de la capa de Bowman de la córnea que se extienden anteriormente dentro del epitelio, con una agudeza visual entre normal y disminuida."},"it":{"name":"Distrofia corneale di Grayson-Wilbrandt","synonyms":["GWCD"],"definition":"La distrofia corneale di Grayson-Wilbrandt (GWCD) è una forma estremamente rara di distrofia della cornea, caratterizzata da quadri variabili di opacizzazione dello strato di Bowman della cornea, che si estende anteriormente fino all'epitelio. L'acuità visiva può essere diminuita o normale."},"nl":{"name":"Grayson-Wilbrandt-corneadystrofie","synonyms":["GWCD"],"definition":"Grayson-Wilbrandt-corneadystrofie (GWCD) is een uitermate zeldzame vorm van corneadystrofie, gekenmerkt door variabele patronen van opacificatie in de Bowmanlaag van de cornea die zich anterieur in het epitheel verspreiden met een verminderde tot normale gezichtsscherpte."},"pt":{"name":"Distrofia corneana de Grayson-Wilbrandt","synonyms":["GWCD"]},"pl":{"name":"Dystrofia rogówki Graysona i Wilbrandta","synonyms":["GWCD"]},"cs":{"name":"Graysonova-Wilbrandtova dystrofie rohovky","synonyms":["GWCD","Grayson-Wilbrandtova dystrofie rohovky"]},"uk":{"name":"Дистрофія рогівки Грейсона-Вільбрандта","synonyms":["GWCD"],"definition":"Grayson-Wilbrandt corneal dystrophy (GWCD) is an extremely rare form of corneal dystrophy characterized by variable patterns of opacification in the Bowman layer of the cornea which extend anteriorly into the epithelium with decreased to normal visual acuity."},"zh":{"name":"Grayson-Wilbrandt角膜营养不良","synonyms":["Grayson-Wilbrandt角膜营养不良"],"definition":"Grayson-Wilbrandt角膜营养不良（GWCD）是一种极为罕见的角膜营养不良，其特征是角膜前弹力层有不同类型的混浊并向前延伸至上皮细胞，伴视力下降。"},"tr":{"name":"Grayson-Wilbrandt kornea distrofisi","synonyms":["GWCD"]}},"xrefs":[{"source":"ICD-10","code":"H18.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9A70.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1667542240"},{"source":"MONDO","code":"0017391","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4305579","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":522562,"name":"Genetic superficial corneal dystrophy","classifications":[156]},{"orpha":98625,"name":"Superficial corneal dystrophy","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Adolescent","Adult","Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Family(ies)","class":null,"value":1.0,"area":"Worldwide","status":"Validated","source":"8230838[PMID]_[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_8230838[PMID]"}],"phenotypes":null}