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Vitamin E Deficiency and Neurologic Disease in Adults with Cystic Fibrosis

MICHAEL D. SITRIN, M.D.; FRANK LIEBERMAN, M.D.; WESLEY E. JENSEN, Ph.D.; AVERTANO NORONHA, M.D.; CAROL MILBURN, R.N.; and WHITNEY ADDINGTON, M.D.
[+] Article and Author Information

▸Requests for reprints should be addressed to Michael D. Sitrin, M.D.; The University of Chicago, 5841 S. Maryland, Box 223; Chicago, IL 60637.


Chicago, Illinois


©1987 American College of PhysiciansAmerican College of Physicians


Ann Intern Med. 1987;107(1):51-54. doi:10.7326/0003-4819-107-1-51
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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.

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