MICHAEL D. SITRIN, M.D.; FRANK LIEBERMAN, M.D.; WESLEY E. JENSEN, Ph.D.; AVERTANO NORONHA, M.D.; CAROL MILBURN, R.N.; WHITNEY ADDINGTON, M.D.
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.
SITRIN MD, LIEBERMAN F, JENSEN WE, et al. Vitamin E Deficiency and Neurologic Disease in Adults with Cystic Fibrosis. Ann Intern Med. 1987;107:51–54. doi: 10.7326/0003-4819-107-1-51
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Published: Ann Intern Med. 1987;107(1):51-54.
Gastroenterology/Hepatology, Neurology, Pancreatic Disease, Pulmonary/Critical Care.
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