{"orpha":83451,"name":"Florid cemento-osseous dysplasia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Florid osseous dysplasia","Focal cemento-osseous dysplasia"],"definition":"Florid cemento-osseous dysplasia (FCOD) is a rare fibro-osseous lesion in the jaw that predominantly affects middle-aged women of African descent. It is generally asymptomatic or may manifest with pain and gingival swelling. Radiologically, it is characterized by multiple dense lobulated bone lesions, often symmetrically located in various regions of the jaw.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83451/","orphanet_url":"https://www.orpha.net/en/disease/detail/83451","names":{"fr":{"name":"Dysplasie cémento-osseuse floride","synonyms":["Dysplasie cémento-osseuse focale","Dysplasie osseuse floride"],"definition":"La dysplasie cémento-osseuse floride est une lésion fibro-osseuse rare de la mâchoire qui affecte surtout les femmes d'âge moyen originaires d'Afrique. Elle est généralement asymptomatique ou peut se manifester par une douleur et un gonflement gingival. Au niveau radiologique, elle se caractérise par de multiples lésions osseuses denses et lobulées, souvent situées de manière symétrique dans plusieurs régions de la mâchoire."},"de":{"name":"Floride Knochenzement-Dysplasie","synonyms":["Dysplasie, floride ossäre"],"definition":"Die Floride Knochenzement-Dysplasie (FCOD) ist eine seltene fibro-ossäre Läsion in Kiefer, die überwiegend bei Frauen mittleren Alters mit afrikanischer Herkunft gesehen wird. Sie bleibt in der Regel symptomfrei oder manifestiert sich mit Schmerzen und Gingivaschwellung. Der radiologische Befund ist gekennzeichnet durch multiple dichte, gelappte Knochenläsionen in verschiedenen Bereichen des Kiefers, oft mit symmetrischer Anordnung."},"es":{"name":"Displasia cemento-ósea florida","synonyms":["Displasia cemento-ósea focal","Displasia florida ósea","Displasia ósea florida"],"definition":"La displasia cemento-ósea florida (FCOD) es una lesión fibro-ósea en la mandíbula poco frecuente que predominantemente afecta a mujeres de edad media de descendencia africana. Generalmente es asintomática o puede manifestarse con dolor e inflamación gingival. Radiológicamente se caracteriza por múltiples lesiones óseas lobuladas densas, a menudo localizadas de manera simétrica en varias regiones de la mandíbula."},"it":{"name":"Displasia florida cemento-ossea","synonyms":["Displasia florida ossea","Displasia focale cemento-ossea"],"definition":"La displasia cemento-ossea florida (FCOD) è una lesione fibro-ossea della mascella, che colpisce prevalentemente le donne di mezza età, di origine africana. È in genere asintomatica o può presentarsi con gonfiore gengivale e dolore. Dal punto di vista radiologico, è caratterizzata da lesioni ossee lobulate dense, spesso localizzate simmetricamente in diverse aree della mascella."},"nl":{"name":"Floride cemento-osseuze dysplasie","synonyms":["Floride osseuze dysplasie","Focale cemento-osseuze dysplasie"],"definition":"Floride cemento-osseuze dysplasie (FCOD) is een zeldzaam fibro-osseus letsel aan de kaak dat voornamelijk voorkomt in vrouwen van middelbare leeftijd en Afrikaanse origine. Doorgaans is FCOD asymptomatisch, of het kan tot uiting komen door pijn en zwelling van het tandvlees. Radiologisch wordt de aandoening gekenmerkt door meerdere gelobde letsels aan het bot die vaak symmetrisch zijn gelokaliseerd in verschillende regio's van de kaak."},"pt":{"name":"Displasia cemento-óssea florida","synonyms":["Displasia cemento-óssea focal","Displasia óssea florida"]},"pl":{"name":"Rozsiana dysplazja cementowo-kostna","synonyms":["Ogniskowa dysplazja cementowo-kostna"]},"cs":{"name":"Floridní cementokostní dysplázie","synonyms":["Floridní kostní dysplázie","Fokální cementokostní dysplázie"]},"uk":{"name":"Флористична цементно-кісткова дисплазія","synonyms":["Флорідна кісткова дисплазія","Фокальна цементно-кісткова дисплазія"],"definition":"Florid cemento-osseous dysplasia (FCOD) is a rare fibro-osseous lesion in the jaw that predominantly affects middle-aged women of African descent. It is generally asymptomatic or may manifest with pain and gingival swelling. Radiologically, it is characterized by multiple dense lobulated bone lesions, often symmetrically located in various regions of the jaw."},"zh":{"name":"菜花样牙骨质-骨化不全","synonyms":["局灶性牙骨质-骨结构不良"]},"tr":{"name":"Florid semento-osseoz displazisi","synonyms":["Florid kemik displazisi","Fokal semento-kemik displazisi"]}},"xrefs":[{"source":"ICD-10","code":"D16.4","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-10","code":"D16.5","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"DA07.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2031259585"},{"source":"MONDO","code":"0019368","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C537063","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0555197","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10173","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":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-10-05","source":"31942128[PMID]_28194495[PMID]_32315206[PMID]","terms":[{"hpo":"HP:0003330","label":"Abnormal bone structure","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100671","label":"Abnormal trabecular bone morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100717","label":"Abnormality of the cementum","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000277","label":"Abnormality of the mandible","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000326","label":"Abnormality of the maxilla","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000309","label":"Abnormal midface morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0430028","label":"Hyperplasia of the maxilla","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001571","label":"Multiple impacted teeth","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000700","label":"Periapical bone loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006483","label":"Abnormal number of teeth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006481","label":"Abnormality of primary teeth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006308","label":"Atrophy of alveolar ridges","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010885","label":"Avascular necrosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031035","label":"Chronic infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000689","label":"Dental malocclusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011968","label":"Feeding difficulties","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030793","label":"Jaw swelling","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007626","label":"Mandibular osteomyelitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012065","label":"Multiple bony cystic lesions","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009088","label":"Speech articulation difficulties","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011069","label":"Supernumerary tooth","frequency":"Occasional (29-5%)","criteria":null}]}}