BIOCHEMICAL ASSESSMENT OF THE NUTRITIONAL-STATUS OF CYSTIC-FIBROSIS PATIENTS TREATED WITH PANCREATIC-ENZYME EXTRACTS

We examined the protein and fat nutritional status of 65 cystic fibros is patients aged 4-26 y ((x) over bar +/- SD: 11.2 +/- 5.6 y). Patient s were treated with pancreatic enzyme extracts to improve nutrient abs orption; in addition, most patients were supplemented with vitamins A and E. Results were compared with those in a control group of 39 subje cts aged 5-29 y ((x) over bar: 14.3 +/- 5.6 y) with no digestive disea ses or nutritional deficiencies. Protein determination showed low albu min concentrations in 42% of the cystic fibrosis patients and decrease d blood concentrations of retinol binding protein in 12% of the patien ts. Lipoprotein components were characterized by decreased cholesterol concentrations in 25% of the cystic fibrosis group. Also, mean concen trations of apolipoprotein A-I were significantly lower in the cystic fibrosis group than in control subjects. The results of fatty acid sta tus, expressed in relative (%) and absolute (mg/L) values, showed conc entrations of essential fatty acids, represented by linoleic and arach idonic acids, to be significantly decreased in cystic fibrosis patient s; this decrease was markedly significant for fatty acid status expres sed in absolute values, especially in the cholesteryl ester subfractio n. Serum retinol and alpha-tocopherol concentrations were lowered by 8 % and 46% in cystic fibrosis patients and control subjects, respective ly: retinol, 1.80 +/- 0.50 and 2.37 +/- 0.60 mu mol/L, P < 0.001, and alpha-tocopherol, 18.1 +/- 8.7 and 25.7 +/- 5.0 mu mol/L, P < 0.001. I n conclusion, despite regular treatment with pancreatic enzyme replace ments, neither protein nor fat malnutrition in cystic fibrosis patient s was completely corrected.