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Clinical Studies
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Behavioural Effects of Phenylalanine-Free Amino Acid Tablet Supplementation in Intellectually Disabled Adults with Untreated PKU (Part I)

Acta Paediatr. 2005 Sep;94(9):1218-22

  • Department of Paediatrics, Nutrition & Metabolism Unit, Hacettepe University, Ankara, Turkey

  • The John F. Kennedy Institute, Glostrup, Denmark

  • Department of Statistics, Hacettepe University, Ankara, Turkey

 

 

Behavioural Effects of Phenylalanine-Free Amino Acid Tablet Supplementation in Patients with Untreated PKU (Part II) 

Study done by:

  • Department of Paediatrics, Nutrition & Metabolism Unit, Hacettepe University, Ankara, Turkey

  • The John F. Kennedy Institute, Glostrup, Denmark

 

 

Brain Phenylalanine Concentrations in PKU: Research and Treatment of Adults (Latest Edition)

Pediatrics 2003;112:1575-1579

  • Rex A. Moats, Ph.D.

  • Kathrine D. Moesley, M.S. R.D

  • Richard Koch, M.D.

  • Marvin Nelson, M.D.

 

 

Classical Treatment Vs. Tyrosine And Tryptophan Supplement in PKU Similar Effects on Plasma Branched Chain Amino Acid Levels

 

SSIEM, Prague 2001

  • Troels Kjær

  • Kirsten Ahring, B.S

  • Ingrid Mikkelsen, B.S

  • Maria Jose Miranda Gimenez-Rico, M.D., Ph.D

  • Flemming Güttler, M.D., Ph.D

  • Hans Lou, M.D., Ph.D

 

 

Cognitive Performance in Adolescent PKU Patients on Relaxed Diet Supplemented with Phenylalanine-Free Amino Acid Tablets Vs. Strict Dietary Therapy (Part I) 

 

Study done by:

  • Jente Andersen, M.Sc

  • Kirsten Ahring, B.S

  • Ingrid Mikkelsen, B.S

  • Maria Jose Miranda Gimenez-Rico, M.D., Ph.D

  • Flemming Güttler, M.D., Ph.D

  • Hans Lou, M.D., Ph.D

 

 

Cognitive Performance in Adolescent PKU Patients on Relaxed Diet Supplemented with Phenylalanine-Free Amino Acid Tablets Vs. Strict Dietary Therapy (Part II) 

Study done by:

  • Jente Andresen, M.Sc

  • Kirsten Ahring, B.S

  • Ingrid Mikkelsen, B.S

  • Maria Jose Miranda Gimenez-Rico, M.D., Ph.D

  • Flemming Güttler, M.D., Ph.D.

  • Hans Lou, MD, Ph.D.

 

 

Diet With Large Neutral Amino Acids Supplementation As A Combined Treatment For Difficult To Control Or Late Diagnosed Patients with PKU – Preliminary Data 

Study done by:

  • M.Gizewska

  • L. Cyrylowski

  • I. Józwiak

  • W. Bich

  • H. Romanowska

  • M. Remigolska

  • A. Bilar

  • E. Bartkowiak

  • M. Walczak

 

 

Effects of Diet Discontinuation and Dietary Tryptophan Supplementation on Neurotransmitter Metabolism in Phenylketonuria 

Brain Dysfunction 1988;1:51-56

  • Jytte Beiber Nielsen

  • Hans Lou

  • Flemming Güttler

At The John F. Kennedy Institute, Glostrup, Denmark

 

 

Effects of Dietary mixtures of Amino Acids on fetal growth and maternal and fetal amino acid pools in experimental  maternal Phenylketonuria

Am J Clin Nutr 1999;69:687-96

  • Richard E Austic

  • Chun-Li Su

  • Barbara J Strupp

  • David A Levitsky

 

 

Future Role of Large Neutral Amino Acids in Transport of Phenylalanine Into the Brain First Edition

Pediatrics 2003;112(6): 1570-1574

  • Reuben Matalon M.D., Ph.D

  • Sankar Surendran, Ph.D

  • Kimberlee Michals Matalon, Ph.D., R.D

  • Stephen Tyring, M.D., Ph.D

  • Michael Quast, Ph.D

  • Wei Jinga, Ph.D

  • Edward Ezell, Ph.D

  • Sylvia Szucs, MS

 

 

Future Role of Large Neutral Amino Acids in Transport of Phenylalanine Into the Brain Latest Edition

Pediatrics 2003;112:1570-1574

  • Reuben Matalon M.D., Ph.D

  • Sankar Surendran, Ph.D

  • Kimberlee Michals Matalon, Ph.D., R.D

  • Stephen Tyring, M.D., Ph.D

  • Michael Quast, Ph.D

  • Wei Jinga, Ph.D

  • Edward Ezell, Ph.D

  • Sylvia Szucs, MS

 

 

Increased Neurotransmitter Biosynthesis in Phenylketonuria Induced by Phenylalanine Restriction or By Supplementation of Unrestricted Diet with Large Amounts of Tyrosine

Study done by:

  • C. Lykkelund

  • J. B. Nielsen

  • H. C. Lou

  • V. Rasmussen

  • A.M. Gerdes

  • E. Christensen

  • F. Güttler

 

 

Increased Vigilance and Dopamine Synthesis by Large Doses of Tyrosine or Phenylalanine Restriction in Phenylketonuria

Acta Paediatr Scand. 1987; 76: 560-565

  • C. Lykkelund

  • H. Udesen

  • H. C. Lou

  • P. Bruhn

  • A.M. Gerdes

 

 

Large Neutral Amino Acids Block Phenylalanine Transport Into Brain Tissue In Patients With Phenylketonuria

J Clin Invest. 1999;103:1169-1178

  • Joachim Pietz

  • Roland Kreis

  • André Rupp

  • Ertan Mayatepek

  • Chris Boesch

  • Hans Joachim Bremer

 


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