{"orpha":98971,"name":"Posterior amorphous corneal dystrophy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PACD","Posterior amorphous stromal dystrophy"],"definition":"Posterior amorphous corneal dystrophy (PACD) is a very rare form of stromal corneal dystrophy characterized by irregular amorphous sheet-like opacities in the posterior corneal stroma and in Descemet membrane and mildly impaired vision.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98971/","orphanet_url":"https://www.orpha.net/en/disease/detail/98971","names":{"fr":{"name":"Dystrophie cornéenne amorphe postérieure","synonyms":["Dystrophie stromale amorphe postérieure","PACD"],"definition":"La dystrophie cornéenne amorphe postérieure (PACD) est une forme très rare de dystrophie cornéenne stromale caractérisée par des opacités irrégulières et amorphes en forme de feuilles localisées dans le stroma cornéen postérieur et la membrane de Descemet, avec une légère détérioration de la vision."},"de":{"name":"Hornhautdystrophie, amorphe posteriore","synonyms":["PACD","Posteriore amorphe Stromadystrophie"],"definition":"Die Hintere amorphe Stroma-Dystrophie (PACD) ist eine sehr seltene Form der Stroma-Hornhautdystrophie (s. dort) und gekennzeichnet durch unregelmäßige, amorphe, blattförmige Trübungen im hinteren Hornhautstroma und in der Descemet-Membran. Der Visus ist nur leicht beeinträchtigt."},"es":{"name":"Distrofia corneal posterior amorfa","definition":"La distrofia corneal posterior amorfa (PACD) es una forma muy rara de distrofia corneal estromal (consulte este término) caracterizada por opacidades irregulares y amorfas diseminadas en el estroma corneal posterior y en la membrana de Descemet, y por una afección leve de la visión."},"it":{"name":"Distrofia corneale amorfa posteriore","synonyms":["Distrofia stromale amorfa posteriore","PACD"],"definition":"La distrofia corneale amorfa posteriore (PACD) è una forma molto rara di distrofia stromale della cornea (si veda questo termine), caratterizzata da opacità amorfe irregolari, simili a foglietti, nello stroma corneale posteriore e nella membrana di Descemet, e da lieve deficit visivo."},"nl":{"name":"Posterieure amorfe corneadystrofie","synonyms":["PACD","Posterieure amorfe hoornvliesdystrofie","Posterieure amorfe stromale dystrofie"],"definition":"Posterieure amorfe corneadystrofie (PACD) is een zeer zeldzame vorm van stromale corneadystrofie (zie deze term) gekenmerkt door onregelmatige amorfe plaatachtige opaciteiten in het posterieure corneastroma en het Descemetmembraan en een mild verslechterd gezichtsvermogen."},"pt":{"name":"Distrofia corneana, posterior amorfa"},"pl":{"name":"Amorficzna tylna dystrofia rogówki","synonyms":["Amorficzna tylna dystrofia istoty właściwej","PACD"]},"cs":{"name":"Zadní amorfní dystrofie rohovky","synonyms":["PACD","Zadní amorfní stromální dystrofie rohovky"]},"uk":{"name":"Задня аморфна дистрофія рогівки","synonyms":["PACD","Задня аморфна стромальна дистрофія"],"definition":"Posterior amorphous corneal dystrophy (PACD) is a very rare form of stromal corneal dystrophy (see this term) characterized by irregular amorphous sheet-like opacities in the posterior corneal stroma and in Descemet membrane and mildly impaired vision."},"zh":{"name":"后部多形性角膜营养不良","synonyms":["后部多形性角膜基质营养不良"],"definition":"后部无定形角膜营养不良(PACD)是一种非常罕见的间质性角膜营养不良(见该词条)，其特征是角膜后基质和后弹力膜出现不规则无定形片状混浊，视力轻度受损。"},"tr":{"name":"Posterior amorf korneal distrofi","synonyms":["PACD","Posterior amorf stromal distrofi"]}},"xrefs":[{"source":"OMIM","code":"612868","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":"347556972"},{"source":"MONDO","code":"0013027","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C567546","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2748502","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":["Infancy","Neonatal"],"prevalence":[{"type":"Cases/families","qualification":"Family(ies)","class":null,"value":11.0,"area":"Worldwide","status":"Validated","source":"301356[PMID]_2314832[PMID]_1582220[PMID]_7573302[PMID]_8600425[PMID]_17273695[PMID]_17300585[PMID]_11862100[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}