{"orpha":83450,"name":"Regional odontodysplasia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Ghost teeth"],"definition":"A rare orodental disease characterized by localized developmental anomaly of the dental tissues, with enamel and dentin hypomineralization affecting one (odontodysplasia) or several (regional odontodysplasia; ROD) teeth (deciduous and permanent, with teeth of the maxilla more frequently involved).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83450/","orphanet_url":"https://www.orpha.net/en/disease/detail/83450","names":{"fr":{"name":"Odontodysplasie régionale","synonyms":["Dents fantômes","Dents évanescentes"],"definition":"Maladie odontologique rare caractérisée par une anomalie de développement localisée des tissus dentaires, avec hypominéralisation de l'email et de la dentine affectant une (odontodysplasie) ou plusieurs (odontodysplasie régionale ; ODR) dents (de lait et définitives ; les dents du maxillaire étant plus fréquemment touchées)."},"de":{"name":"Odontodysplasie, regionale","definition":"Eine seltene orodentale Erkrankung, die durch eine örtlich begrenzte Entwicklungsstörung des Zahngewebes mit Hypomineralisierung von Zahnschmelz und Dentin bei Milch- und/oder bleibenden Zähnen gekennzeichnet ist. Sie kann einen Zahn (Odontodysplasie) oder mehrere Zähne (regionale Odontodysplasie, ROD) umfassen. Dabei sind die Zähne des Oberkiefers häufiger betroffen."},"es":{"name":"Odontodisplasia regional","synonyms":["Diente fantasma","Dientes fantasmas"],"definition":"Es una enfermedad bucodental poco frecuente caracterizada por una anomalía localizada del desarrollo de los tejidos dentarios, con hipomineralización del esmalte y la dentina que afecta a uno (odontodisplasia) o varios (odontodisplasia regional; OR) dientes (deciduos y permanentes, con los dientes del maxilar afectados con mayor frecuencia)."},"it":{"name":"Odontodisplasia regionale","synonyms":["Denti fantasma"]},"nl":{"name":"Regionale odontodysplasie","synonyms":["Ghost teeth"],"definition":"Een zeldzame orodentale aandoening, gekenmerkt door gelokaliseerde ontwikkelingsanomalie van dentale weefsels, met hypomineralisatie van tandglazuur en dentine die één (odontodysplasie) of meerdere (regionale odontodysplasie; ROD) tanden aantast (melktanden en blijvende tanden, met vaker betrokkenheid van tanden van bovenkaak)."},"pt":{"name":"Odontodisplasia regional","synonyms":["Dentes fantasmas"],"definition":"Doença orodentária rara, caracterizada por anomalia localizada do desenvolvimento dos tecidos dentários, com hipomineralização do esmalte e da dentina afetando um (odontodisplasia) ou vários (odontodisplasia regional; ROD) dentes (decíduos e permanentes, com dentes da maxila mais frequentemente envolvidos)."},"pl":{"name":"Miejscowa odontodysplazja","synonyms":["Zęby widmo"]},"cs":{"name":"Regionální odontodysplázie","synonyms":["Ghost teeth"]},"uk":{"name":"Регіональна одонтодисплазія","synonyms":["Зуби привидів"],"definition":"A rare orodental disease characterized by localized developmental anomaly of the dental tissues, with enamel and dentin hypomineralization affecting one (odontodysplasia) or several (regional odontodysplasia; ROD) teeth (deciduous and permanent, with teeth of the maxilla more frequently involved)."},"zh":{"name":"局部牙发育不全","synonyms":["阴影牙"]},"tr":{"name":"Bölgesel odontodisplazi","synonyms":["Hayalet dişler"]}},"xrefs":[{"source":"ICD-10","code":"K00.4","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"DA07.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1516505714"},{"source":"MONDO","code":"0019367","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206554","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98026,"name":"Rare odontologic disease"},"parents":[{"orpha":164001,"name":"Rare odontal or periodontal disorder","classifications":[197]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":140.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-02-03","source":"31337932[PMID]_22762183[PMID]_30900210[PMID]","terms":[{"hpo":"HP:0011073","label":"Abnormality of dental color","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000682","label":"Abnormality of dental enamel","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010299","label":"Abnormality of dentin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006481","label":"Abnormality of primary teeth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006479","label":"Abnormality of the dental pulp","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000670","label":"Carious teeth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000684","label":"Delayed eruption of teeth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0009722","label":"Dental enamel pits","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006297","label":"Enamel hypoplasia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000230","label":"Gingivitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011084","label":"Hypocalcification of dental enamel","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040264","label":"Jaw pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000694","label":"Odontodysplasia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003771","label":"Pulp calcification","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030757","label":"Tooth abscess","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000706","label":"Unerupted tooth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006286","label":"Yellow-brown discoloration of the teeth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0009085","label":"Alveolar ridge overgrowth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000212","label":"Gingival overgrowth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000685","label":"Hypoplasia of teeth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001572","label":"Macrodontia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200025","label":"Mandibular pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006283","label":"Multiple unerupted teeth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006336","label":"Short dental roots","frequency":"Occasional (29-5%)","criteria":null}]}}