{"orpha":98972,"name":"Central cloudy dystrophy of François","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["CCDF","Central cloudy corneal dystrophy of François"],"definition":"Central cloudy dystrophy of François is a very rare form of stromal corneal dystrophy characterized by polygonal or rounded stromal opacities surrounded by clear tissue, and generally no effect on vision.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98972/","orphanet_url":"https://www.orpha.net/en/disease/detail/98972","names":{"fr":{"name":"Dystrophie nuageuse centrale de François","synonyms":["CCDF","Dystrophie cornéenne nuageuse centrale de François"],"definition":"La dystrophie nuageuse centrale de François est une forme très rare de dystrophie cornéenne stromale caractérisée par des opacités polygonales ou rondes entourées d'un tissu clair, généralement sans impact sur la vision."},"de":{"name":"Zentral-wolkenförmige Dystrophie François","synonyms":["CCDF","Hornhautdystrophie, zentral-wolkenförmige, Typ François"],"definition":"Die Zentral-wolkenförmige Dystrophie von François ist eine sehr seltene Form der Stroma-Hornhautdystrophie (s. dort) und gekennzeichnet durch polygonale oder abgerundete, von klarem Gewebe umgebenen Stromatrübungen, die in der Regel den Visus nicht beeinträchtigen."},"es":{"name":"Distrofia nebulosa central de François","synonyms":["CCDF","Distrofia corneal nebulosa central de François"],"definition":"La distrofia nebulosa central de François es una forma muy rara de distrofia corneal estromal (consulte este término) caracterizada por opacidades estromales redondeadas o poligonales, rodeadas de tejido claro, y por no tener, por lo general, efecto en la visión."},"it":{"name":"Distrofia torbida centrale di François","synonyms":["Distrofia corneale torbida centrale di François","CCDF"],"definition":"La distrofia torbida centrale di François è una forma molto rara di distrofia dello stroma corneale (si veda questo termine), caratterizzata da opacità stromali di forma arrotondata o poligonale circondate da tessuti chiari, in genere senza compromissione della vista."},"nl":{"name":"Centrale troebele dystrofie van François","synonyms":["'Central cloudy dystrophy' van François","CCDF","Centrale troebele corneadystrofie van François"],"definition":"Centrale troebele dystrofie van François is een zeer zeldzame vorm van stromale corneadystrofie (zie deze term) gekenmerkt door polygonale of afgeronde stromale opaciteiten omgeven door helder weefsel, en meestal zonder effect op het gezichtsvermogen."},"pt":{"name":"Distrofia corneana, nublada central, tipo Francois"},"pl":{"name":"Dystrofia centralnego zmętnienia François","synonyms":["CCDF","Dystrofia centralnego zmętnienia rogówki François"]},"cs":{"name":"Francoisova centrální cloudy dystrofie","synonyms":["CCDF","Centrální Francoisova cloudy dystrofie rohovky"]},"uk":{"name":"Центральна мутна дистрофія Франсуа","synonyms":["CCDF","Центральна мутна дистрофія рогівки Франсуа"],"definition":"Central cloudy dystrophy of François is a very rare form of stromal corneal dystrophy (see this term) characterized by polygonal or rounded stromal opacities surrounded by clear tissue, and generally no effect on vision."},"zh":{"name":"Franþois角膜中央浑浊性角膜营养不良","synonyms":["François中心性云翳状角膜营养不良"],"definition":"角膜中央性云状角膜营养是一种非常罕见的间质性角膜营养不良(见该词条)，其特征是被透明组织包围的多角形或圆形间质混浊，通常对视力没有影响。"},"tr":{"name":"François santral bulutlu distrofisi","synonyms":["CCDF","François santral bulutlu kornea distrofisi"]}},"xrefs":[{"source":"OMIM","code":"217600","qualifier":"E","icd_relation":null,"validated":true},{"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":"982651620"},{"source":"MONDO","code":"0009018","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C563262","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1622427","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":98626,"name":"Stromal corneal dystrophy","classifications":[156,189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":24.0,"area":"Worldwide","status":"Validated","source":"5305046[PMID]_1083619[PMID]_15734014[PMID]_15534129[PMID]_9258229[PMID]_8776559[PMID]_20337272[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}