Vitamin A status in biliary atresia: intestinal absorption and liver storage of retinol.

The vitamin A status of 19 patients with corrected biliary atresia was examined. They had been receiving 5,000 IU of oral vitamin A daily postoperatively. Plasma vitamin A levels in the nonjaundiced group were almost within normal range, whereas those in the jaundiced group were significantly low compared with the controls. In the oral vitamin A tolerance test, plasma vitamin A levels increased from 33.1 +/- 11.8 to 215.4 +/- 100.7 micrograms/dL in the nonjaundiced group, and from 23.1 +/- 10.3 to 209.8 +/- 154.2 micrograms/dL in the slightly jaundiced group, at 4 hours after the administration of vitamin A, showing no difference between both group and control. In the severely jaundiced group, plasma vitamin A levels increased from 13.5 +/- 3.5 to 30.0 +/- 14.6 micrograms/dL, a significantly smaller increase compared with controls. However, liver vitamin A levels were greater than 20 micrograms/g liver in all patients, irrespective of the presence of jaundice. This study suggested that nutritional support to facilitate the synthesis of retinol-binding protein may be an important factor in addition to vitamin A supplementation.