Vitamin E deficiency and neurologic disease in adults with cystic fibrosis.
We report the cases of two adult patients with cystic fibrosis affecting the pancreas and liver, who also had severe vitamin E deficiency and neurologic disease. The most prominent clinical features were abnormal eye movements, diminished reflexes, decreased vibratory and position sense, ataxia, and muscle weakness. Treatment with intramuscular injections of vitamin E partially corrected the neurologic deficits. Vitamin E absorption tests documented severe malabsorption, which was later alleviated by the addition of dessicated ox bile to the regimen of alpha-tocopheryl acetate. These studies suggest that a decreased intraluminal concentration of bile salts is an important factor in the development of severe vitamin E deficiency and in the poor response to oral replacement therapy that is seen in some patients with cystic fibrosis.
D000284:Administration, Oral; D000328:Adult; D001647:Bile Acids and Salts / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D007273:Injections, Intramuscular; D008286:Malabsorption Syndromes / Q000503:physiopathology*; D009422:Nervous System Diseases / Q000209:etiology* / Q000628:therapy; D024505:Tocopherols; D014810:Vitamin E / Q000031:analogs & derivatives / Q000627:therapeutic use; D014811:Vitamin E Deficiency / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D024502:alpha-Tocopherol / Q000031:analogs & derivatives*
D001647:Bile Acids and Salts; D014810:Vitamin E; D024502:alpha-Tocopherol; D024505:Tocopherols