title: Progressive liver disease in haemophilia: an understated problem?
abstract: In an 8-year study of 79 unselected patients with haemophilia who had received clotting factor concentrates, there was evidence of chronic progressive liver disease in at least 17 (21%). 8 patients had chronic active hepatitis and 9 had cirrhosis (5 with oesophageal varices). Histological evidence suggested that non-A non-B hepatitis was mainly responsible, although the influence of other viruses could not be excluded. Serial liver biopsies showed progression from chronic persistent hepatitis to chronic active hepatitis and cirrhosis within 6 years, suggesting that chronic persistent hepatitis in haemophiliacs is not as benign as hitherto supposed. Symptoms and abnormal physical signs were uncommon in these patients. There was no relation between degree of abnormality of serum aminotransferase levels and severity of the underlying liver disease. It is anticipated that liver disease in haemophiliacs will become an increasing clinical problem in the future.
authors.forename: C R
authors.initials: CR
authors.lastname: Hay
authors.forename: F E
authors.initials: FE
authors.lastname: Preston
authors.forename: D R
authors.initials: DR
authors.lastname: Triger
authors.forename: J C
authors.initials: JC
authors.lastname: Underwood
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005164:Factor IX / Q000009:adverse effects; D005169:Factor VIII / Q000009:adverse effects; D006467:Hemophilia A / Q000150:complications* / Q000628:therapy; D006505:Hepatitis / Q000209:etiology / Q000473:pathology; D006801:Humans; D008099:Liver / Q000201:enzymology / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology; D008107:Liver Diseases / Q000201:enzymology / Q000209:etiology* / Q000473:pathology; D008875:Middle Aged; D000637:Transaminases / Q000378:metabolism
chemical_list: D005169:Factor VIII; D005164:Factor IX; D000637:Transaminases