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New Studies

Double blind placebo control trial of large neutral amino acids
in treatment of PKU: Effect on blood phenylalanine(PDF Format) Large neutral amino acids (LNAA) have been used on a limited number of patients with phenylketonuria(PKU) with the purpose of decreasing the influx of phenylalanine
(Phe) to the brain. In an open-label study using LNAA, a surprising decline of blood Phe concentration was found in patients with PKU in metabolic treatment centres in Russia, the Ukraine, and the United States.
Double Blind Placebo Control Trial in PKU with NeoPhe(PDF Format) LNAA can reduce blood Phe levels when given with meals. Longer term double-blind placebo control studies are needed. Establishing the efficacy and safety of LNAA
can improve treatment of PKU.
Double Blind Placebo Control Trial in PKU with NeoPhe (Spain)
(PDF Format) LNAA can decrease blood Phe levels in patients with classical PKU. Decreasing of Blood Phe levels is accompanied by increased of blood Tyr levels. The grade of the decrease of Phe levels can be higher after the short period without LNAA. It will be necessary to check the different hypotheses of LNAA effects.
Denmark/Russia/US LNAA Studies(PDF Format) For the first time mixture of LNAA can lower blood phenylalanine. Using NeoPhe avoids lysine deficiency and
negative nitrogen balance. NeoPhe offers new options to treat PKU. Diet can be more liberal with NeoPhe. Still blood phe- levels need to be controlled.
Large neutral amino acids in the treatment of phenylketonuria (PKU)(PDF Format) Large neutral amino acids (LNAAs) have been used on a limited number of patients with phenylketonuria (PKU) with the purpose of decreasing the influx of phenylalanine (Phe) to the brain. In earlier studies on mice with PKU (ENU2/ENU2), LNAAs were given and a surprising decline in blood Phe concentrations was observed. The formula used in the mouse experiment (PreKUnil) lacked lysine.

Past Studies

 

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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