{"orpha":178487,"name":"Adult intestinal botulism","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Adult intestinal colonization botulism","Adult intestinal toxemia botulism","Adult intestinal toxin-mediated botulism","Infant-like botulism"],"definition":"A very rare form of botulism, a rare acquired neuromuscular junction disease with descending flaccid paralysis caused by botulinum neurotoxins (BoNTs), and is due to intestinal colonization by <i>Clostridium botulinum</i> leading to toxin-mediated infection with toxemia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178487/","orphanet_url":"https://www.orpha.net/en/disease/detail/178487","names":{"fr":{"name":"Botulisme intestinal de l'adulte","synonyms":["Botulisme \"infant-like\" de l'adulte","Botulisme colonisant","Botulisme par colonisation intestinale de l'adulte","Botulisme par toxi-infection intestinale de l'adulte"],"definition":"Le botulisme intestinal de l'adulte est une forme très rare de botulisme , une maladie acquise rare de la jonction neuromusculaire avec paralysie flasque descendante due à la neurotoxine botulique (BoNT), qui constitue une toxi-infection par colonisation intestinale de <i>Clostridium botulinum</i>."},"de":{"name":"Botulismus, intestinaler, adulter","synonyms":["Botulismus, intestinaler, des Erwachsenen","Erwachsenenbotulismus"],"definition":"Der adulte intestinale Botulismus ist eine sehr seltene Form des Botulismus, einer erworbenen Störung der Muskelendplatte mit absteigender schlaffer Lähmung, verursacht durch Botulinum-Neurotoxine (BoNTs). Ursache ist die Besiedlung des Darms durch das neurotoxinbildende Clostridium botulinum und Übertritt der BoNTs in das Blut (Toxämie)."},"es":{"name":"Botulismo intestinal del adulto","synonyms":["Botulismo por colonización intestinal en el adulto","Botulismo similar al infantil","Botulismo con toxemia intestinal de adultos","Botulismo intestinal del adulto por toxinas"],"definition":"Es una forma muy rara de botulismo, una enfermedad rara adquirida de la unión neuromuscular con parálisis flácida descendente causada por la neurotoxina botulínica (BoNT), que constituye una toxiinfección por colonización intestinal de <i>Clostridium botulinum</i>."},"it":{"name":"Botulismo intestinale dell'adulto","synonyms":["Botulismo dell'adulto da colonizzazione intestinale","Botulismo dell'adulto da tossiemia intestinale","Botulismo infantile-simile","Botulismo intestinale tossino-mediato dell'adulto"],"definition":"Il botulismo intestinale dell'adulto è una forma molto rara di botulismo (si veda questo termine), una malattia acquisita delle giunzioni neuromuscolari associata a paralisi flaccida discendente da neurotissina del botulino (BoNT), causata dalla colonizzazione intestinale da parte del Clostridium botulinum, che causa un'infezione tossino-mediata con tossiemia."},"nl":{"name":"Intestinaal botulisme bij volwassene","synonyms":["Infantiel gelijkend botulisme","Intestinaal kolonisatiebotulisme bij volwassene","Intestinaal toxemisch botulisme bij volwassene","Intestinaal toxinegemedieerd botulisme bij volwassene"],"definition":"Een zeer zeldzame vorm van botulisme, een zeldzame verworven aandoening van neuromusculaire overgang met afdalende slappe verlamming veroorzaakt door botuline-neurotoxinen (BoNT's), en te wijten aan intestinale kolonisatie door <i>Clostridium botulinum</i> die leidt tot toxine-gemedieerde infectie met toxemie."},"pt":{"name":"Botulismo intestinal do adulto","definition":"O botulismo intestinal do adulto é uma forma muito rara de botulismo (ver este termo), uma doença rara adquirida da junção neuromuscular com paralisia flácida descendente causada por neurotoxinas botulínicas (BoNTs), e é causado por colonização intestinal por <i> Clostridium botulinum </ i> levando a infecção mediada por toxina com toxemia."},"pl":{"name":"Botulizm jelitowy dorosłych","synonyms":["Adult intestinal colonization botulism","Adult intestinal toxemia botulism","Adult intestinal toxin-mediated botulism","Infant-like botulism"]},"cs":{"name":"Adultní střevní botulismus","synonyms":["Adultní střevní kolonizující botulismus","Adultní střevní toxemický botulismus","Adultní střevní botulismus způsobený toxinem","Botulismus podobný dětskému"]},"uk":{"name":"Кишковий ботулізм у дорослих","synonyms":["Ботулізм кишкової колонізації у дорослих","Ботулізм з кишковою токсикоінфекцією у дорослих","Ботулізм, спричинений кишковим токсином у дорослих","Дитячий ботулізм"],"definition":"A very rare form of botulism, a rare acquired neuromuscular junction disease with descending flaccid paralysis caused by botulinum neurotoxins (BoNTs), and is due to intestinal colonization by <i>Clostridium botulinum</i> leading to toxin-mediated infection with toxemia."},"zh":{"name":"成年型肠肉毒中毒","synonyms":["成人型肠毒素介导肉毒中毒"],"definition":"成人肠肉毒中毒是一种非常罕见的肉毒中毒，是一种罕见的获得性神经肌肉连接疾病，由于肉毒杆菌的肠道定植引起毒素介导毒血症所致，其主要临床表现为肉毒杆菌神经毒素（BoNTs）引起的下行性弛缓性麻痹。"},"tr":{"name":"Yetişkin bağırsak botulizmi","synonyms":["Bebek-benzeri botulizm","Erişkin bağırsak toksemi botulizmi","Erişkin bağırsak kolonizasyonu botulizmi","Erişkin bağırsak toksin aracılı botulizm"]}},"xrefs":[{"source":"ICD-10","code":"A05.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"1A11.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1601222948"},{"source":"MONDO","code":"0015806","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4289991","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":178481,"name":"Intestinal botulism","classifications":[181,189,203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":19.0,"area":"Worldwide","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0100021","label":"Cerebral palsy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0006824","label":"Cranial nerve paralysis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0006597","label":"Diaphragmatic paralysis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000508","label":"Ptosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002747","label":"Respiratory insufficiency due to muscle weakness","frequency":"Frequent (79-30%)","criteria":null}]}}