{"orpha":64743,"name":"Hepatoportal sclerosis","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Obliterative portal venopathy"],"definition":"A form of portosinusoidal vascular disease characterized histologically by varying degrees of phlebosclerosis, primarily involving the small and medium branches of the portal vein with heterogeneous distribution, in the absence of cirrhosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-64743/","orphanet_url":"https://www.orpha.net/en/disease/detail/64743","names":{"fr":{"name":"Veinopathie portale oblitérante","synonyms":["Sclérose hépatoportale"],"definition":"Forme de maladie vasculaire portosinusoïdale, caractérisée histologiquement par des degrés variables de phlébosclérose affectant principalement, de manière hétérogène, les branches portes intrahépatiques de moyen ou petit calibre, en l'absence de cirrhose."},"de":{"name":"Hepatoportale Sklerose","synonyms":["Venopathie, obliterative portale"],"definition":"Ein Subtyp der portosinusoidalen Gefäßerkrankung, der histologisch durch einen unterschiedlichen Grad der Phlebosklerose gekennzeichnet ist, die hauptsächlich die kleinen und mittleren Äste der Pfortader mit heterogener Verteilung befällt, ohne dass eine Zirrhose vorliegt."},"es":{"name":"Esclerosis hepatoportal","synonyms":["Venopatía portal obliterante"],"definition":"Es una forma de enfermedad vascular portosinusoidal caracterizada histológicamente por diversos grados de fleboesclerosis, que afecta principalmente a las ramas pequeñas y medianas de la vena porta con distribución heterogénea, en ausencia de cirrosis."},"it":{"name":"Sclerosi epatoportale","synonyms":["Venopatia portale obliterativa"],"definition":"Si tratta di una forma di malattia vascolare porto-sinusoidale, caratterizzata dal punto di vista istologico da flebosclerosi di gravità variabile, che interessa prevalentemente i rami piccoli e medi della vena porta, con una distribuzione eterogenea, in assenza di cirrosi."},"nl":{"name":"Hepatoportale sclerose","synonyms":["Obliteratieve portale venopathie"],"definition":"Een vorm van porto-sinusoïdale vasculaire aandoening, histologisch gekenmerkt door variabele gradaties van flebosclerose, hoofdzakelijk met betrokkenheid van kleine en middelgrote aftakkingen van poortader met heterogene distributie, in afwezigheid van cirrose."},"pt":{"name":"Esclerose hepato-portal","synonyms":["Fibrose portal não-cirrótica","Venopatia portal obliterante"],"definition":"Forma de doença vascular portossinusoidal caracterizada histologicamente por diferentes graus de flebosclerose, envolvendo principalmente os ramos pequenos e médios da veia porta com distribuição heterogênea, sem evidência de cirrose."},"pl":{"name":"Stwardnienie wątrobowo-wrotne","synonyms":["Obliterative portal venopathy"]},"cs":{"name":"Hepatoportální skleróza","synonyms":["Obliterující portální venopatie"]},"uk":{"name":"Гепатопортальний склероз","synonyms":["Облітеруюча портальна венопатія"],"definition":"A form of portosinusoidal vascular disease characterized histologically by varying degrees of phlebosclerosis, primarily involving the small and medium branches of the portal vein with heterogeneous distribution, in the absence of cirrhosis."},"zh":{"name":"肝门脉硬化症","synonyms":["闭塞性门静脉病变"]},"tr":{"name":"Hepatoportal skleroz","synonyms":["Obliteratif portal venopati"]}},"xrefs":[{"source":"ICD-10","code":"K74.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018991","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4273756","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":596937,"name":"Portosinusoidal vascular disease","classifications":[183]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-09-14","source":"27555800 [PMID]_24133357[PMID]_29043403[PMID]","terms":[{"hpo":"HP:0001903","label":"Anemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001892","label":"Abnormal bleeding","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030146","label":"Abnormal liver parenchyma morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006707","label":"Abnormality of the hepatic vasculature","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002040","label":"Esophageal varix","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030169","label":"Gastric varix","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001971","label":"Hypersplenism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031015","label":"Intrahepatic portal vein sclerosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001882","label":"Leukopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001409","label":"Portal hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008151","label":"Prolonged prothrombin time","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003262","label":"Smooth muscle antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0020136","label":"Anticardiolipin IgG antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0020137","label":"Anticardiolipin IgM antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100543","label":"Cognitive impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002480","label":"Hepatic encephalopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002904","label":"Hyperbilirubinemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003073","label":"Hypoalbuminemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011954","label":"Nodular regenerative hyperplasia of liver","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001405","label":"Periportal fibrosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030242","label":"Portal vein thrombosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002719","label":"Recurrent infections","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001402","label":"Hepatocellular carcinoma","frequency":"Very rare (<4-1%)","criteria":null}]}}