{"orpha":98627,"name":"Posterior corneal dystrophy","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"Posterior corneal dystrophies refers to a group of rare genetically determined corneal dystrophies (CDs) characterized by lesions affecting the corneal endothelium and Descemet membrane, and variable effects on vision depending on the type of dystrophy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98627/","orphanet_url":"https://www.orpha.net/en/disease/detail/98627","names":{"fr":{"name":"Dystrophie cornéenne postérieure","definition":"Le terme de dystrophie cornéenne postérieure se rapporte à un groupe de dystrophies cornéennes génétiques rares caractérisées par des lésions impliquant l'endothélium cornéen et la membrane de Descemet, avec des effets variables sur la vision selon le type de dystrophie."},"de":{"name":"Hornhautdystrophie, posteriore","definition":"Die Hinteren Hornhautdystrophien umfassen eine Gruppe seltener, genetisch determinierter Hornhautdystrophien (CDs). Sie sind gekennzeichnet durch Läsionen des Hornhautendothels und der Descemet-Membran, mit je nach Typ der Dystrophie unterschiedlicher Auswirkung auf den Visus."},"es":{"name":"Distrofia corneal posterior","definition":"Las distrofias corneales posteriores designan un grupo de distrofias corneales (DCs) raras y genéticamente determinadas, caracterizadas por lesiones que afectan al endotelio corneal y a la membrana de Descemet, y por efectos variables en la visión en función del tipo de distrofia."},"it":{"name":"Distrofia corneale posteriore","definition":"Le distrofie corneali posteriori appartengono al gruppo delle distrofie rare della cornea (CD), geneticamente determinate, caratterizzate da lesioni che interessano l'endotelio della cornea e la membrana di Descemet, che comportano varie conseguenze sulla vista, in rapporto al tipo di distrofia."},"nl":{"name":"Posterieure corneadystrofie","definition":"Posterieure corneadystrofieën verwijzen naar een groep zeldzame genetisch bepaalde corneadystrofieën (CD's), gekenmerkt door letsels van het cornea-endotheel en Descemetmembraan, en met wisselende effecten op het gezichtsvermogen, afhankelijk van het type dystrofie."},"pt":{"name":"Distrofia da córnea posterior"},"pl":{"name":"Dystrofia tylna rogówki"},"cs":{"name":"Zadní korneální dystrofie"},"uk":{"name":"Задня дистрофія рогівки","definition":"Posterior corneal dystrophies refers to a group of rare genetically determined corneal dystrophies (CDs) characterized by lesions affecting the corneal endothelium and Descemet membrane, and variable effects on vision depending on the type of dystrophy."},"zh":{"name":"后角膜营养不良","definition":"角膜后部营养不良是指一组罕见的由基因决定的角膜营养不良(CDs)，角膜内皮和后弹力膜受损，根据营养不良的类型会对视力产生不同的影响。"},"tr":{"name":"Posterior kornea distrofisi"}},"xrefs":[{"source":"MONDO","code":"0020214","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2063478","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":34533,"name":"Corneal dystrophy","classifications":[189]},{"orpha":522560,"name":"Genetic corneal dystrophy","classifications":[156]}],"children":[{"orpha":293603,"name":"Congenital hereditary endothelial dystrophy type II","type":"Disease"},{"orpha":98974,"name":"Fuchs endothelial corneal dystrophy","type":"Disease"},{"orpha":98973,"name":"Posterior polymorphous corneal dystrophy","type":"Disease"},{"orpha":293621,"name":"X-linked endothelial corneal dystrophy","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Autosomal recessive","X-linked recessive"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}