Letter: Muscular performance and vitamin E in cystic fibrosis.

Patients with cystic fibrosis have tocopherol deficiency presumably as a result of 
steatorrhoea. Evidence for possible muscular involvement in cystic fibrosis patients is 
clinical (muscular weakness and wasting in many patients), biochemical (creatinuria on 
a creatine-poor diet, with reversal of this finding after ingestion of 
tocopherol esters, accompanied by a decrease in creatine in plasma and 
an increase in creatine in muscle), and pathological (focal lesions in 
skeletal muscle resembling nutritional muscular dystrophy, and ceroid pigment in smooth 
muscle). Muscle strength was measured with a bulb ergograph in 45 
patients with cystic fibrosis attending the clinics at the Babies' Hospital, 
Columbia Presbyterian Medical Center, New York. In a double-blind study, in 
which half the cases received tocopherol orally, and the remainder placebo, 
no significant increase in muscle strength was found in those patients 
receiving tocopherol over a period of 6 months as compared to 
the placebo group. Blood tocopherol levels in the treated group returned 
to normal. 