{"orpha":67039,"name":"Segmental odontomaxillary dysplasia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare oral disease characterized by unilateral enlargement of the right or left maxillary alveolar bone and gingiva in the region distal to the canines towards the maxillary tuberosity. In the enlarged region, dental abnormalities such as missing teeth, abnormal spacing and delayed eruption occur.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-67039/","orphanet_url":"https://www.orpha.net/en/disease/detail/67039","names":{"fr":{"name":"Dysplasie odonto-maxillaire segmentaire","definition":"Maladie odontologique rare caractérisée par une hypertrophie unilatérale de l'os alvéolaire maxillaire droit ou gauche et de la gencive, dans la région allant de l'arrière des canines à la tubérosité maxillaire. Des anomalies dentaires sont observées dans la région présentant l'hypertrophie, telles que des dents manquantes, anormalement espacées ou d'éruption tardive."},"de":{"name":"Dysplasie, odonto-maxilläre segmentale","definition":"Eine seltene orale Erkrankung, die durch eine einseitige Vergrößerung des rechten oder linken Oberkiefer-Alveolarknochens und der Gingiva in der Region distal der Eckzähne in Richtung der Oberkiefertuberositas gekennzeichnet ist. In der vergrößerten Region kommt es zu Zahnanomalien wie fehlenden Zähnen, abnormalen Zahnabständen und verzögertem Durchbruch."},"es":{"name":"Displasia odontomaxilar segmentaria","definition":"Es una enfermedad poco frecuente de la cavidad oral caracterizada por un agrandamiento unilateral del hueso alveolar superior derecho o izquierdo y del tejido gingival en la región distal a los caninos hacia la tuberosidad maxilar. En la región agrandada, se producen anomalías dentarias, como dientes faltantes, mayores espacios interdentales y retraso de la erupción."},"it":{"name":"Displasia odontomascellare segmentale"},"nl":{"name":"Segmentale odontomaxillaire dysplasie","synonyms":["SOD"],"definition":"Een zeldzame orale aandoening, gekenmerkt door unilaterale vergroting van linker of rechter maxillair alveolair bot en gingiva distaal van de hoektanden en in de richting van de tuber van maxilla. In het gebied van de vergroting komen dentale afwijkingen voor, zoals ontbrekende tanden, abnormale tussenruimtes tussen tanden en vertraagde eruptie."},"pt":{"name":"Displasia odontomaxilar segmentar","synonyms":["DOS"],"definition":"Doença oral rara caracterizada pelo aumento unilateral do osso alveolar maxilar direito ou esquerdo e gengiva na região distal aos caninos em direção à tuberosidade maxilar. Na região aumentada, ocorrem anomalias dentárias, como dentes ausentes, espaçamento anómalo e erupção tardia."},"pl":{"name":"Segmentalna dysplazja zębowo-szczękowa","synonyms":["SOD"]},"cs":{"name":"Segmentální odontomaxilární dysplázie","synonyms":["SOD"]},"uk":{"name":"Сегментарна одонтомаксилярна дисплазія","definition":"A rare oral disease characterized by unilateral enlargement of the right or left maxillary alveolar bone and gingiva in the region distal to the canines towards the maxillary tuberosity. In the enlarged region, dental abnormalities such as missing teeth, abnormal spacing and delayed eruption occur."},"zh":{"name":"节段性牙颌骨异型增生"},"tr":{"name":"Segmental odontomaksiller displazi"}},"xrefs":[{"source":"ICD-10","code":"K00.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DA07.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1309035766"},{"source":"MONDO","code":"0019029","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3698531","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":32.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}