{"orpha":98626,"name":"Stromal corneal dystrophy","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"The stromal corneal dystrophies refer to a group of rare genetically determined corneal dystrophies (CDs) characterized by lesions affecting the corneal stroma, and variable effects on vision depending on the type of dystrophy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98626/","orphanet_url":"https://www.orpha.net/en/disease/detail/98626","names":{"fr":{"name":"Dystrophie cornéenne stromale","definition":"Les dystrophies cornéennes stromales constituent un groupe de dystrophies cornéennes d'origine génétique caractérisé par des lésions touchant le stroma cornéen, avec des effets variables sur la vision selon le type de dystrophie."},"de":{"name":"Hornhautdystrophie, stromale","synonyms":["Stroma-Hornhautdystrophie, kongenitale"],"definition":"Als Stroma-Hornhautdystrophien wird eine Gruppe von seltenen, genetisch determinierten Hornhautdystrophien (CDs) mit Läsionen im Hornhautstroma und mit je nach Dystrophietyp unterschiedlichen Auswirkungen auf den Visus bezeichnet"},"es":{"name":"Distrofia corneal estromal","definition":"Las distrofias corneales estromales representan un grupo de distrofias corneales (DCs) raras genéticamente determinadas caracterizadas por lesiones que afectan al estroma corneal, y efectos varibales en la visión dependiendo del tipo de distrofia."},"it":{"name":"Distrofia corneale stromale","definition":"Le distrofie corneali stromali appartengono al gruppo delle distrofie corneali (CD) rare determinate geneticamente, caratterizzate da lesioni che interessano lo stroma corneale, con conseguenze variabili sulla vista, correlate al tipo di distrofia."},"nl":{"name":"Stromale corneadystrofie","synonyms":["Stromale hoornvliesdystrofie"],"definition":"De stromale corneadystrofieën verwijzen naar een groep zeldzame genetisch bepaalde corneadystrofieën (CD's), gekenmerkt door letsels die het corneastroma aantasten, en met wisselende effecten op het gezichtsvermogen afhankelijk van het type dystrofie."},"pt":{"name":"Distrofia do estroma da córnea"},"pl":{"name":"Dystrofia stromalna rogówki","definition":"Dystrofie stromalne rogówki odnoszą się do grupy rzadkich, genetycznie określonych dystrofii rogówki (CD), charakteryzujących się uszkodzeniami stromy rogówki oraz różną ekspresją w zależności od rodzaju dystrofii."},"cs":{"name":"Stromální dystrofie rohovky"},"uk":{"name":"Стромальна дистрофія рогівки","definition":"The stromal corneal dystrophies refer to a group of rare genetically determined corneal dystrophies (CDs) characterized by lesions affecting the corneal stroma, and variable effects on vision depending on the type of dystrophy."},"zh":{"name":"基质角膜营养不良","definition":"基质角膜营养不良是指一组罕见的由基因决定的角膜营养不良(CDs)，病变影响角膜基质，根据营养不良的类型对视力会产生不同的影响。"},"tr":{"name":"Stromal kornea distrofisi"}},"xrefs":[{"source":"MONDO","code":"0020213","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0038457","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":98972,"name":"Central cloudy dystrophy of François","type":"Disease"},{"orpha":101068,"name":"Congenital stromal corneal dystrophy","type":"Disease"},{"orpha":98970,"name":"Fleck corneal dystrophy","type":"Disease"},{"orpha":98962,"name":"Granular corneal dystrophy type I","type":"Disease"},{"orpha":98963,"name":"Granular corneal dystrophy type II","type":"Disease"},{"orpha":98964,"name":"Lattice corneal dystrophy type I","type":"Disease"},{"orpha":98969,"name":"Macular corneal dystrophy","type":"Disease"},{"orpha":98971,"name":"Posterior amorphous corneal dystrophy","type":"Disease"},{"orpha":293462,"name":"Pre-Descemet corneal dystrophy","type":"Disease"},{"orpha":98967,"name":"Schnyder corneal dystrophy","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Autosomal recessive"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}