{"orpha":231457,"name":"Acute pandysautonomia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acute panautonomic Guillain-Barré syndrome","Acute panautonomic neuropathy","Acute panautonomic GBS"],"definition":"A rare variant of Guillain-Barré syndrome characterized by acute post-ganglionic sympathetic and parasympathetic failure presenting several weeks after acute infection with gastrointestinal symptoms (abdominal pain, vomiting, constipation, diarrhea, gastroparesis, ileus), orthostatic hypotension, erectile dysfunction, urinary frequency, urgency or retention, vasomotor instability with acrocyanosis and reduced salivation, lacrimation and sweating.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231457/","orphanet_url":"https://www.orpha.net/en/disease/detail/231457","names":{"fr":{"name":"Pandysautonomie aiguë","synonyms":["Neuropathie panautonomique aiguë","SGB panautonomique aigu","Syndrome de Guillain-Barré panautonomique aigu"],"definition":"Variant du syndrome de Guillain-Barré caractérisé par une défaillance des systèmes sympathique et parasympathique post-ganglionnaire aiguë se manifestant plusieurs semaines après une infection aiguë accompagnée de symptômes gastro-intestinaux (douleurs abdominales, vomissements, constipation, diarrhée, gastroparésie, iléus), d'hypotension orthostatique, de dysfonction érectile, de mictions fréquentes, d'urgence ou de rétention urinaire, d'instabilité vasomotrice avec acrocyanose et diminution de la production de salive, de larmes et de sueur."},"de":{"name":"Pandysautonomie, akute","synonyms":["Guillain-Barré-Syndrom, akutes panautonomisches","Panautonomische Neuropathie, akute"]},"es":{"name":"Pandisautonomía aguda","synonyms":["GBS panautonómico agudo","Neuropatía panautonómica aguda","Síndrome de Guillain-Barré panautonómico agudo"],"definition":"Es una variante poco frecuente del síndrome de Guillain-Barré caracterizada por insuficiencia simpática y parasimpática posganglionar aguda que se presenta varias semanas después de la infección aguda con síntomas gastrointestinales (dolor abdominal, vómitos, estreñimiento, diarrea, gastroparesia, íleo), hipotensión ortostática, disfunción eréctil, frecuencia urinaria, urgencia o retención, inestabilidad vasomotora con acrocianosis y disminución de la salivación, lagrimeo y sudoración."},"it":{"name":"Pandisautonomia acuta","synonyms":["GBS panautonomica acuta","Neuropatia panautonomica acuta","Sindrome panautonomica acuta di Guillain-Barré"]},"nl":{"name":"Acute pandysautonomie","synonyms":["Acute panautonome neuropathie","Acuut panautonoom GBS","Acuut panautonoom syndroom van Guillain-Barré"],"definition":"Een zeldzame variant van syndroom van Guillain-Barré, gekarakteriseerd door acuut, postganglionair sympathisch en parasympathisch falen dat zich enkele weken na acute infectie presenteert met gastro-intestinale symptomen (abdominale pijn, braken, constipatie, diarree, gastroparese, ileus), orthostatische hypotensie, impotentie, pollakisurie, urinaire urgentie of retentie, vasomotorische instabiliteit met acrocyanose, en verminderde speeksel-, traan- en zweetproductie."},"pt":{"name":"Pandisautonomia aguda"},"pl":{"name":"Ostra pandysautonomia","synonyms":["Ostra neuropatia panautonomiczna","Ostry panautonomiczny GBS","Ostry panautonomiczny zespół Guillaina i Barrégo"]},"cs":{"name":"Akutní pandysautonomie","synonyms":["Akutní panautonomní GBS","Akutní panautonomní Guillainův-Barrého syndrom","Akutní panautonomní neuropatie","Akutní panautonomní Guillain-Barrého syndrom"]},"uk":{"name":"Гостра пандисавтономія","synonyms":["Гострий панавтоматичний GBS","Гострий панавтоматичний синдром Гійєна-Барре","Гостра панавтоматична нейропатія"],"definition":"A rare variant of Guillain-Barré syndrome characterized by acute post-ganglionic sympathetic and parasympathetic failure presenting several weeks after acute infection with gastrointestinal symptoms (abdominal pain, vomiting, constipation, diarrhea, gastroparesis, ileus), orthostatic hypotension, erectile dysfunction, urinary frequency, urgency or retention, vasomotor instability with acrocyanosis and reduced salivation, lacrimation and sweating."},"zh":{"name":"急性全植物神经失调症","synonyms":["急性全植物神经性吉兰-巴雷综合征"],"definition":"一种罕见的吉兰-巴雷综合征，特征是急性感染数周后出现急性节后交感神经和副交感神经功能衰竭，伴有胃肠道症状（腹痛、呕吐、便秘、腹泻、胃轻瘫、肠梗阻）、直立性低血压、勃起功能障碍、尿频，尿急或尿潴留，血管舒缩功能不稳定伴肢端紫绀，唾液分泌、流泪和出汗减少。"},"tr":{"name":"Akut pandisotonomia","synonyms":["Akut panotonomik GBS","Akut panotonomik nöropati","Akut panotonomik Guillain-Barré sendromu"]}},"xrefs":[{"source":"ICD-10","code":"G61.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8C01.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"936714888"},{"source":"MONDO","code":"0016499","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2315246","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":231419,"name":"Functional variant of Guillain-Barré syndrome","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["All ages"],"prevalence":[],"phenotypes":null}