title: Long-term liver-related morbidity and mortality related to chronic hepatitis C virus infection in Swedish patients with inherited bleeding disorders.
abstract: BACKGROUND
  Hepatitis C virus (HCV) infection is common in patients with inherited bleeding disorders treated with clotting factor concentrates prior to the introduction of viral inactivation of these products. The long-term consequences of hepatitis C infection in Swedish patients are not fully understood.
  
  
  OBJECTIVE
  To examine the impact of HCV infection on liver-related morbidity and mortality in Swedish patients with inherited bleeding disorders.
  
  
  METHODS
  We retrospectively collected data on 183 patients with inherited bleeding disorders infected with HCV who attended the Coagulation Unit at Karolinska University Hospital, Sweden. Data regarding end-stage liver disease (ESLD), defined as presence of ascites, encephalopathy, variceal bleeding, hepatocellular carcinoma or liver-related death, were collected from the patient records and the national registers.
  
  
  RESULTS
  The median follow-up time was 35.9 years (IQR 29.0-41.2). A total of 41% had achieved sustained virological response (SVR) after treatment. In total, 14.2% developed ESLD at the median age of 52.6 years (IQR 46.5-64.7). Nineteen (35.8%) of all deaths were due to liver-related causes. Co-infection with human immunodeficiency virus (HIV), older age at time of infection and severe form of bleeding disorder was associated with higher risk of developing ESLD, while SVR was a strong protective factor.
  
  
  CONCLUSIONS
  This study demonstrated that liver-related morbidity and mortality was significant in patients with bleeding disorders and HCV infection in Sweden. Patients with HCV-infection should be candidates for treatment with the new highly effective antiviral drugs, since SVR proved to be a strong protective factor.
authors.affiliation: Coagulation Unit, Department of Medicine Solna, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
authors.forename: M
authors.initials: M
authors.lastname: Holmström
authors.affiliation: Department of Infectious Diseases, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Nangarhari
authors.affiliation: Karolinska Institutet at Karolinska Hospital, Stockholm, Sweden.
authors.forename: J
authors.initials: J
authors.lastname: Öhman
authors.affiliation: Department of Infectious Diseases, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
authors.forename: A-S
authors.initials: AS
authors.lastname: Duberg
authors.affiliation: Coagulation Unit, Department of Medicine Solna, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.|Department of Infectious Diseases, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Majeed
authors.affiliation: Department of Infectious Diseases, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
authors.forename: S
authors.initials: S
authors.lastname: Aleman
mesh_terms: D015331:Cohort Studies; D018450:Disease Progression; D004932:Esophageal and Gastric Varices / Q000209:etiology*; D005260:Female; D019698:Hepatitis C, Chronic / Q000150:complications / Q000401:mortality; D006801:Humans; D008099:Liver / Q000473:pathology*; D008113:Liver Neoplasms / Q000150:complications / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013548:Sweden
keywords: haemophilia; hepatitis C; hepatocellular carcinoma; liver complications; liver-related death