{"orpha":2456,"name":"Familial supernumerary nipples","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Isolated polythelia"],"definition":"Familial supernumerary nipples is a rare breast malformation characterized by the presence, in various members of a single family, of one or more nipple(s) and/or their related tissue, in addition to the normal bilateral chest nipples. The anomaly is usually situated along the embryonic milk line, from axillae to inguinal regions, but other locations are also possible. Association with dental abnormalities, Becker nevus, renal or underlying breast tissue malignancy and genitourinary malformations has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2456/","orphanet_url":"https://www.orpha.net/en/disease/detail/2456","names":{"fr":{"name":"Mamelon surnuméraire héréditaire","synonyms":["Polythélie isolée"],"definition":"Une malformation mammaire rare caractérisée par la présence chez plusieurs membres d'une même famille, d'un ou plusieurs mamelon(s) et/ou leurs tissus associés, en plus des mamelons bilatéraux thoraciques normaux. L'anomalie se situe habituellement le long de la ligne de lait embryonnaire, depuis la région axillaire à la région inguinale, mais d'autres localisations sont aussi possibles. Elle peut être associée à des anomalies dentaires, un nævus de Becker, une tumeur rénale ou affectant le tissu mammaire sous-jacent, et des malformations génito-urinaires."},"de":{"name":"Mamillen, überzählige, familiäre Form","synonyms":["Polythelie, isolierte"],"definition":"Eine seltene Brustfehlbildung, die durch das Vorhandensein einer oder mehrerer Brustwarze(n) und/oder ihres zugehörigen Gewebes bei verschiedenen Mitgliedern einer Familie zusätzlich zu den normalen bilateralen Brustwarzen gekennzeichnet ist. Die Anomalie befindet sich normalerweise entlang der embryonalen Milchlinie, von den Achselhöhlen bis zu den Leisten, aber auch andere Lokalisationen sind möglich. Eine Assoziation mit Zahn- Anomalien, Becker-Naevus, Malignomen des Nieren- oder darunter liegenden Brustgewebes sowie urogenitale Fehlbildungen wurde ebenfalls berichtet."},"es":{"name":"Pezones supernumerarios familiares","synonyms":["Politelia aislada"],"definition":"Los pezones supernumerarios familiares, son una malformación mamaria poco frecuente caracterizada por la presencia, en varios miembros de una familia, de uno o más pezones y/o su tejido relacionado, además de los pezones torácicos bilaterales normales. La anomalía suele situarse a lo largo de la línea láctea embrionaria, desde las axilas hasta las regiones inguinales, pero también son posibles otras ubicaciones. Se ha descrito una asociación con anomalías dentales, nevo de Becker, neoplasia renal o del tejido mamario subyacente y malformaciones genitourinarias."},"it":{"name":"Capezzoli soprannumerari familiari","definition":"I capezzoli soprannumerari familiari sono una malformazione rara della mammella, caratterizzata dalla presenza, in più membri di una famiglia, di uno o più capezzoli e/o del relativo tessuto, in aggiunta ai normali capezzoli mammari bilaterali. Di solito l'anomalia si localizza lungo la linea embrionale del latte, dall'ascella alla regione inguinale, ma sono possibili altre localizzazioni. È stata documentata un'associazione con le anomalie dei denti, con il nevo di Becker, con i tumori dei tessuti renali o il tessuto mammario sottostante e con le malformazioni genito-urinarie."},"nl":{"name":"Familiale surnumeraire tepels","synonyms":["Geïsoleerde polythelie"],"definition":"Familiale boventallige tepels is een zeldzame borstmalformatie die gekarakteriseerd wordt door de aanwezigheid, bij verschillende leden van een enkele familie, van een of meer tepels en/of gerelateerd weefsel bovenop de normale bilaterale tepels op de borst. De anomalie situeert zich meestal langs de embryonale melklijn, van de oksels tot de liesstreek, maar andere locaties zijn ook mogelijk. Associatie met dentale afwijkingen, naevus van Becker, maligniteiten van nierweefsel of onderliggend borstweefsel, en genito-urinaire malformaties werd ook reeds gerapporteerd."},"pt":{"name":"Mamilos supranumerários familiar","synonyms":["Politelia familiar"]},"pl":{"name":"Rodzinne dodatkowe gruczoły sutkowe","synonyms":["Politelia izolowana"]},"cs":{"name":"Nadpočetná bradavka","synonyms":["Izolovaná polytelie"]},"uk":{"name":"Сімейні надлишкові соски","synonyms":["Ізольована політелія"],"definition":"Familial supernumerary nipples is a rare breast malformation characterized by the presence, in various members of a single family, of one or more nipple(s) and/or their related tissue, in addition to the normal bilateral chest nipples. The anomaly is usually situated along the embryonic milk line, from axillae to inguinal regions, but other locations are also possible. Association with dental abnormalities, Becker nevus, renal or underlying breast tissue malignancy and genitourinary malformations has been reported."},"zh":{"name":"家族遗传性多乳头","synonyms":["孤立型多乳头畸形"]},"tr":{"name":"Ailesel süpernümerik meme uçları","synonyms":["İzole politeli"]}},"xrefs":[{"source":"OMIM","code":"163700","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"Q83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB63","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0008101","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707878","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2259","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":96344,"name":"Rare gynecologic or obstetric disease"},"parents":[{"orpha":180170,"name":"Excess breast volume or number","classifications":[205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":46.0,"area":"Worldwide","status":"Validated","source":"9809822[PMID]_1638072[PMID]_28361071[PMID]_20465693[PMID]_8603338[PMID]_11421419[PMID]_12514363[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"9809822[PMID]_1638072[PMID]_28361071[PMID]_20465693[PMID]_8603338[PMID]_11421419[PMID]_12514363[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0002558","label":"Supernumerary nipple","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000119","label":"Abnormality of the genitourinary system","frequency":"Occasional (29-5%)","criteria":null}]}}