title: Biochemical assessment of the nutritional status of cystic fibrosis patients treated with pancreatic enzyme extracts.
abstract: We examined the protein and fat nutritional status of 65 cystic fibrosis patients aged 4-26 y (x +/- SD: 11.2 +/- 5.6 y). Patients were treated with pancreatic enzyme extracts to improve nutrient absorption; in addition, most patients were supplemented with vitamins A and E. Results were compared with those in a control group of 39 subjects aged 5-29 y (x: 14.3 +/- 5.6 y) with no digestive diseases or nutritional deficiencies. Protein determination showed low albumin concentrations in 42% of the cystic fibrosis patients and decreased blood concentrations of retinol binding protein in 12% of the patients. Lipoprotein components were characterized by decreased cholesterol concentrations in 25% of the cystic fibrosis group. Also, mean concentrations of apolipoprotein A-I were significantly lower in the cystic fibrosis group than in control subjects. The results of fatty acid status, expressed in relative (%) and absolute (mg/L) values, showed concentrations of essential fatty acids, represented by linoleic and arachidonic acids, to be significantly decreased in cystic fibrosis patients; this decrease was markedly significant for fatty acid status expressed in absolute values, especially in the cholesteryl ester subfraction. Serum retinol and alpha-tocopherol concentrations were lowered by 8% and 46% in cystic fibrosis patients and control subjects, respectively: retinol, 1.80 +/- 0.50 and 2.37 +/- 0.60 micromol/L, P < 0.001, and alpha-tocopherol, 18.1 +/- 8.7 and 25.7 +/- 5.0 micromol/L, P < 0.001. In conclusion, despite regular treatment with pancreatic enzyme replacements, neither protein nor fat malnutrition in cystic fibrosis patients was completely corrected.
authors.affiliation: Laboratoire de Biochimie B et d'Immunologie, UF Lipides-Glucides, Centre Hospitalier Lyon Sud, France.
authors.forename: H
authors.initials: H
authors.lastname: Benabdeslam
authors.forename: I
authors.initials: I
authors.lastname: Garcia
authors.forename: G
authors.initials: G
authors.lastname: Bellon
authors.forename: R
authors.initials: R
authors.lastname: Gilly
authors.forename: A
authors.initials: A
authors.lastname: Revol
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002788:Cholesterol Esters / Q000097:blood; D003550:Cystic Fibrosis / Q000097:blood* / Q000188:drug therapy*; D005227:Fatty Acids / Q000097:blood; D005260:Female; D006801:Humans; D008074:Lipoproteins / Q000097:blood; D008297:Male; D009752:Nutritional Status; D010179:Pancreas / Q000201:enzymology; D010184:Pancreatic Extracts / Q000627:therapeutic use*; D010194:Pancreatin / Q000627:therapeutic use; D010743:Phospholipids / Q000097:blood; D012177:Retinol-Binding Proteins / Q000378:metabolism; D012995:Solubility; D014815:Vitamins / Q000097:blood
chemical_list: D015415:Biomarkers; D002788:Cholesterol Esters; D005227:Fatty Acids; D008074:Lipoproteins; D010184:Pancreatic Extracts; D010743:Phospholipids; D012177:Retinol-Binding Proteins; D014815:Vitamins; D010194:Pancreatin