{"orpha":243367,"name":"Acute fatty liver of pregnancy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["AFLP"],"definition":"A rare, severe complication occurring in the third trimester of pregnancy or in early postpartum period bearing a risk for perinatal and maternal mortality and characterized by jaundice, rise of hepatic injuries and evolving to acute liver failure and encephalopathy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-243367/","orphanet_url":"https://www.orpha.net/en/disease/detail/243367","names":{"fr":{"name":"Stéatose hépatique aiguë gravidique","synonyms":["AFLP","SHAG","Stéatose hépatique aiguë de la grossesse"],"definition":"Complication rare et grave survenant au cours du troisième trimestre de la grossesse ou au début de la période post-partum, comportant un risque de mortalité périnatale et maternelle, caractérisée par un ictère, l'apparition des lésions hépatiques, évoluant vers une insuffisance hépatique aiguë et une encéphalopathie."},"de":{"name":"Akute Schwangerschaft-Fettleber","definition":"Die Akute Schwangerschafts-Fettleber ist eine seltene, aber schwere Komplikation des dritten Trimenons oder der frühen postpartalen Periode und beinhaltet das Risiko perinataler und maternaler Mortalität. Charakteristische Symptome sind Ikterus, Leberschaden und die Entwicklung eines akuten Leberversagens mit Enzephalopathie."},"es":{"name":"Hígado graso agudo del embarazo","synonyms":["AFLP"],"definition":"Es una complicación grave aunque poco frecuente que se produce en el tercer trimestre del embarazo o en el inicio del periodo postparto. Constituye un riesgo para la mortalidad perinatal y materna, y se caracteriza por ictericia e incremento de las lesiones hepáticas, e implica una insuficiencia hepática aguda y encefalopatía."},"it":{"name":"Fegato grasso acuto della gravidanza","synonyms":["AFLP"],"definition":"Il fegato grasso acuto della gravidanza è una complicazione grave rara, che si presenta nel terzo trimestre della gravidanza o nel primo periodo post-parto, che comporta il rischio di mortalità prenatale e materna, ed è caratterizzata da ittero, aumento delle lesioni epatiche con evoluzione in insufficienza epatica acuta ed encefalopatia."},"nl":{"name":"Acute leververvetting tijdens zwangerschap","synonyms":["AFLP"],"definition":"Een zeldzame, ernstige complicatie tijdens het derde trimester van de zwangerschap of vroege postpartumperiode, die een risico op perinatale en maternale mortaliteit inhoudt en die gekarakteriseerd wordt door geelzucht, toename van letsels van de lever en evolutie naar acuut leverfalen en encefalopathie."},"pt":{"name":"Fígado gordo agudo da gravidez"},"pl":{"name":"Ostre stłuszczenie wątroby w ciąży","synonyms":["AFLP"]},"cs":{"name":"Akutní těhotenská steatóza","synonyms":["AFLP"]},"uk":{"name":"Гостра жирова дистрофія печінки при вагітності","synonyms":["AFLP"],"definition":"A rare, severe complication occurring in the third trimester of pregnancy or in early postpartum period bearing a risk for perinatal and maternal mortality and characterized by jaundice, rise of hepatic injuries and evolving to acute liver failure and encephalopathy."},"zh":{"name":"妊娠期急性脂肪肝","synonyms":["AFLP"],"definition":"妊娠晚期一种罕见的严重并发症，发生于妊娠晚期或产后早期，有围产期孕产妇死亡的风险，其特征是黄疸、肝损害增加并演变为急性肝衰竭和脑病。"},"tr":{"name":"Hamilelikte akut yağlı karaciğer","synonyms":["AFLP"]}},"xrefs":[{"source":"ICD-10","code":"O26.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"JA65.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1226221530"},{"source":"MONDO","code":"0016573","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C537957","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1455728","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9578","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10000746","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":163637,"name":"Rare disorder related with pregnancy, childbirth and puerperium","classifications":[205]},{"orpha":101939,"name":"Rare parenchymal liver disease","classifications":[183]}],"children":[],"genes":[{"symbol":"HADHA","name":"hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"28515471[PMID]","hgnc":"4801","ensembl":"ENSG00000084754","omim":"600890","loci":["2p23.3"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["Adult"],"prevalence":[],"phenotypes":null}