{"orpha":596941,"name":"Incomplete septal cirrhosis","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Incomplete septal fibrosis"],"definition":"A histopathological form of portosinusoidal vascular disease characterized by the presence of incomplete, thin, perforated, or blind-ended septa, which intermittently delimit rudimentary nodules, although complete cirrhotic-type regenerative nodules are not seen. Isolated collagen bundles can also be observed within the parenchyma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-596941/","orphanet_url":"https://www.orpha.net/en/disease/detail/596941","names":{"fr":{"name":"Cirrhose septale incomplète","synonyms":["Fibrose septale incomplète"],"definition":"Forme histopathologique de maladie vasculaire portosinusoïdale, caractérisée par la présence de septa fibreux fins, incomplets, perforés ou de terminaison en cul-de-sac, qui délimitent de manière intermittente des nodules incomplets, sans nodule de régénération complet cirrhotique. Des amas isolés de fibres de collagène peuvent également être observés dans le parenchyme."},"de":{"name":"Unvollständige Septumzirrhose","synonyms":["Unvollständige Septumfibrose"],"definition":"Eine histopathologische Form der portosinusoidalen Gefäßerkrankung, die durch das Vorhandensein unvollständiger, dünner, perforierter oder blind endender Septen gekennzeichnet ist, die intermittierend rudimentäre Knötchen begrenzen, obwohl keine vollständigen regenerativen Knötchen vom zirrhotischen Typ sichtbar sind. Einzelne Kollagenbündel können auch im Parenchym beobachtet werden."},"es":{"name":"Cirrosis septal incompleta","synonyms":["Fibrosis septal incompleta"],"definition":"Es una forma histopatológica de enfermedad vascular portosinusoidal caracterizada por la presencia de septos incompletos, delgados, perforados o ciegos, que delimitan intermitentemente nódulos rudimentarios, aunque no se observan nódulos regenerativos completos de tipo cirrótico. También se puede observar haces aislados de colágeno dentro del parénquima."},"it":{"name":"Cirrosi settale incompleta","synonyms":["Fibrosi settale incompleta"],"definition":"Si tratta di una forma istopatologica di malattia vascolare porto-sinusoidale, caratterizzata dalla presenza di setti incompleti, sottili, perforati o ciechi, che delimitano in maniera intermittente noduli rudimentali, in assenza di evidenti noduli rigenerativi completi di tipo cirrotico. All'interno del parenchima si osservano anche fasci isolati di collagene."},"nl":{"name":"Incomplete septale cirrose","synonyms":["Incomplete septale fibrose"],"definition":"Een histopathologische vorm van porto-sinusoïdale vasculaire ziekte, gekenmerkt door de aanwezigheid van onvolledige, dunne, geperforeerde, of blind eindigende septa, die intermitterend rudimentaire noduli begrenzen, hoewel volledige cirrotische regeneratieve noduli niet worden geobserveerd. Geïsoleerde collageenbundels kunnen ook waargenomen worden in parenchym."},"pt":{"name":"Cirrose septal incompleta","synonyms":["Fibrose septal incompleta"],"definition":"Forma histopatológica da doença vascular portossinusoidal caracterizada pela presença de septos incompletos, finos, perfurados ou com extremidade cega, que delimitam intermitentemente nódulos rudimentares, embora não sejam observados nódulos regenerativos completos do tipo cirrótico. Feixes isolados de colágeno também podem ser observados no parênquima."},"pl":{"name":"Niekompletne włóknienie przegrodowe","synonyms":["Incomplete septal fibrosis"]},"cs":{"name":"Neúplná septální cirhóza","synonyms":["Incomplete septal fibrosis"]},"uk":{"name":"Неповний цироз печінки","synonyms":["Неповний фіброз перегородки"],"definition":"A histopathological form of portosinusoidal vascular disease characterized by the presence of incomplete, thin, perforated, or blind-ended septa, which intermittently delimit rudimentary nodules, although complete cirrhotic-type regenerative nodules are not seen. Isolated collagen bundles can also be observed within the parenchyma."},"zh":{"name":"Incomplete septal cirrhosis","synonyms":["Incomplete septal fibrosis"]},"tr":{"name":"Incomplete septal cirrhosis","synonyms":["Incomplete septal fibrosis"]}},"xrefs":[{"source":"ICD-10","code":"K74.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0035696","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0544815","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":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}