{"orpha":137917,"name":"Choanal atresia, unilateral","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, usually, sporadic congenital anomaly that is more commonly seen in females than in males (2:1), where the nose is blocked by bony or soft tissue formed during embryologic development on only one side (more commonly on the right side) and which is characterized by nasal obstruction and rhinorrhea, usually presenting at birth but that may go undetected until a respiratory infection aggravates the condition.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-137917/","orphanet_url":"https://www.orpha.net/en/disease/detail/137917","names":{"fr":{"name":"Atrésie unilatérale des choanes","synonyms":["Atrésie choanale unilatérale"],"definition":"Anomalie congénitale généralement sporadique, observée plus fréquemment chez les patients féminins que masculins (ratio 2 :1). Le nez est bloqué d'un seul côté (surtout le droit) par du tissu mou ou osseux formé au cours du développement embryologique, ce qui conduit à une obstruction nasale et une rhinorrhée qui se présentent habituellement à la naissance, mais qui peuvent passer inaperçues jusqu'à ce qu'une infection respiratoire aggrave la situation."},"de":{"name":"Choanalatresie, unilaterale","definition":"Die einseitige Choanalatresie ist eine meist sporadische angeborene Anomalie. Das weibliche Geschlecht ist häufiger betroffen (w:m = 2:1). Die Nase ist blockiert durch knöchernes oder weiches Gewebe, das sich während der embryologischen Entwicklung auf nur einer Seite (meist rechts) bildet. Der Verschluß der Nase und die seit Geburt bestehende Rhinorrhoe bleiben zunächst unbeachtet, bis eine Atemwegsinfektion die Probleme verstärkt."},"es":{"name":"Atresia de coanas unilateral","synonyms":["Atresia coanal unilateral"],"definition":"La atresia unilateral de coanas es una anomalía congénita, normalmente esporádica, que se ve con más frecuencia en mujeres que en hombres (2:1), y en la que la nariz está bloqueada por hueso o tejido blando formado durante el desarrollo embrionario en un solo lado (más frecuentemente en el lado derecho) y que se caracteriza por obstrucción nasal y rinorrea, por lo general de presentación al nacimiento pero que puede pasar inadvertida hasta que una infección respitaroria agrave la condición."},"it":{"name":"Atresia coanale unilaterale","definition":"L'atresia monolaterale delle coane è un difetto congenito, di solito sporadico, più comune nelle femmine (rapporto F/M 2:1), nel quale il naso è otturato dai tessuti molli od ossei che si formano durante lo sviluppo embrionale su un solo lato (più spesso il lato destro). È caratterizzata dall'ostruzione nasale e dalla rinorrea, che di solito è presente alla nascita, ma può restare non diagnosticata fino a quando un'infezione respiratoria non aggrava il quadro clinico."},"nl":{"name":"Choane atresie, unilaterale","definition":"Unilaterale choanale atresie is een, gewoonlijk sporadische, aangeboren afwijking die meer voorkomt bij vrouwen dan bij mannen (2:1), waarbij de neus geblokkeerd is door benig of zacht weefsel dat gevormd werd tijdens de embryologische ontwikkeling aan slechts een zijde (meestal aan de rechterkant) en die gekenmerkt is door nasale obstructie en rinorroe, wat zich gewoonlijk voordoet bij de geboorte maar ongedetecteerd kan blijven tot een luchtweginfectie de conditie verergert."},"pt":{"name":"Atresia das coanas, unilateral"},"pl":{"name":"Jednostronna atrezja nozdrzy"},"cs":{"name":"Choanální atrézie, unilaterální"},"uk":{"name":"Атрезія хоан, одностороння","definition":"A rare, usually, sporadic congenital anomaly that is more commonly seen in females than in males (2:1), where the nose is blocked by bony or soft tissue formed during embryologic development on only one side (more commonly on the right side) and which is characterized by nasal obstruction and rhinorrhea, usually presenting at birth but that may go undetected until a respiratory infection aggravates the condition."},"zh":{"name":"单侧鼻后孔闭锁","definition":"单侧鼻后孔闭塞是一种通常为偶发的先天性异常，女性比男性更常见（2:1），胚胎发育过程中形成的骨质或软组织阻塞单侧鼻部（通常为右侧），其特征是鼻塞和鼻漏，通常在出生时有表现，但可能在呼吸道感染加重病情后才能被发觉。"},"tr":{"name":"Koanal atrezi, tek taraflı"}},"xrefs":[{"source":"ICD-10","code":"Q30.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA70.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1247369366"},{"source":"MONDO","code":"0015312","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680630","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19889","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":137914,"name":"Choanal atresia","classifications":[147,200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET_EUROCAT European surveillance of congenital anomalies[OTHER]"},{"type":"Prevalence at birth","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET_EUROCAT European surveillance of congenital anomalies[OTHER]"}],"phenotypes":null}