Literature DB >> 16483397

A randomized trial of long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria.

Carlo Agostoni1, Ann Harvie, Daphne L McCulloch, Colin Demellweek, Forrester Cockburn, Marcello Giovannini, Gordon Murray, R Angus Harkness, Enrica Riva.   

Abstract

Forty-two infants (20 males, 22 females) with classical phenylketonuria (PKU) entered a prospective, double-blind, randomized study to investigate the effects on biochemical and physiological outcomes of a phenylalanine-free infant formula containing a fat blend supplemented with the long-chain polyunsaturated fatty acids (LC-PUFA), docosahexaenoic acid (DHA, C22:6 n-3), and arachidonic acid (AA, C20:4 n-6). Between entry and 20 weeks (entry and 1y) of age, median DHA levels in erythrocyte membrane phospholipids decreased by 15% (22%) in the LC-PUFA supplemented group (n=21) and by 61% (64%) in the control group (p<0.001; n=18). A dietary supply of LC-PUFA in infants with PKU prevents the decline in DHA levels associated with a diet supplying minimal sources of LC-PUFA. DHA status in turn, independent of diet, may influence the maturation of the visual system in infants with PKU.

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Year:  2006        PMID: 16483397     DOI: 10.1017/S0012162206000442

Source DB:  PubMed          Journal:  Dev Med Child Neurol        ISSN: 0012-1622            Impact factor:   5.449


  10 in total

1.  A randomized, placebo-controlled, double-blind trial of supplemental docosahexaenoic acid on cognitive processing speed and executive function in females of reproductive age with phenylketonuria: A pilot study.

Authors:  S H L Yi; J A Kable; M L Evatt; R H Singh
Journal:  Prostaglandins Leukot Essent Fatty Acids       Date:  2011-10-14       Impact factor: 4.006

Review 2.  Nutritional issues in treating phenylketonuria.

Authors:  François Feillet; Carlo Agostoni
Journal:  J Inherit Metab Dis       Date:  2010-02-12       Impact factor: 4.982

3.  A cross-sectional study of docosahexaenoic acid status and cognitive outcomes in females of reproductive age with phenylketonuria.

Authors:  Sarah H L Yi; Julie A Kable; Marian L Evatt; Rani H Singh
Journal:  J Inherit Metab Dis       Date:  2011-02-09       Impact factor: 4.982

4.  n-3 Fatty Acid Supplementation in Mothers, Preterm Infants, and Term Infants and Childhood Psychomotor and Visual Development: A Systematic Review and Meta-Analysis.

Authors:  Masha Shulkin; Laura Pimpin; David Bellinger; Sarah Kranz; Wafaie Fawzi; Christopher Duggan; Dariush Mozaffarian
Journal:  J Nutr       Date:  2018-03-01       Impact factor: 4.798

5.  Phenylketonuria: nutritional advances and challenges.

Authors:  Marcello Giovannini; Elvira Verduci; Elisabetta Salvatici; Sabrina Paci; Enrica Riva
Journal:  Nutr Metab (Lond)       Date:  2012-02-03       Impact factor: 4.169

6.  Pre-attentive processing in children with early and continuously-treated PKU. Effects of concurrent Phe level and lifetime dietary control.

Authors:  Leo M J de Sonneville; Stephan C J Huijbregts; Robert Licht; Joseph A Sergeant; Francjan J van Spronsen
Journal:  J Inherit Metab Dis       Date:  2011-05-04       Impact factor: 4.982

Review 7.  Long-chain polyunsaturated fatty acids in inborn errors of metabolism.

Authors:  Katalin Fekete; Tamás Decsi
Journal:  Nutrients       Date:  2010-09-15       Impact factor: 5.717

Review 8.  New Strategies for the Treatment of Phenylketonuria (PKU).

Authors:  Pietro Strisciuglio; Daniela Concolino
Journal:  Metabolites       Date:  2014-11-04

Review 9.  Protein Substitutes in PKU; Their Historical Evolution.

Authors:  Anne Daly; Sharon Evans; Alex Pinto; Catherine Ashmore; Anita MacDonald
Journal:  Nutrients       Date:  2021-02-02       Impact factor: 5.717

Review 10.  Phenylketonuria: dietary and therapeutic challenges.

Authors:  M Giovannini; E Verduci; E Salvatici; L Fiori; E Riva
Journal:  J Inherit Metab Dis       Date:  2007-03-08       Impact factor: 4.750

  10 in total

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