>Plasma creatine concentrations, as measured by a modified semi-micro technic, and urinary excretion of creatine were determined in a group of patients with cystic fibrosis of the pancreas before and ...after the administration of tocopherol. Eighteen patients (69 per cent) showed excessive creatinuria while on creatine-poor diets and in twelve of fourteen creatine excretion diminished following vitamin E supplements. At plasma creatine levels less than 0.6 mg. per cent, little creatine was noted in the urine while at higher concentrations creatine excretion was significant but not directly correlated with the plasma level. A decrease in blood creatine concentration was associated with a concomitant reduction in the urinary output of creatine.
Studies of creatine and noncollagenous nitrogen content in muscle biopsy specimens from a group of hospital normal patients and patients with cystic fibrosis of the pancreas and congenital atresia of the bile ducts showed no differences in noncollagenous nitrogen content. Creatine concentrations per gram noncollagenous nitrogen were significantly lower in muscle specimens from patients with biliary atresia as compared with the normal patients. Excluding the relatively high value for muscle creatine in a six month old infant who showed few clinical signs of malnutrition, the mean muscle creatine concentration for the remaining ten patients with cystic fibrosis of the pancreas was also significantly lower than the average for normal patients. In six of seven patients with cystic fibrosis, from whom muscle biopsy specimens were obtained before and after tocopherol supplementation, creatine content per gram noncollagenous nitrogen rose significantly (48 to 290 per cent) after the administration of vitamin E. In contrast, in two patients with paralytic poliomyelitis, repeat muscle biopsy specimens obtained over a two month interval showed no significant alteration of creatine concentration. The findings in patients with cystic fibrosis of the pancreas resemble the biochemical alterations in the animal with nutritional muscular dystrophy due to tocopherol deficiency and support the view that a counterpart of this manifestation of vitamin E deficiency can develop in the human subject.
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