In contrast to deficiencies of vitamins A, D and K, little is known of the prevalence, clinical manifestations and mechanisms of vitamin E deficiency in adult patients with cholestasis. We measured serum vitamin E levels in 45 patients with primary biliary cirrhosis, 20 with primary sclerosing cholangitis, 9 with cryptogenic cirrhosis and 12 with alcoholic cirrhosis. To correct for the hyperlipidemia often found in patients with primary biliary cirrhosis and primary sclerosing cholangitis, total serum lipids were measured and vitamin E levels were expressed as the vitamin E/total serum lipid ratio. Serum vitamin A and D levels and prothrombin time were also determined. Six of 45 patients with primary biliary cirrhosis (13%) but none of the patients with sclerosing cholangitis, cryptogenic cirrhosis or alcoholic cirrhosis had subnormal vitamin E/total serum lipids ratios. Vitamin E deficiency was found in two of eight patients with asymptomatic primary biliary cirrhosis. There was no correlation between standard liver biochemical tests, fasting serum cholylglycine and vitamin E levels. Patients with primary biliary cirrhosis and primary sclerosing cholangitis had significantly lower vitamin E/total serum lipids ratios than patients with either cryptogenic or alcoholic cirrhosis. Twenty‐three percent of patients with primary biliary cirrhosis were vitamin D deficient and 14% had low vitamin A levels. Two of the six patients with vitamin E deficiency were also deficient in vitamin D, only one was vitamin A deficient and none had prolonged prothrombin time. We also investigated the gastrointestinal absorption of vitamin E in nine patients with primary biliary cirrhosis and normal vitamin E levels as well as in six normal controls. Following the administration of a single oral dose (2,000 IU) of dl‐tocopherol, serum vitamin E levels were measured every 3 hr for 12 hr, and then daily for 3 days. Oral vitamin E absorption profiles revealed significant decreases in the area under the curve, peak levels and maximal rise in vitamin E levels in the patients with primary biliary cirrhosis. Neurological examination and hemolysis tests were normal in all patients. We conclude that, as a group, patients with primary biliary cirrhosis and primary sclerosing cholangitis have lower serum vitamin E levels when compared to patients with noncholestatic liver disease. Biochemical evidence of vitamin E deficiency was found in 13% of patients with primary biliary cirrhosis, including two individuals with early, asymptomatic biliary disease. Concomitant deficiency of two or more lipid‐soluble vitamins was unusual. The gastrointestinal absorption of the vitamin E is decreased in primary biliary cirrhosis, even in the presence of normal serum levels. Thus, our results suggest that the mechanism of vitamin E deficiency in primary biliary cirrhosis is related to gastrointestinal malabsorption of vitamin E. Because toxicity associated with vitamin E administration is rare, therapy with vitamin E should be considered in all patients with primary biliary cirrhosis.
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