{"orpha":402823,"name":"Hepatitis delta","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["HDV","Hepatitis D virus"],"definition":"Hepatitis delta is a rare hepatic disease characterized by variable degrees of acute hepatitis resulting from infection with the hepatitis delta virus. Occasionally it may present a benign course, but most frequently it manifests with severe liver disease that may include fulminant liver failure, hepatic decompensation and rapid progression to cirrhosis. All patients present concomitant hepatitis B virus infection and an increased risk of developing hepatocellular carcinoma has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-402823/","orphanet_url":"https://www.orpha.net/en/disease/detail/402823","names":{"fr":{"name":"Hépatite delta","synonyms":["VHD","Virus de l'hépatite D"],"definition":"Maladie hépatique rare caractérisée par une hépatite aiguë de degrés variables résultant d'une infection par le virus de l'hépatite delta. Même si dans certains cas l'évolution est bénigne, il s'agit le plus souvent d'une maladie hépatique grave pouvant entraîner une insuffisance hépatique fulminante, une décompensation hépatique et une progression rapide vers la cirrhose. Tous les patients présentent une infection concomitante par le virus de l'hépatite B et un risque accru de développer un carcinome hépatocellulaire a été rapporté."},"de":{"name":"Hepatitis delta","synonyms":["HDV","Hepatitis D-Virus"]},"es":{"name":"Hepatitis delta","synonyms":["VHD","Virus de la hepatitis D"],"definition":"Es una enfermedad hepática poco frecuente, caracterizada por diferentes grados de hepatitis aguda como resultado de la infección por el virus de la hepatitis delta. Ocasionalmente, puede presentar un curso benigno, pero se manifiesta, con mayor frecuencia, como enfermedad hepática grave, incluyendo insuficiencia hepática fulminante, descompensación hepática y progresión rápida a cirrosis. Todos los afectados presentan una infección concomitante por el virus de la hepatitis B y también se ha descrito un aumento del riesgo de desarrollar carcinoma hepatocelular."},"it":{"name":"Epatite Delta","definition":"L'epatite delta è un'epatopatia rara, caratterizzata da epatite acuta di grado variabile, secondaria all'infezione da virus dell'epatite delta. Occasionalmente il decorso è benigno, ma, nella maggior parte dei pazienti, la malattia esordisce con un'epatopatia grave, che può comprendere l'insufficienza epatica fulminante, lo scompenso epatico e la rapida progressione verso la cirrosi. Tutti i pazienti presentano un'infezione concomitante da virus dell'epatite B e sono a rischio di sviluppare il carcinoma epatocellulare."},"nl":{"name":"Hepatitis delta","synonyms":["Hepatitis D-virus","HDV"],"definition":"Hepatitis delta is een zeldzame leverziekte die gekarakteriseerd wordt door variabele gradaties van acute hepatitis als gevolg van infectie met hepatitis delta-virus. Soms vertoont de ziekte een goedaardig verloop, maar meestal manifesteert ze zich met ernstige leverziekte met mogelijks fulminant leverfalen, leverdecompensatie en snelle progressie naar cirrose. Alle patiënten vertonen gelijktijdige infectie met hepatitis B-virus en er werd een verhoogd risico op ontwikkeling van hepatocellulair carcinoom gerapporteerd."},"pt":{"name":"Hepatite Delta"},"pl":{"name":"Zapalenie wątroby typu delta","synonyms":["HDV","Wirusowe zapalenie wątroby D"],"definition":"*Zapalenie wątroby typu delta jest rzadką chorobą wątroby, która charakteryzuje się ostrym zapaleniem wątroby o różnym stopniu nasilenia, które jest związane z zakażeniem wirusem zapalenia wątroby typu delta. Czasami przebieg kliniczny może być łagodny, ale najczęściej objawia się ciężką chorobą wątroby, która może manifestować się jako piorunująca niewydolność wątroby, dekompensacja czynności wątroby lub szybko rozwija się marskość wątroby. Wszyscy pacjenci są jednocześnie zakażeni wirusem zapalenia wątroby typu B; opisywano zwiększone ryzyko rozwoju raka wątrobowokomórkowego."},"cs":{"name":"Hepatitida delta","synonyms":["HDV","Hepatitida D"]},"uk":{"name":"Гепатит дельта","synonyms":["HDV","Вірус гепатиту D"],"definition":"Hepatitis delta is a rare hepatic disease characterized by variable degrees of acute hepatitis resulting from infection with the hepatitis delta virus. Occasionally it may present a benign course, but most frequently it manifests with severe liver disease that may include fulminant liver failure, hepatic decompensation and rapid progression to cirrhosis. All patients present concomitant hepatitis B virus infection and an increased risk of developing hepatocellular carcinoma has been reported."},"zh":{"name":"丁型肝炎","synonyms":["丁型病毒肝炎"],"definition":"丁型肝炎是一种罕见的肝病，其特征是感染了丁型肝炎病毒后会导致不同程度的急性肝炎。有时它可能表现为良性病程，但最常见的是表现为严重的肝脏疾病，包括暴发性肝衰竭，肝代偿失调和快速发展为肝硬化。所有患者均伴有乙型肝炎病毒感染，据报道发生肝细胞癌的风险增加。"},"tr":{"name":"Hepatit delta","synonyms":["HDV","Hepatit D virüsü"]}},"xrefs":[{"source":"ICD-10","code":"B17.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1E51.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1011250453"},{"source":"MONDO","code":"0005789","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D003699","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0011226","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10019762","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":57146,"name":"Rare hepatic disease","classifications":[183]},{"orpha":163585,"name":"Rare viral disease","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":40.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2014[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2023-08-31","source":"29261923[PMID]_37338616[PMID]_34239267[PMID]","terms":[{"hpo":"HP:0001394","label":"Cirrhosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031964","label":"Elevated circulating alanine aminotransferase concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012115","label":"Hepatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001892","label":"Abnormal bleeding","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000978","label":"Bruising susceptibility","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031956","label":"Elevated circulating aspartate aminotransferase concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004787","label":"Fulminant hepatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001399","label":"Hepatic failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033834","label":"Malaise","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001402","label":"Hepatocellular carcinoma","frequency":"Very rare (<4-1%)","criteria":null}]}}