American Journal of Clinical Nutrition, Vol 46, 606-613, Copyright © 1987 by The American Society for Clinical Nutrition, Inc

ORIGINAL RESEARCH COMMUNICATIONS

Protein metabolism in cystic fibrosis: responses to malnutrition and taurine supplementation

GN Thompson and FM Tomas
Department of Chemical Pathology, Adelaide Children's Hospital, South Australia.

Increased protein breakdown has been cited as an important cause of nutrient loss in cystic fibrosis (CF). Taurine deficiency, which is common in CF, may contribute to the increased breakdown. The occurrence of and the benefit of taurine supplementation to abnormal protein metabolism in apparently optimally treated CF were assessed using a 12- mo double-blind crossover technique in 14 well-nourished and seven mildly-moderately malnourished infection-free preadolescent CF children. Muscle protein breakdown (urinary 3-methylhistidine technique) was significantly decreased in well-nourished (1.35% degraded/24 h +/- 0.15, p less than 0.05) and malnourished (1.24 +/- 0.11, p less than 0.001) CF children compared with controls (1.50 +/- 0.17, n = 13). Whole-body protein flux, synthesis, and catabolism ([15N]-glycine technique) were similar in all groups. Net protein gain was greater in CF children, particularly those who were well-nourished (0.55 g/(kg X 10 h) +/- 0.35, p less than 0.01) compared with controls (0.16 +/- 0.26). Taurine supplementation did not significantly affect any of the indices. In the absence of infection, protein metabolism in CF children responds appropriately to malnutrition.

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				A. Moran, C. Milla, R. Ducret, and K. S. Nair Protein Metabolism in Clinically Stable Adult Cystic Fibrosis Patients With Abnormal Glucose Tolerance Diabetes, June 1, 2001; 50(6): 1336 - 1343. [Abstract] [Full Text]