retinol-palmitate and Cystic-Fibrosis

To compare the absorption of a lysophosphatidylcholine, monoglyceride, and fatty acid matrix (organized lipid matrix, OLM) with that of a triacylglycerol (TG)-based fat meal in patients with cystic fibrosis (CF).. Five adolescents with CF and 3 control patients were given fat meals supplemented with retinyl palmitate of either OLM or TG at a 2-week interval. In a clinical trial, 73 patients with CF were randomly assigned to nutritional supplements containing either OLM or TG for a 1-year double-blind trial followed by a 6-month observation period.. The peak increases and areas under the curve for TG and retinyl palmitate after the fat meal were 10-fold higher after OLM than after the TG fat load and did not differ from values obtained in control patients. OLM led to better clinical outcomes in terms of energy intake from the diet, weight-for-age Z score, essential fatty acid status, vitamin E, and retinol binding protein. Height-for-age Z score and FEV(1) only reached statistical significance at the end of the 6-month observation period.. These results suggest that OLM is a readily absorbable source of fat and energy in CF and is an effective nutritional supplement.

Topics: Adolescent; Adult; Anthropometry; Area Under Curve; beta Carotene; Canada; Child; Child Welfare; Cystic Fibrosis; Dietary Fats; Diterpenes; Double-Blind Method; Eicosanoids; Energy Intake; Fatty Acids; Female; Follow-Up Studies; Forced Expiratory Volume; Humans; Intestinal Absorption; Liver; Male; Malondialdehyde; Patient Compliance; Retinol-Binding Proteins; Retinyl Esters; Time Factors; Treatment Outcome; Triglycerides; Vitamin A; Vitamins

Topics: Corneal Diseases; Cystic Fibrosis; Descemet Membrane; Diterpenes; Female; Hernia; Humans; Infant; Keratoplasty, Penetrating; Retinyl Esters; Vitamin A; Xerophthalmia

Topics: beta Carotene; Cystic Fibrosis; Dietary Fats; Dietary Supplements; Diterpenes; Eicosanoids; Energy Intake; Fatty Acids, Essential; Forced Expiratory Volume; Humans; Intestinal Absorption; Malondialdehyde; Retinol-Binding Proteins; Retinyl Esters; Triglycerides; Vitamin A; Vitamins

Vitamin A deficiency is a common manifestation in cystic fibrosis (CF), but high levels of vitamin A in the liver have also been described. High levels of vitamin A in the liver are toxic, while normal levels might be protective against liver damage. In order to investigate whether liver damage in patients with CF is related to vitamin A content of the liver, vitamin A status was investigated in 15 patients with CF aged 8 to 34 years.. Liver biopsy was performed on clinical indication and the vitamin A concentration in the liver was determined as retinylpalmitate. Serum levels of retinol and retinol-binding protein were investigated on the morning of the biopsy. Eight patients had morphologic signs of cirrhosis. Eight patients had been on treatment with ursodeoxycholic acid for 1 to 3 years. All but three patients had been on vitamin A supplementation for years.. Five patients had serum concentrations of retinol below the reference range and seven patients had decreased serum levels of retinol-binding protein. There was a strong correlation between serum levels of retinol and retinol-binding protein (rs = 0.90, p = 0.01), but no correlations with age, Shwachamn score, or genotype. Six of the patients had vitamin A concentrations in the liver < 40 micrograms/g wet weight, and the concentrations decreased significantly with age (rs = 0.77, p = 0.01), without correlation to clinical score or liver disease. There was no indication of hypervitaminosis, although younger patients had been or were being treated with vitamin A in fat-water emulsion.. Our results indicate that the risk of vitamin a deficiency in cystic fibrosis increases with age. The data do not support the view that patients are at risk for hypervitaminosis by long-term supplementation with vitamin A. No correlation was found between the severity of liver disease and the vitamin A content in the liver.

Topics: Adolescent; Adult; Aging; Biopsy; Child; Cystic Fibrosis; Diterpenes; Female; Humans; Liver; Male; Reference Values; Retinol-Binding Proteins; Retinyl Esters; Vitamin A

The aim of this study was to determine the efficacy of long-term oral beta-carotene supplementation for correcting impaired beta-carotene status in cystic fibrosis patients. Thirty-five patients (2.3-30.5 years of age) with coefficients of fat absorption of 46-96% (median 88%) received beta-carotene 0.5 mg/kg daily and were followed over a 16-month treatment period. Baseline plasma beta-carotene concentrations in patients (mean +/- SD, 0.09 +/- 0.06 mumol/l) were significantly lower than those of age-matched controls (0.86 +/- 0.56 mumol/l) (p < 0.0001). Concentrations increased rapidly and reached a plateau at or before 3 weeks that was maintained throughout the study period. Values obtained at 3 weeks (0.89 +/- 0.64 mumol/l) were significantly higher (p < 0.0001) than those at baseline and did not differ from controls. Plasma retinol and alpha-tocopherol concentrations increased during the observation period, but remained within normal ranges. Plasma retinyl palmitate, which was below the detection limit in all but one patient at baseline, did not increase. Thus oral beta-carotene supplementation is effective and normalizes beta-carotene status of cystic fibrosis patients without evidence of significant side effects.

Topics: Adjuvants, Immunologic; Adolescent; Antioxidants; beta Carotene; Carotenoids; Child; Cholesterol; Cystic Fibrosis; Diterpenes; Female; Follow-Up Studies; Food, Fortified; Humans; Male; Regression Analysis; Retinyl Esters; Statistics, Nonparametric; Vitamin A; Vitamin E

We treated two infants with failure to thrive who presented with clinical evidence of conjunctival and corneal xerosis. One patient was referred with possible infectious corneal ulcer thought to exist because there were deep peripheral ulcerations of the cornea and associated hypopyon. The other patient was initially thought to have a nasolacrimal duct obstruction because of excessive tearing. Xerophthalmia secondary to vitamin A deficiency was suspected and led to the diagnosis and treatment of cystic fibrosis in each case. Therapy with vitamin A promptly resolved the xerosis, but it also caused a transient rise in intracerebral pressure. Xerophthalmia can still be a problem in developed countries when underlying disorders, such as cystic fibrosis, lead to vitamin A malabsorption.

Topics: Cystic Fibrosis; Diterpenes; Female; Humans; Infant; Malabsorption Syndromes; Male; Retinyl Esters; Vitamin A; Vitamin A Deficiency; Xerophthalmia