{"orpha":480524,"name":"Idiopathic peliosis hepatis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic peliosis hepatitis"],"definition":"A rare vascular liver disease characterized by widespread or focal cystic dilatation of sinusoidal blood-filled spaces of the liver without any known cause. Lesions can vary in diameter between few millimeters and several centimeters. The condition may remain asymptomatic or manifest with complications including rupture and intraperitoneal hemorrhage, hepatomegaly, portal hypertension, cholestasis, and liver failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-480524/","orphanet_url":"https://www.orpha.net/en/disease/detail/480524","names":{"fr":{"name":"Péliose hépatique idiopathique","definition":"Maladie vasculaire rare du foie caractérisée par une dilatation kystique généralisée ou focale des espaces sinusoïdaux remplis de sang du foie sans cause connue. Le diamètre des lésions peut varier de quelques millimètres à plusieurs centimètres. La maladie peut rester asymptomatique ou se manifester par des complications, notamment une rupture et une hémorragie intrapéritonéale, une hépatomégalie, une hypertension portale, une cholestase et une insuffisance hépatique."},"de":{"name":"Peliosis hepatis, idiopathische","definition":"Eine seltene vaskuläre Lebererkrankung, die durch eine weit verbreitete oder fokale zystische Erweiterung der mit Blut gefüllten Sinusoidalräume der Leber ohne bekannte Ursache gekennzeichnet ist. Der Durchmesser der Läsionen kann zwischen einigen Millimetern und mehreren Zentimetern variieren. Die Erkrankung kann asymptomatisch bleiben oder mit Komplikationen wie Ruptur und intraperitonealer Blutung, Hepatomegalie, portaler Hypertension, Cholestase und Leberversagen einhergehen."},"es":{"name":"Peliosis hepática idiopática","synonyms":["Hepatitis peliótica idiopática"],"definition":"Es una enfermedad hepática vascular poco frecuente caracterizada por dilatación quística focal o generalizada de los espacios sinusoidales hepáticos llenos de sangre sin causa conocida. Las lesiones pueden variar entre unos pocos milímetros y varios centímetros de diámetro. La afección puede permanecer asintomática o manifestarse con complicaciones, que incluyen ruptura y hemorragia intraperitoneal, hepatomegalia, hipertensión portal, colestasis e insuficiencia hepática."},"it":{"name":"Peliosi epatica idiopatica","definition":"Si tratta di una malattia epatica vascolare rara, caratterizzata dalla dilatazione cistica diffusa o focale degli spazi sinusoidali del fegato contenti sangue, ad eziologia ignota. Le lesioni possono avere un diametro di alcuni millimetri o centimetri. I pazienti possono essere asintomatici oppure presentare complicanze, compresa la rottura e l'emorragia intraperitoneale, l'epatomegalia, l'ipertensione portale, la colestasi e l'insufficienza epatica."},"nl":{"name":"Idiopathische peliosis hepatis","synonyms":["Idiopathische peliosis hepatitis"],"definition":"Een zeldzame vasculaire aandoening van lever, gekarakteriseerd door wijdverspreide of focale cystische dilatatie van sinusoïdale, met bloed gevulde ruimtes van lever zonder enige gekende oorzaak. De diameter van laesies kan variëren van enkele millimeters tot meerdere centimeters. De aandoening kan asymptomatisch blijven of zich manifesteren met complicaties, waaronder ruptuur en intraperitoneale hemorragie, hepatomegalie, portale hypertensie, cholestase, en leverfalen."},"pt":{"name":"Peliose hepática idiopática"},"pl":{"name":"Prosowate wylewy krwawe wątroby","synonyms":["Idiopathic peliosis hepatitis"]},"cs":{"name":"Idiopatická pelióza jater","synonyms":["Idiopathic peliosis hepatitis","Idiopatická hepatická pelióza"]},"uk":{"name":"Ідіопатичний пеліоз печінки","synonyms":["Ідіопатичний пеліозний гепатит"],"definition":"A rare vascular liver disease characterized by widespread or focal cystic dilatation of sinusoidal blood-filled spaces of the liver without any known cause. Lesions can vary in diameter between few millimeters and several centimeters. The condition may remain asymptomatic or manifest with complications including rupture and intraperitoneal hemorrhage, hepatomegaly, portal hypertension, cholestasis, and liver failure."},"zh":{"name":"特发性紫癜性肝炎","synonyms":["Idiopathic peliosis hepatitis"],"definition":"一种罕见的血管性肝病，以无任何已知原因的肝窦状充血性广泛或局灶性囊性扩张为特征。病变直径可在几毫米到几厘米之间。这种情况可能一直是无症状的，或表现为并发症，包括破裂和腹腔内出血、肝肿大、门脉高压、胆汁淤积和肝衰竭。"},"tr":{"name":"İdiyopatik peliosis hepatis","synonyms":["İdiyopatik peliosis hepatiti"]}},"xrefs":[{"source":"ICD-10","code":"K76.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DB98.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018809","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680101","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":101938,"name":"Rare vascular liver disease","classifications":[183]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}