{"orpha":238637,"name":"Megacystis-megaureter syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Megaureter-megacystis syndrome"],"definition":"Megacystic-megaureter syndrome is an urinary tract malformation characterized by the presence of a massive primary non-obstructive vesicoureteral reflux and a large capacity, smooth, thin walled bladder due to the continual recycling of refluxed urine. Recurrent urinary infections are commonly associated with this condition.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-238637/","orphanet_url":"https://www.orpha.net/en/disease/detail/238637","names":{"fr":{"name":"Syndrome mégavessie-méga-uretères","synonyms":["Syndrome méga-uretères-mégavéssie"],"definition":"Le syndrome mégavessie-méga-uretère est une malformation de l'appareil urinaire caractérisée par la présence d'un reflux massif vésico-urétéral primitif, non-obstructif, et d'une vessie à parois fines et lisses, de grande capacité, dû au recyclage continuel de l'urine refluée. Des infections urinaires récurrentes sont fréquemment associées à cette condition."},"de":{"name":"Megazystis-Megaureter-Syndrom","synonyms":["Megaureter-Megazystis-Syndrom"],"definition":"Das Megazystis-Megaureter-Syndrom beschreibt das Vorhandensein eines massiven primären nicht-obstruktiven vesikoureteralen Refluxes mit einer großvolumigen, dünnwandigen, weichen Harnblase. Ursache ist das kontinuierliche Recycling des zurückgeflossen Urins. In der Regel sind mit dieser Störung rezidivierende Harnwegsinfekte assoziiert."},"es":{"name":"Síndrome megavejiga-megauréter","synonyms":["Síndrome megauréter-megavejiga"],"definition":"El síndrome megavejiga-megauréter describe la presencia de un reflujo vesicoureteral masivo primario no obstructivo, y una vejiga de gran capacidad, lisa, de pared delgada, debido al reciclaje continuo del reflujo de orina. Las infecciones urinarias recurrentes se suelen asociar con esta enfermedad."},"it":{"name":"Sindrome megacisti-megauretere","synonyms":["Sindrome megauretere-megacisti"],"definition":"La sindrome megacisti-megauretere è caratterizzata da un importante reflusso vescico-ureterale non ostruttivo primitivo e da una vescica di capacità abnormemente aumentata, con pareti lisce e sottili a causa del continuo riciclaggio dell'urina che refluisce. La malattia si associa spesso alle infezioni urinarie ricorrenti."},"nl":{"name":"Megacystis - mega-ureter-syndroom","synonyms":["Mega-ureter - megacystis-syndroom"],"definition":"Een malformatie van urinewegstelsel, gekenmerkt door de aanwezigheid van ernstige primaire niet-obstructieve vesico-ureterale reflux en een blaas met grote capaciteit en gladde, dunne wand als gevolg van continue recirculatie van teruggevloeide urine. Doorgaans zijn recidiverende infecties van urinewegen geassocieerd met deze aandoening."},"pt":{"name":"Síndrome megabexiga-megaureter"},"pl":{"name":"Zespół olbrzymiego pęcherza moczowego i moczowodów olbrzymich","synonyms":["Zespół moczowodów olbrzymich i olbrzymiego pęcherza moczowego"]},"cs":{"name":"Syndrom zahrnující megacystis a megaureter","synonyms":["Syndrom zahrnující megaureter a megacystis"]},"uk":{"name":"Синдром мегацистис-мегауретер","synonyms":["Синдром мегауретера-мегациститу"],"definition":"Megacystic-megaureter syndrome is an urinary tract malformation characterized by the presence of a massive primary non-obstructive vesicoureteral reflux and a large capacity, smooth, thin walled bladder due to the continual recycling of refluxed urine. Recurrent urinary infections are commonly associated with this condition."},"zh":{"name":"巨膀胱-巨输尿管综合征","synonyms":["巨输尿管-巨膀胱综合征"],"definition":"巨输尿管-巨膀胱综合征是一种泌尿系统畸形，其特征是存在大量原发性非梗阻性膀胱输尿管反流和由于尿液不断循环回流所致的容量大的，光滑的，薄壁的膀胱。通常与复发性尿路感染相关。"},"tr":{"name":"Megasitis-megaüreter sendromu","synonyms":["Megaüreter-megasist sendromu"]}},"xrefs":[{"source":"ICD-10","code":"Q62.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB31.7","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1872503107"},{"source":"MONDO","code":"0016548","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0431752","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":101433,"name":"Rare urogenital disease"},"parents":[{"orpha":93546,"name":"Non-syndromic renal or urinary tract malformation","classifications":[147,188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy","Neonatal"],"prevalence":[],"phenotypes":null}