Literature DB >> 1628465

A treatment program for adolescents with phenylketonuria.

L A Gleason1, K Michals, R Matalon, P Langenberg, S Kamath.   

Abstract

A treatment program for adolescents with phenylketonuria (PKU), incorporating education, goal-setting, self-monitoring, contracts, and rewards, was evaluated by measuring knowledge of PKU, blood phenylalanine concentrations, and health locus of control (LOC) before and after participation in the program. Of the 16 subjects, seven subjects successfully completed the program by achieving behavioral goals. These subjects increased their knowledge of PKU and decreased their blood phenylalanine concentrations, but the nine nonsuccessful subjects did not. There was no significant change in LOC scores for either group. There was a significant relationship between baseline blood phenylalanine levels and success with the program. Therefore, this pilot study demonstrates that adolescents who have already achieved some measure of metabolic control can be expected to be most successful with this program and realize the greatest benefits from it in the form of increased knowledge of PKU and even better metabolic control.

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Year:  1992        PMID: 1628465     DOI: 10.1177/000992289203100603

Source DB:  PubMed          Journal:  Clin Pediatr (Phila)        ISSN: 0009-9228            Impact factor:   1.168


  7 in total

1.  Elevated plasma phenylalanine concentrations may adversely affect bone status of phenylketonuric mice.

Authors:  S Yannicelli; D M Medeiros
Journal:  J Inherit Metab Dis       Date:  2002-09       Impact factor: 4.982

2.  Demographic and Psychosocial Influences on Treatment Adherence for Children and Adolescents with PKU: A Systematic Review.

Authors:  Emma Medford; Dougal Julian Hare; Anja Wittkowski
Journal:  JIMD Rep       Date:  2017-08-25

3.  Large neutral amino acids in the treatment of phenylketonuria (PKU).

Authors:  R Matalon; K Michals-Matalon; G Bhatia; E Grechanina; P Novikov; J D McDonald; J Grady; S K Tyring; F Guttler
Journal:  J Inherit Metab Dis       Date:  2006-09-21       Impact factor: 4.982

4.  Intellectual and school performance in adolescents with phenylketonuria according to their dietary compliance. The Czech-Slovak Collaborative Study.

Authors:  J Zeman; A Pijackova; J Behulova; O Urge; J Hyanek
Journal:  Eur J Pediatr       Date:  1996-07       Impact factor: 3.183

Review 5.  The truth of treating patients with phenylketonuria after childhood: the need for a new guideline.

Authors:  F J van Spronsen; P Burgard
Journal:  J Inherit Metab Dis       Date:  2008-08-12       Impact factor: 4.982

6.  Double blind placebo control trial of large neutral amino acids in treatment of PKU: effect on blood phenylalanine.

Authors:  R Matalon; K Michals-Matalon; G Bhatia; A B Burlina; A P Burlina; C Braga; L Fiori; M Giovannini; E Grechanina; P Novikov; J Grady; S K Tyring; F Guttler
Journal:  J Inherit Metab Dis       Date:  2007-02-27       Impact factor: 4.750

7.  Long-term treatment of phenylketonuria with a new medical food containing large neutral amino acids.

Authors:  D Concolino; I Mascaro; M T Moricca; G Bonapace; K Matalon; J Trapasso; G Radhakrishnan; C Ferrara; R Matalon; P Strisciuglio
Journal:  Eur J Clin Nutr       Date:  2016-09-14       Impact factor: 4.016

  7 in total

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