{"orpha":276405,"name":"Hyperbiliverdinemia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Green jaundice"],"definition":"Hyperbiliverdinemia is a rare, genetic hepatic disease characterized by the presence of green coloration of the skin, urine, plasma and other body fluids (ascites, breastmilk) or parts (sclerae) due to increased serum levels of biliverdin in association with biliary obstruction and/or liver failure. Association with malnutrition, medication, and congenital biliary atresia has also been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-276405/","orphanet_url":"https://www.orpha.net/en/disease/detail/276405","names":{"fr":{"name":"Hyperbiliverdinémie","synonyms":["Jaunisse verte"],"definition":"Maladie hépatique génétique rare caractérisée par la présence d'une coloration verdâtre de la peau, de l'urine, du plasma et d'autres fluides corporels (ascite, lait maternel) ou de structures (sclérotiques) due à des taux sériques accrus de biliverdine en association avec une obstruction biliaire et/ou une insuffisance hépatique. Des cas d'association avec la malnutrition, des médicaments et une atrésie biliaire congénitale ont également été rapportés."},"de":{"name":"Hyperbiliverdinämie","synonyms":["Verdinikterus"]},"es":{"name":"Hiperbiliverdinemia","synonyms":["Ictericia verde"],"definition":"Es una enfermedad hepática, genética y poco frecuente, caracterizada por una coloración verdosa de la piel, orina, plasma y otros fluidos (ascítico, leche materna) o de estructuras anatómicas (escleróticas) debido al aumento de los niveles séricos de biliverdina junto con obstrucción biliar y/o insuficiencia hepática. También se ha descrito la asociación con desnutrición, fármacos y atresia biliar congénita."},"it":{"name":"Iperbiliverdinemia","synonyms":["Ittero verde"],"definition":"L'iperbiliverdinemia è un'epatopatia genetica rara, caratterizzata dalla colorazione verde della cute, delle urine, del plasma e di altri liquidi corporei (asciti, latte materno) o di parti del corpo (sclere), secondaria ad un aumento dei livelli di biliverdina nel siero secondario all'ostruzione biliare e/o all'insufficienza epatica. Può associarsi a malnutrizione, assunzione di alcuni farmaci ed atresia biliare congenita."},"nl":{"name":"Hyperbiliverdinemie","synonyms":["Groene geelzucht"],"definition":"Hyperbiliverdinemie is een zeldzame, genetische leverziekte die gekarakteriseerd wordt door de aanwezigheid van groene verkleuring van huid, urine, plasma en andere lichaamsvloeistoffen (ascites, moedermelk) of lichaamsdelen (sclerae) door verhoogde gehaltes van biliverdine, in associatie met obstructie van de galwegen en/of leverfalen. Associatie met malnutritie, medicatie en congenitale biliaire atresie werd ook reeds gerapporteerd."},"pt":{"name":"Hiperbiliverdémia"},"pl":{"name":"Hiperbiliwerdynemia","synonyms":["Zielona żółtaczka"],"definition":"*Hiperbiliwerdynemia jest rzadką, genetycznie uwarunkowaną chorobą wątroby, która charakteryzuje się obecnością zielonego zabarwienia skóry, moczu, osocza i innych płynów ustrojowych (płyn otrzewnowy, mleko matki) oraz niektórych struktur (twardówka) z powodu podwyższonego poziomu biliwerdyny w surowicy krwi, z towarzyszącą niedrożnością dróg żółciowych i/lub) niewydolnością wątroby. Opisywano również związek z niedożywieniem, przyjmowaniem leków i wrodzoną atrezją dróg żółciowych."},"cs":{"name":"Hyperbiliverdinémie","synonyms":["Zelená žloutenka"]},"uk":{"name":"Гіпербілівердинемія","synonyms":["Зелена жовтяниця"],"definition":"Hyperbiliverdinemia is a rare, genetic hepatic disease characterized by the presence of green coloration of the skin, urine, plasma and other body fluids (ascites, breastmilk) or parts (sclerae) due to increased serum levels of biliverdin in association with biliary obstruction and/or liver failure. Association with malnutrition, medication, and congenital biliary atresia has also been reported."},"zh":{"name":"高胆绿素血症","synonyms":["绿色黄疸"],"definition":"高胆绿素血症是一种罕见的遗传性肝病，其特征是皮肤、尿液、血浆和其他体液（腹水、母乳）或部分（巩膜）呈绿色，血清胆绿素升高，伴有胆道梗阻和/或肝衰竭。也有报道与营养不良，药物治疗，先天性胆道闭锁有关。"},"tr":{"name":"Hiperbiliverdinemi","synonyms":["Yeşil sarılık"]}},"xrefs":[{"source":"OMIM","code":"614156","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"K76.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5C90.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0013595","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3279964","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":156601,"name":"Rare genetic hepatic disease","classifications":[156]},{"orpha":57146,"name":"Rare hepatic disease","classifications":[183]}],"children":[],"genes":[{"symbol":"BLVRA","name":"biliverdin reductase A","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"21278388[PMID]","hgnc":"1062","ensembl":"ENSG00000106605","omim":"109750","loci":["7p13"]}],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Autosomal recessive"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":2.0,"area":"Worldwide","status":"Validated","source":"19580635[PMID]_21278388[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}