{"orpha":48372,"name":"Nodular regenerative hyperplasia of the liver","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Non-cirrhotic nodulation"],"definition":"A form of portosinusoidal vascular disease characterized histologically by the absence of cirrhosis and diffuse benign transformation of the hepatic parenchyma into multiple small nodules (typically 1-3 mm).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-48372/","orphanet_url":"https://www.orpha.net/en/disease/detail/48372","names":{"fr":{"name":"Hyperplasie nodulaire régénérative du foie","synonyms":["Nodules d'origine non cirrhotique","Transformation nodulaire du foie"],"definition":"Forme de maladie vasculaire portosinusoïdale caractérisée, sur le plan histologique, par l'absence de cirrhose et par la transformation bénigne et diffuse du parenchyme hépatique en de multiples nodules de petite taille (généralement de 1 à 3 mm)."},"de":{"name":"Noduläre regenerative Hyperplasie der Leber","synonyms":["Nicht-zirrhotische Nodulation"],"definition":"Eine Form der portosinusoidalen Gefäßerkrankung, die histologisch durch das Fehlen einer Zirrhose und eine diffuse gutartige Umwandlung des Leberparenchyms in mehrere kleine Knötchen (typischerweise 1-3 mm) gekennzeichnet ist."},"es":{"name":"Hiperplasia nodular regenerativa del hígado","synonyms":["Nódulo no cirrótico"],"definition":"Es una forma de enfermedad vascular portosinusoidal caracterizada histológicamente por la ausencia de cirrosis por y una transformación benigna difusa del parénquima hepático en múltiples nódulos pequeños (típicamente de 1 a 3 mm)."},"it":{"name":"Iperplasia rigenerativa nodulare del fegato","synonyms":["Nodulazione non cirrotica"],"definition":"Si tratta di una forma di malattia vascolare porto-sinusoidale, caratterizzata dal punto di vista istologico dall'assenza di cirrosi e dalla trasformazione benigna diffusa del parenchima epatico in piccoli noduli (di solito di 1-3 mm)."},"nl":{"name":"Nodulaire regeneratieve hyperplasie van lever","synonyms":["Niet-cirrotische nodulatie"],"definition":"Een vorm van porto-sinusoïdale vasculaire aandoening, histologisch gekenmerkt door afwezigheid van cirrose en diffuse goedaardige transformatie van leverparenchym tot multipele kleine noduli (doorgaans 1-3 mm)."},"pt":{"name":"Hiperplasia hepática regenerativa nodular","synonyms":["Hipertensão portal idiopática não-cirrótica","Nodularidade não-cirrótica"],"definition":"Forma de doença vascular portossinusoidal caracterizada histologicamente pela ausência de cirrose e transformação benigna difusa do parênquima hepático em vários pequenos nódulos (tipicamente 1-3 mm)."},"pl":{"name":"Ogniskowy rozrost guzkowy wątroby","synonyms":["Non-cirrhotic nodulation"]},"cs":{"name":"Nodulární regenerativní hyperplázie jater","synonyms":["Non-cirhotická nodulace"]},"uk":{"name":"Вузлова регенеративна гіперплазія печінки","synonyms":["Нециротична нодуляція"],"definition":"A form of portosinusoidal vascular disease characterized histologically by the absence of cirrhosis and diffuse benign transformation of the hepatic parenchyma into multiple small nodules (typically 1-3 mm)."},"zh":{"name":"肝脏结节性再生性增生","synonyms":["非肝硬化性肝脏结瘤"]},"tr":{"name":"Karaciğerin nodüler rejeneratif hiperplazisi","synonyms":["Sirotik olmayan nodülasyon"]}},"xrefs":[{"source":"ICD-10","code":"K76.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DB98.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1642018758"},{"source":"MONDO","code":"0018835","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5779783","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10929","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]},{"orpha":506210,"name":"Rare disorder potentially indicated for liver transplant","classifications":[233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.34,"area":"Spain","status":"Validated","source":"2586240[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.1,"area":"Spain","status":"Validated","source":"2586240[PMID]"},{"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":"2016-06-01","source":"26415006[PMID]","terms":[{"hpo":"HP:0006707","label":"Abnormality of the hepatic vasculature","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001409","label":"Portal hypertension","frequency":"Occasional (29-5%)","criteria":null}]}}