[Benign recurrent intrahepatic cholestasis]

When aged 25 years, a now 38-year-old man had acute hepatitis A with jaundice. In the subsequent years he had recurrent jaundice with severe itching and insomnia. Multiple hospital admissions with laparoscopy and liver biopsy failed to find a cause. On admission he was again jaundiced, but there was a discrepancy (as on previous examinations) between the degree of jaundice and the good general condition. The liver was only slightly enlarged to palpation, the spleen was just palpable below the costal margin.

Bilirubin concentration was 50.4 mg/dl (direct bilirubin 14 mg/dl), alkaline phosphatase 1027 U/l, GOT and GPT activities were only slightly increased. Ultrasound of the upper abdomen showed deformation of the middle and small-calibre portal vein branches and echo-rich, ribbon-shaped coverings. Electronmicroscopy of a liver biopsy also revealed moderate hepatic fibrosis, indicating benign recurrent intrahepatic cholestasis.

Ursodeoxycholic acid was administered (250 mg two to three times daily by mouth), glycerol trinitrate (0.8 mg six times daily by mouth), colestyramine (4 g up to five times daily by mouth), and flunitrazepam (0.5-1 mg orally at night). There was only slight improvement, but the liver function parameters became normal after three months. There has been no recurrence after 4 years.