{"orpha":435372,"name":"Anterior urethral valve","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, congenital, fetal lower urinary tract obstruction (LUTO) anomaly occurring in males and characterized by a posteriorly directed semilunar fold arising from the floor of the anterior urethra and causing urethral obstruction during micturition. The valves may be located anywhere distal to the membranous urethra. Clinical presentation is highly variable, depending on age and degree of urinary obstruction, and includes urinary incontinence, urinary retention, weak urinary stream, post-micturitional dribbling, bulging on the ventral penis, urinary tract infection, and urosepsis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-435372/","orphanet_url":"https://www.orpha.net/en/disease/detail/435372","names":{"fr":{"name":"Valve de l'urètre antérieur","definition":"Anomalie congénitale rare consistant en l'obstruction du bas appareil urinaire (LUTO pour lower urinary tract obstruction) chez le foetus, affectant les individus de sexe masculin et caractérisée par une encoche en forme de demi-lune siégeant sur le versant de l'urètre antérieur et provoquant une obstruction urétrale pendant la miction. Les valves peuvent être situées à n'importe quel endroit distal de l'urètre membraneux. Le tableau clinique est très variable, fonction de l'âge et du degré d'obstruction urinaire, et comprend l'incontinence et la rétention urinaire, un faible jet urinaire, un écoulement d'urine après miction (dit post-micturitional dribbling), un renflement situé sur la surface ventrale du pénis, l'infection des voies urinaires et l'urosepsie."},"de":{"name":"Anteriore Urethralklappen","definition":"Eine seltene kongenitale fetale Obstruktion der unteren Harnwege (LUTO), die bei Männern auftritt und durch eine nach hinten gerichtete semilunare Falte gekennzeichnet ist, die vom Boden der vorderen Harnröhre ausgeht und eine Harnröhrenobstruktion während der Miktion verursacht. Die Harnröhrenklappen können sich überall distal der membranösen Harnröhre befinden. Das klinische Erscheinungsbild ist je nach Alter und Ausmaß der Harnwegsobstruktion sehr unterschiedlich und umfasst Harninkontinenz, Harnverhalt, schwachen Harnstrahl, Nachtröpfeln nach der Miktion, Ausbuchtungen am ventralen Penis, Harnwegsinfektionen und Urosepsis."},"es":{"name":"Válvula uretral anterior","definition":"Es una anomalía congénita, poco frecuente, de obstrucción fetal del tracto urinario inferior (LUTO, por sus siglas en inglés), que ocurre en varones. Está caracterizada por un pliegue semilunar orientado posteriormente que surge de la base de la uretra anterior, causando obstrucción uretral durante la micción. Las válvulas pueden estar localizadas en cualquier lugar distal a la uretra membranosa. La presentación clínica es muy variable, dependiendo de la edad y del grado de obstrucción urinaria, e incluye incontinencia urinaria, retención urinaria, flujo urinario débil, goteo post-miccional, abombamiento ventral del pene, infección del tracto urinario y urosepsis."},"it":{"name":"Valvola uretrale anteriore","definition":"La valvola uretrale anteriore (AUV) è un'ostruzione congenita rara delle basse vie urinarie (LUTO) del feto, che colpisce i maschi ed è caratterizzata da una plica a forma di semiluna orientata posteriormente, che origina dal pavimento dell'uretra anteriore e ne causa l'ostruzione durante la minzione. Le valvole possono essere posizionate in qualsiasi posizione distale rispetto all'uretra membranosa. Il quadro clinico è molto variabile e dipende dell'età e dal grado dell'ostruzione urinaria; comprende l'incontinenza urinaria, la ritenzione urinaria, il flusso urinario debole, il gocciolamento post-minzionale, il gonfiore localizzato sulla porzione ventrale del pene, le infezioni delle vie urinarie e l'urosepsi."},"nl":{"name":"Anterieure urethraklep","definition":"Een zeldzame congenitale foetale obstructie van lagere urinewegen (LUTO) die voorkomt bij mannen, gekarakteriseerd door een posterieur gerichte, halvemaanvormige plooi die ontstaat uit de bodem van anterieure urethra en die obstructie van urethra tijdens mictie veroorzaakt. De kleppen kunnen op eender welke plaats distaal van de membraneuze urethra gesitueerd zijn. Klinische presentatie is heel variabel, afhankelijk van leeftijd en mate van obstructie van urinewegen, en omvat urinaire incontinentie, urineretentie, zwakke urinestraal, nadruppelen van urine, uitpuiling op ventrale penis, urineweginfectie, en urosepsis."},"pt":{"name":"Válvula uretral anterior"},"pl":{"name":"Zastawka przedniej cewki moczowej"},"cs":{"name":"Chlopeň přední uretry"},"uk":{"name":"Передній уретральний клапан","definition":"A rare, congenital, fetal lower urinary tract obstruction (LUTO) anomaly occurring in males and characterized by a posteriorly directed semilunar fold arising from the floor of the anterior urethra and causing urethral obstruction during micturition. The valves may be located anywhere distal to the membranous urethra. Clinical presentation is highly variable, depending on age and degree of urinary obstruction, and includes urinary incontinence, urinary retention, weak urinary stream, post-micturitional dribbling, bulging on the ventral penis, urinary tract infection, and urosepsis."},"zh":{"name":"前尿道瓣膜"},"tr":{"name":"Anterior üretral kapak"}},"xrefs":[{"source":"ICD-10","code":"Q64.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LB31.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1037620137"},{"source":"MONDO","code":"0018560","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0431755","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":435365,"name":"Fetal lower urinary tract obstruction","classifications":[147,156,185,188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Antenatal","Childhood","Infancy","Neonatal"],"prevalence":[],"phenotypes":null}