1. Preface

   • Horst Bickel
   • Peter Burgard
   • Reinhild Link

   EditorialNotes Pages: S1 - S1

2. The first treatment of phenylketonuria

   • H. Bickel

   OriginalPaper Pages: S2 - S3

3. The introduction of newborn screening for phenylketonuria

   • R. Guthrie
   • H. Bickel

   OriginalPaper Pages: S4 - S5

4. The influence of mutations on enzyme activity and phenylalanine tolerance in phenylalanine hydroxylase deficiency

   • F. Güttler
   • P. Guldberg

   OriginalPaper Pages: S6 - S10

5. Phenylalanine hydroxylase genotypes, predicted residual enzyme activity and phenotypic parameters of diagnosis and treatment of phenylketonuria

   • P. Burgard
   • A. Rupp
   • U. Lichter-Konecki

   OriginalPaper Pages: S11 - S15

6. Gene therapy for phenylketonuria

   • R. C. Eisensmith
   • S. L. C. Woo

   OriginalPaper Pages: S16 - S19

7. Maternal non-phenylketonuric mild hyperphenylalaninemia

   • H. L. Levy
   • S. E. Waisbren
   • F. de la Cruz

   OriginalPaper Pages: S20 - S25

8. Untreated non-phenylketonuric-hyperphenylalaninaemia: intellectual and neurological outcome

   • J. Weglage
   • K. Ullrich
   • H. G. Koch

   OriginalPaper Pages: S26 - S28

9. Summary of findings from the United States Collaborative Study of children treated for phenylketonuria

   • C. Azen
   • R. Koch
   • K. Fishier

   OriginalPaper Pages: S29 - S32

10. Intellectual development of the patients of the German Collaborative Study of children treated for phenylketonuria

    • P. Burgard
    • E. Schmidt
    • H. J. Bremer

    OriginalPaper Pages: S33 - S38

11. Long-term follow up of patients with classical phenylketonuria after diet relaxation at 5 years of age

    • F. Rey
    • V. Abadie
    • J. Rey

    OriginalPaper Pages: S39 - S44

12. Austrian report on longitudinal outcome in phenylketonuria

    • S. Scheibenreiter
    • M. Tiefenthaler
    • M. Schadler

    OriginalPaper Pages: S45 - S49

13. A longitudinal study of phenylketonuria based on the data of the Budapest Screening Center

    • Á. Schuler
    • Cs. Somogyi
    • A. Nagy

    OriginalPaper Pages: S50 - S52

14. Longitudinal study on early diagnosis and treatment of phenylketonuria in Poland

    • M. B. Cabalska
    • I. Nowaczewska
    • K. Zorska

    OriginalPaper Pages: S53 - S55

15. Intellectual and school performance in adolescents with phenylketonuria according to their dietary compliance

    • J. Zeman
    • A. Pijackova
    • J. Hyanek

    OriginalPaper Pages: S56 - S58

16. Long-term follow-up of patients treated for phenylketonuria (PKU)

    • P. Çechák
    • L. Hejcmanová
    • A. Rupp

    OriginalPaper Pages: S59 - S63

17. Studies of multimodal evoked potentialsin treated phenylketonuria: the pattern of vulnerability

    • A. C. Ludolph
    • U. Vetter
    • K. Ullrich

    OriginalPaper Pages: S64 - S68

18. Magnetic resonance imaging of the brain in adolescents with phenylketonuria and in one case of 6-pyruvoyl tetrahydropteridine synthase deficiency

    • J. Pietz
    • U. K. Meyding-Lamadé
    • H. Schmidt

    OriginalPaper Pages: S69 - S73

19. Effect of l-dopa on visual evoked potentials and neuropsychological tests in adult phenylketonuria patients

    • K. Ullrich
    • J. Weglage
    • J. P. Colombo

    OriginalPaper Pages: S74 - S77

20. Neurotransmitter positron emission tomographic-studies in adults with phenylketonuria, a pilot study

    • A. M. J. Paans
    • J. Pruim
    • K. Ullrich

    OriginalPaper Pages: S78 - S81

21. Effects of concurrent phenylalanine levels on sustained attention and calculation speed in patients treated early for phenylketonuria

    • E. Schmidt
    • P. Burgard
    • A. Rupp

    OriginalPaper Pages: S82 - S86

22. A prefrontal dysfunction model of early-treated phenylketonuria

    • M. C. Welsh

    OriginalPaper Pages: S87 - S89

23. Care of the adult with phenylketonuria

    • R. Koch
    • C. Azen
    • T. Lin

    OriginalPaper Pages: S90 - S92

24. Phenylketonuria: treatment in adolescence and adult life

    • D. P. Brenton
    • M. Lilburn
    • J. C. Cabrera-Abreu

    OriginalPaper Pages: S93 - S96

25. Intelligence and professional career in young adults treated early for phenylketonuria

    • H. Schmidt
    • P. Burgard
    • A. Rupp

    OriginalPaper Pages: S97 - S100

26. Psychosocial aspects in phenylketonuria

    • J. Weglage
    • B. Flinders
    • E. Schmidt

    OriginalPaper Pages: S101 - S104

27. Towards self-monitoring and self-treatment in phenylketonuria — a way to better diet compliance

    • U. Wendel
    • U. Langenbeck

    OriginalPaper Pages: S105 - S107

28. Amino acid composition of food products used in the treatment of patients with disorders of the amino acid and protein metabolism

    • H. J. Bremer
    • A. Anninos
    • B. Schulz

    OriginalPaper Pages: S108 - S114

29. Utilisation of amino acid mixtures in adolescents with phenylketonuria

    • E. Mönch
    • M. E. Herrmann
    • M. Keller

    OriginalPaper Pages: S115 - S120

30. Recommendations for protein and energy intakes by patients with phenylketonuria

    • P. B. Acosta

    OriginalPaper Pages: S121 - S124

31. Recommendations for protein and amino acid intake in phenylketonuric patients

    • F. Cockburn
    • B. J. Clark

    OriginalPaper Pages: S125 - S129

32. Recommendations for protein and amino acid intake in phenylketonuria patients

    • H. Przyrembel

    OriginalPaper Pages: S130 - S131

33. Fatty acid metabolism in phenylketonuria

    • M. Giovannini
    • C. Agostoni
    • E. Riva

    OriginalPaper Pages: S132 - S135

34. Nutrition studies in treated infants and children with phenylketonuria: vitamins, minerals, trace elements

    • P. B. Acosta

    OriginalPaper Pages: S136 - S139

35. Selenium status in infants and children with phenylketonuria and in maternal phenylketonuria

    • I. Lombeck
    • F. Jochum
    • K. Terwolbeck

    OriginalPaper Pages: S140 - S144

36. Vitamin B₁₂ deficiency in adolescents and young adults with phenylketonuria

    • W. B. Hanley
    • A. S. J. Feigenbaum
    • V. J. Austin

    OriginalPaper Pages: S145 - S147

37. Decreased bone mineralization in children with phenylketonuria under treatment

    • L. Hillman
    • C. Schlotzhauer
    • R. Hillman

    OriginalPaper Pages: S148 - S152

38. Comparison of the protein quality of dietetically treated phenylketonuria patients with the recommendations of the WHO expert consultation

    • G. Krauch
    • E. Müller
    • H. J. Bremer

    OriginalPaper Pages: S153 - S157

39. The international collaborative study on maternal phenylketonuria: organization, study design and description of the sample

    • E. Gross Friedman
    • R. Koch
    • F. de la Cruz

    OriginalPaper Pages: S158 - S161

40. Outcome implications of the International Maternal Phenylketonuria Collaborative Study (MPKUCS): 1994

    • R. Koch
    • H. Levy
    • F. de la Cruz

    OriginalPaper Pages: S162 - S164

41. Nutrition and reproductive outcome in maternal phenylketonuria

    • K. Michals
    • P. B. Acosta
    • C. Azen

    OriginalPaper Pages: S165 - S168

42. The North American Maternal Phenylketonuria Collaborative Study, developmental assessment of the offspring: preliminary report

    • W. B. Hanley
    • R. Koch
    • F. de la Cruz

    OriginalPaper Pages: S169 - S172

43. German Maternal Phenylketonuria Study

    • S. Cipcic-Schmidt
    • F. K. Trefz
    • K. Ullrich

    OriginalPaper Pages: S173 - S176

44. Maternal phenylketonuria

    • D. P. Brenton
    • M. Lilburn

    OriginalPaper Pages: S177 - S180