{"orpha":180074,"name":"True unicornuate uterus","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Complete unilateral Müllerian aplasia","Complete unilateral aplasia of the Müllerian ducts","Unicornuate uterus without rudimentary horn"],"definition":"A rare, non-syndromic uterovaginal malformation characterized by a crescent-shaped, small-sized uterus containing a single horn and fallopian tube with no rudimentary horn. Urinary tract anomalies are frequently associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180074/","orphanet_url":"https://www.orpha.net/en/disease/detail/180074","names":{"fr":{"name":"Uterus unicorne vrai","synonyms":["Aplasie müllerienne unilatérale complète","Aplasie unilatérale complète des canaux de Müller","Uterus unicorne sans utérus rudimentaire"],"definition":"Une malformation utéro-vaginale rare, non syndromique, caractérisée par un utérus de petite taille en forme de croissant, contenant une seule corne utérine et une seule trompe de Fallope, sans corne utérine rudimentaire. Des anomalies des voies urinaires sont fréquemment associées."},"de":{"name":"Echter Uterus unicornis","synonyms":["Aplasie des Müller-Ganges, komplette unilaterale","Uterus unicornis ohne rudimentäres Horn"]},"es":{"name":"Útero unicorne verdadero","synonyms":["Aplasia mulleriana unilateral completa","Aplasia unilateral completa de los conductos de Müller","Útero unicorne sin cuerno rudimentario"],"definition":"Es una malformación uterovaginal no sindrómica poco frecuente caracterizada por un útero de tamaño pequeño y en forma de media luna con un solo cuerno y trompa de Falopio, sin cuerno rudimentario. Se asocian con frecuencia anomalías en el tracto urinario."},"it":{"name":"Utero unicorne vero","synonyms":["Aplasia mülleriana unilaterale completa","Aplasia unilaterale completa del dotto mülleriano","Utero unicorne senza corno rudimentale"],"definition":"L'utero unicorne vero è una malformazione utero-vaginale rara non sindromica, caratterizzata da utero di ridotte dimensioni, a forma di mezzaluna, contenente un solo corno e una sola tuba di Falloppio, in assenza di corna rudimentali. Spesso le pazienti presentano anomalie delle vie urinarie."},"nl":{"name":"Echte eenhoornige baarmoeder","synonyms":["Eenhoornige baarmoeder zonder rudimentaire hoorn","Volledige unilaterale Mülleriaanse aplasie","Volledige unilaterale aplasie van gang van Müller"],"definition":"Een zeldzame, niet-syndromale uterovaginale malformatie, gekarakteriseerd door een halvemaanvormige, kleine uterus met één enkele hoorn en eileider, en zonder een rudimentaire tweede hoorn. Anomalieën van het urinewegstelsel zijn frequent geassocieerd."},"pt":{"name":"Útero unicórneo verdadeiro"},"pl":{"name":"Prawdziwa macica jednorożna","synonyms":["Całkowita jednostronna aplazja przewodu Müllera","Macica jednorożna bez rogu szczątkowego"],"definition":"*Prawdziwa macica jednorożna to rzadka, izolowana wada macicy, która charakteryzuje się małą macicą w kształcie półksiężyca z pojedynczym rogiem i jajowodem, bez szczątkowego drugiego rogu. Często towarzyszą wady układu moczowego."},"cs":{"name":"Uterus unicornis","synonyms":["Úplná jednostranná Müllerova aplázie","Kompletní jednostranná aplázie Müllerových vývodů"]},"uk":{"name":"Справжня однорога матка","synonyms":["Повна одностороння аплазія мюллерових проток","Повна одностороння мюллерова аплазія","Однорога матка без рудиментарного рогу"],"definition":"A rare, non-syndromic uterovaginal malformation characterized by a crescent-shaped, small-sized uterus containing a single horn and fallopian tube with no rudimentary horn. Urinary tract anomalies are frequently associated."},"zh":{"name":"真性单角子宫","synonyms":["单角子宫不伴残角"],"definition":"真性单角子宫是一种罕见的非综合征性子宫输卵管畸形，其主要临床表现为偏小的新月形单角子宫和正常输卵管，常伴有泌尿道异常。"},"tr":{"name":"Gerçek unikollis uterus","synonyms":["Tam unilateral Müllerian aplazisi","İlkel boynuz ile seyretmeyen unikollis uterus","Müllerian kanallarının tam unilateral aplazisi"]}},"xrefs":[{"source":"ICD-10","code":"Q51.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB44.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"113532659"},{"source":"MONDO","code":"0015832","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5848180","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":180071,"name":"Unilateral aplasia of the Müllerian ducts","classifications":[147,156,185,205,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}