{"orpha":477781,"name":"Primary condylar hyperplasia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Type 1 condylar hyperplasia"],"definition":"A rare temporomandibular joint anomaly characterized by progressive, asymmetrical, non-neoplastic overgrowth of a mandibular condyle. It is unilateral in most cases and leads to progressive facial asymmetry, mandibular deviation, articular dysfunction, and dental malocclusion.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-477781/","orphanet_url":"https://www.orpha.net/en/disease/detail/477781","names":{"fr":{"name":"Hyperplasie condylienne primitive","synonyms":["Hyperplasie condylienne type 1"],"definition":"Anomalie rare de l'articulation temporo-mandibulaire caractérisée par un excès de croissance, qui est progressive et asymétrique, de l'un des condyles mandibulaires. L'anomalie est unilatérale dans la plupart des cas et entraîne une asymétrie faciale progressive, une déviation mandibulaire, un dysfonctionnement articulaire et une malocclusion dentaire."},"de":{"name":"Kondyläre Hyperplasie, primäre","synonyms":["Hyperplasie, kondyläre, Typ 1","Kondylushyperplasie, primäre"],"definition":"Eine seltene Kiefergelenksanomalie, die durch ein fortschreitendes, asymmetrisches, nicht-neoplastisches Überwachsen eines Kieferkondylus gekennzeichnet ist. Sie ist in den meisten Fällen einseitig und führt zu fortschreitender Gesichtsasymmetrie, Unterkieferdeviation, Gelenkdysfunktion und Zahnfehlstellungen."},"es":{"name":"Hiperplasia condilar primaria","synonyms":["Hiperplasia condilar tipo 1"],"definition":"Es una anomalía poco frecuente de la articulación temporomandibular caracterizada por un sobrecrecimiento progresivo, asimétrico y no neoplásico de un cóndilo mandibular. Es unilateral en la mayoría de los casos y conduce a asimetría facial progresiva, desviación mandibular, disfunción articular y maloclusión dental."},"it":{"name":"Iperplasia condilare primitiva","definition":"Si tratta di un'anomalia rara dell'articolazione temporo-mandibolare, caratterizzata dall'iperaccrescimento progressivo, asimmetrico e non neoplastico di un condilo mandibolare. Nella maggior parte dei pazienti è monolaterale e causa asimmetria facciale progressiva, deviazione della mandibola, disfunzione dell'articolazione e malocclusione dei denti."},"nl":{"name":"Primaire condylaire hyperplasie","synonyms":["Condylaire hyperplasie type 1"],"definition":"Een zeldzame anomalie van temporomandibulair gewricht, gekarakteriseerd door progressieve, asymmetrische, niet-neoplastische overgroei van gewrichtskop van kaakgewricht. De aandoening is in de meeste gevallen unilateraal en leidt tot progressieve asymmetrie van aangezicht, deviatie van mandibula, disfunctie van gewricht, en dentale malocclusie."},"pt":{"name":"Hiperplasia condilar primária"},"pl":{"name":"Pierwotna hiperplazja kłykcia żuchwy","synonyms":["Hiperplazja kłykcia żuchwy typu 1"]},"cs":{"name":"Primární kondylární hyperplázie","synonyms":["Type 1 condylar hyperplasia","Hyperplázie kondylů typu 1"]},"uk":{"name":"Первинна гіперплазія гострих кондилом","synonyms":["Гіперплазія кондилом 1-го типу"],"definition":"A rare temporomandibular joint anomaly characterized by progressive, asymmetrical, non-neoplastic overgrowth of a mandibular condyle. It is unilateral in most cases and leads to progressive facial asymmetry, mandibular deviation, articular dysfunction, and dental malocclusion."},"zh":{"name":"原发性踝增生","synonyms":["1型踝增生"],"definition":"一种罕见的颞下颌关节异常，其特征是下颌骨髁状突进行性、不对称、非肿瘤性过度生长。大多数情况下是单侧的，会导致渐进性面部不对称、下颌骨偏斜、关节功能障碍和牙齿畸形。"},"tr":{"name":"Primer kondiler hiperplazi","synonyms":["Kondiler hiperplazi Tip 1"]}},"xrefs":[{"source":"ICD-10","code":"K07.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018793","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5568570","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68329,"name":"Rare maxillo-facial surgical disease"},"parents":[{"orpha":210581,"name":"Temporomandibular joint anomaly","classifications":[209]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_26629479[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-05-28","source":"18778456[PMID]_19164410[PMID]_26147813[PMID]_30737324[PMID]","terms":[{"hpo":"HP:3000077","label":"Abnormal mandible condylar process morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000306","label":"Abnormality of the chin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010754","label":"Abnormality of the temporomandibular joint","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0009102","label":"Anterior open-bite malocclusion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000324","label":"Facial asymmetry","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001572","label":"Macrodontia","frequency":"Occasional (29-5%)","criteria":null}]}}