{"orpha":199293,"name":"Congenital microgastria","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Congenital microgastria is a rare malformation where the embryological development of the stomach is interrupted, leading to an abnormally small foregut in newborns and characterized by extreme feeding intolerance and malnutrition along with growth retardation and death if untreated. It is usually associated with multiple congenital anomalies.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-199293/","orphanet_url":"https://www.orpha.net/en/disease/detail/199293","names":{"fr":{"name":"Microgastrie congénitale","definition":"La microgastrie congénitale est une malformation rare où le développement embryonnaire de l'estomac est interrompu, résultant en une partie antérieure de l'intestin anormalement petite chez le nouveau-né et caractérisée par une intolérance alimentaire et une malnutrition extrême ainsi que par un retard de croissance et décès en l'absence de traitement. Elle est généralement associée à de nombreuses anomalies congénitales."},"de":{"name":"Mikrogastrie, kongenitale","definition":"Die Kongenitale Mikrogastrie ist eine seltene, durch Unterbrechung der embryologischen Entwicklung des Magens verursachte Dysplasie mit abnormem Vorderdarm. Die Neugeborenen haben eine extreme Fütter-Intoleranz, sind mangelernährt und im Wachstum retardiert. Ohne Behandlung ist der Ausgang letal. In der Regel bestehen weitere angeborene Fehlbildungen."},"es":{"name":"Microgastria congénita","definition":"La micrograstria congénita es una enfermedad displásica poco frecuente en la que el desarrollo embrionario del estómago se interrumpe, dando lugar a un intestino anterior anormalmente pequeño en recién nacidos, y que se caracteriza por una intolerancia alimentaria extrema y malnutrición, junto con retraso en el crecimiento y muerte si no es tratado. Está asociada normalmente con anomalías congénitas múltiples."},"it":{"name":"Microgastria congenita","definition":"La microgastria congenita è una condizione displastica rara nella quale si interrompe lo sviluppo embrionale dello stomaco, che esita nel ridotto sviluppo della porzione cefalica dell'intestino primitivo nei neonati. È caratterizzata da grave intolleranza alimentare e malnutrizione, associate ad un ritardo della crescita e, in assenza di trattamento, a morte precoce. Si associa di solito ad anomalie congenite multiple."},"nl":{"name":"Congenitale microgastrie","definition":"Een zeldzame malformatie door onderbreking van embryonale ontwikkeling van maag, wat leidt tot abnormaal kleine voordarm bij neonaten, gekenmerkt door extreme voedselintolerantie en malnutritie, alsook groeiretardatie en overlijden zonder behandeling. Het is meestal geassocieerd met multipele congenitale anomalieën."},"pt":{"name":"Microgastria congénita"},"pl":{"name":"Wrodzona mikrogastria"},"cs":{"name":"Vrozená mikrogastrie"},"uk":{"name":"Вроджена мікрогастрія","definition":"Congenital microgastria is a rare malformation where the embryological development of the stomach is interrupted, leading to an abnormally small foregut in newborns and characterized by extreme feeding intolerance and malnutrition along with growth retardation and death if untreated. It is usually associated with multiple congenital anomalies."},"zh":{"name":"先天性小胃","definition":"先天性小胃症是一种罕见的畸形，由于胚胎期胃发育停止所致，其主要临床表现为新生儿前肠异常小，出现极度的喂养不耐受和营养不良，如果治疗不当会导致生长迟缓甚至死亡。该病通常与多种先天性畸形相关。"},"tr":{"name":"Doğumsal mikrogastri"}},"xrefs":[{"source":"ICD-10","code":"Q40.2","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"LB13.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1695007532"},{"source":"MONDO","code":"0016041","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0266150","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":108963,"name":"Non-syndromic gastroduodenal malformation","classifications":[147,182]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Antenatal","Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_28361011[PMID]"}],"phenotypes":null}