{"orpha":98958,"name":"Climatic droplet keratopathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Honey-droplet corneal dystrophy"],"definition":"A rare superficial corneal dystrophy characterized by progressive opacity of the most anterior corneal layers. Slit-lamp examination reveals typical confluent translucent subepithelial deposits, extending in size and growing into clusters of golden droplets covering the cornea with disease progression. Patients present variably compromised visual acuity, depending on the stage of the disease. In advanced stages, decreased corneal sensation may lead to corneal trophic changes, perforation, and permanent visual loss.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98958/","orphanet_url":"https://www.orpha.net/en/disease/detail/98958","names":{"fr":{"name":"Kératopathie climatique en gouttelettes","synonyms":["Dystrophie cornéenne sphéroïdale en bandelettes"],"definition":"Dystrophie cornéenne superficielle rare caractérisée par une opacité progressive des couches cornéennes les plus antérieures. L'examen à la lampe à fente met en évidence des dépôts sous-épithéliaux translucides confluents typiques, qui s'étendent pour former des grappes composées de gouttelettes dorées recouvrant la cornée au fur et à mesure de la progression de la maladie. L'acuité visuelle des patients est plus ou moins compromise suivant le stade de la maladie. À un stade avancé, la diminution de la sensibilité cornéenne peut entraîner des troubles trophiques de la cornée, une perforation et une perte visuelle permanente."},"de":{"name":"Tröpfchenkeratopathie, klimatisch bedingte","synonyms":["Labrador-Keratopathie"],"definition":"Eine seltene oberflächliche Hornhautdystrophie, die durch eine fortschreitende Trübung der vordersten Hornhautschichten gekennzeichnet ist. Bei der Spaltlampenuntersuchung sind typische konfluierende, durchscheinende subepitheliale Ablagerungen zu erkennen, die mit dem Fortschreiten der Erkrankung größer werden und sich zu Ansammlungen von goldenen Tröpfchen entwickeln, die die Hornhaut bedecken. Die Sehschärfe der Patienten ist je nach Stadium der Erkrankung unterschiedlich stark beeinträchtigt. In fortgeschrittenen Stadien kann eine verminderte Hornhautsensibilität zu trophischen Veränderungen der Hornhaut, Perforation und permanentem Sehverlust führen."},"es":{"name":"Queratopatía climática en gotas","synonyms":["Distrofia corneal en \"gotas de miel\""],"definition":"Es una distrofia corneal superficial poco frecuente caracterizada por opacidad progresiva de las capas corneales más superficiales. El examen con lámpara de hendidura revela típicamente depósitos subepiteliales translúcidos de crecimiento confluente que aumentan de tamaño y se agrupan en gotas de color dorado que cubren la córnea con la progresión de la enfermedad. Los pacientes presentan una afectación variable de la agudeza visual, dependiendo de la etapa de la enfermedad. En estadios avanzados, la disminución de la sensación corneal puede derivar en cambios tróficos corneales, perforación y pérdida visual permanente."},"it":{"name":"Cheratopatia climatica a goccia","definition":"Si tratta di una distrofia superficiale rara della cornea, caratterizzata da opacità progressiva degli strati anteriori della cornea. L'esame con la lampada a fessura evidenzia caratteristici depositi subepiteliali traslucidi confluenti, che aumentano di dimensione e si raggruppano sotto forma di gocce dorate che, con la progressione della malattia, ricoprono la cornea. A seconda dello stadio della malattia, i pazienti presentano una compromissione variabile dell'acuità visiva. Negli stati più avanzati, la riduzione della sensibilità corneale può comportare alterazioni trofiche della cornea, perforazione e cecità permanente."},"nl":{"name":"Klimatologische druppelvormige keratopathie","synonyms":["Druppelvormige corneadystrofie"],"definition":"Een zeldzame, oppervlakkige corneadystrofie, gekarakteriseerd door progressieve opaciteit van de voorste lagen van het hoornvlies. Spleetlamponderzoek toont typische confluerende, translucente subepitheliale afzettingen die groter worden en uitgroeien tot clusters van goudkleurige druppels die het hoornvlies bedekken naarmate de ziekte vordert. Patiënten presenteren zich met variabel aangetaste gezichtsscherpte, afhankelijk van het stadium van de ziekte. In vergevorderde stadia kan verminderde corneale gevoeligheid leiden tot trofische veranderingen en perforatie van hoornvlies, en permanent verlies van gezichtsvermogen."},"pt":{"name":"Distrofia corneana, gota de mel"},"pl":{"name":"Keratopatia klimatyczna kropelkowa","synonyms":["Honey-droplet corneal dystrophy"]},"cs":{"name":"Klimatická kapičková keratopatie","synonyms":["Honey-droplet corneal dystrophy"]},"uk":{"name":"Кліматична крапельна кератопатія","synonyms":["Медова краплинна дистрофія рогівки"],"definition":"A rare superficial corneal dystrophy characterized by progressive opacity of the most anterior corneal layers. Slit-lamp examination reveals typical confluent translucent subepithelial deposits, extending in size and growing into clusters of golden droplets covering the cornea with disease progression. Patients present variably compromised visual acuity, depending on the stage of the disease. In advanced stages, decreased corneal sensation may lead to corneal trophic changes, perforation, and permanent visual loss."},"zh":{"name":"气候性滴状角膜病变","synonyms":["蜜滴状角膜营养不良"],"definition":"该病是一种罕见的浅表性角膜营养不良，其特征是最前面的角膜层逐渐浑浊。裂隙灯检查显示典型的融合性半透明上皮下沉积，随着疾病的进展，其大小不断扩大，并生长成覆盖角膜的金色小滴簇。在疾病的不同阶段，患者会出现不同程度的视力下降。晚期，角膜感知下降可能导致角膜营养改变、穿孔和永久性视力丧失。"},"tr":{"name":"İklimsel damlacık keratopatisi","synonyms":["Bal damlacığı kornea distrofisi"]}},"xrefs":[{"source":"ICD-10","code":"H18.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9A70.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"896313309"},{"source":"MONDO","code":"0020363","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0339300","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":98625,"name":"Superficial corneal dystrophy","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}