'Hypotyrosinemia' in phenylketonuria

Molecular Genetics and Metabolism

Volumn 69, Issue 4, 2000, Pages 286-294

  Hanley, W B ^a   Lee, A W ^a   Hanley, A J G ^a   Lehotay, D C ^a   Austin, V J ^a   Schoonheyt, W E ^a   Platt, B A ^a   Clarke, J T R ^a  

^a HOSPITAL FOR SICK CHILDREN   (Canada)

Author keywords

Learning disabilities; Neurotransmitters; Phenylalanine; Phenylketonuria; PKU; Tyrosine

Indexed keywords

NEUROTRANSMITTER; PHENYLALANINE; TYROSINE;

ADOLESCENT; ADULT; ARTICLE; CHILD; CONTROLLED STUDY; HUMAN; INFANT; LEARNING DISORDER; MAJOR CLINICAL STUDY; MEMBRANE TRANSPORT; PHENYLKETONURIA; PRIORITY JOURNAL; TYROSINEMIA;

EID: 0034107456     PISSN: 10967192     EISSN: None     Source Type: Journal    
DOI: 10.1006/mgme.2000.2985     Document Type: Article

References (54)

1. N Holtzman R Kronmal W van Doornink C Azen R Koch Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria N Engl J Med 314 1986 593 598
2. ER Naughten B Kiely I Saul D Murphy Phenylketonuria: Outcome and problems in a diet for life clinic Eur J Pediatr 146 1987 A23 24
3. C Netley WB Hanley HL Rudner Phenylketonuria and it's variants: Observations on intellectual functioning Can Med Assoc J 131 1984 751 755
4. I Smith MG Beasley AE Ades Intelligence and quality of dietary treatment in phenylketonuria Arch Dis Child 65 1990 472 478
5. BF Pennington WJ van Doornink LL McCabe E RB McCabe Neuropsychological defects in early treated phenylketonuric children Am J Mental Defects 89 1985 467 474
6. HK Berry DJ O'Grady LJ Perlmutter MK Bofinger Intellectual development and academic achievements of children treated early for phenylketonuria Dev Med Child Neurol 21 1979 311 320
7. L MJ De Sonneville E Schmidt U Michel Information processing in early treated phenylketonuria J Clin Exp Neuropsychol 11 1989 53
8. CG Azen R Koch E Gross Friedman S Berlow J Coldwell W Krause Intellectual development in 12 year old children treated for phenylketonuria Am J Dis Child 145 1991 35 39
9. F Guttler Hyperphenylalaninemia Acta Paediatr Scand 280 1980 1 80
10. MC Welsh BF Pennington S Ozonoff B Rouse E RB McCabe Neuropsychology of early treated phenylketonuria: Specific executive function deficits Child Dev 61 1990 1697 1713
11. AC Diamond V Ciaramitaro E Donner Prefrontal cortex cognitive deficits in early treated phenylketonuria: Results of a longitudinal study in children and of an animal model Soc Neurosci Abstr 18 1992 1063
12. HC Lou C Lykkelund AM Gerdes H Udesen P Bruhn Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria Acta Paediatr Scan 76 1987 560 565
13. W Krause M Halminski L McDonald P Dembure R Salvo D Friedes L Elsas Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria J Clin Invest 75 1985 40 48
14. M Salter RG Knowles CI Pogson Quantification of the importance of individual steps in the control of aromatic amino acid metabolism Biochem J 234 1986 635
15. R Koch HL Levy R Matalon B Rouse WB Hanley The international collaborative study of maternal phenylketonuria: Status report 1994 Acta Paediatr 407 1994 111 119
16. K Fukada Y Nishi T Usui Free amino acid concentration in plasma, erythrocytes, granulocytes and lymphocytes in umbilical cord blood, children and adolescents J Pediatr Gastroenterol Nutr 3 1984 432 439
17. JC Dickinson H Rosenblum PB Hamilton Ion exchange chromatography of the free amino acids in the plasma of infants <2500 gm at birth Pediatrics 45 1970 606 612
18. T Tikanoja O Simell M Viikari AL Jarvenpaa Plasma amino acids in term neonates after a feed of human milk or formula Acta Paediatr Scand 71 1982 391 397
19. JC Dickinson H Rosenblum PB Hamilton Ion exchange chromatography of the free amino acids in the plasma of the newborn infant Pediatrics 36 1965 2 13
20. P YK Wu N Edwards MC Storm plasma amino acid patterns in normal term breast fed infants J Pediatr 109 1986 347 349
21. DM Gregory D Sovetts CL Clow CR Scriver Plasma free amino acid values in children and adolescents Metabolism 35 1986 967 969
22. CR Scriver DM Gregory D Sovetts G Tissenbaum Normal plasma free amino acid values in adults: The influence of some common physiological variables Metabolism 34 1985 868 873
23. CR Scriver CL Clow P Lamm Plasma amino acids; screening, quantitation and interpretation Am J Clin Nutr 24 1971 876 890
24. MD Armstrong U Stave A study of plasma free amino acid levels. II Normal values for children and adults Metabolism 4 1973 561 569
25. L Hagenfeldt L Bjerkenstedt G Edman G Sedvall F-A Wiesel Amino acids in plasma and CSF and monoamine metabolites in CSF: Interrelationship in healthy subjects J Neurochem 3 1984 833 837
26. SAS Procedures Guide 1990 SAS Institute Cary
27. CR Scriver S Kaufman RC Eisensmith S LC Woo The Hyperphenylalaninemias CR Scriver AL Beaudet WS Sly D Valle The Metabolic and Molecular Basis of Inherited Disease 1995 McGraw-Hill New York
28. SE Snyderman P Norton LE Holt Effects of tyrosine administration on phenylketonuria Fed Am Soc Exp Biol Proc 14 1955 450 451
29. SP Bessman The justification theory: The essential nature of the non-essential amino acids Nutr Rev 7 1979 209 220
30. ML Batshaw D Valle SP Bessman Unsuccessful treatment of phenylketonuria with tyrosine J Pediatr 99 1981 159 161
31. M Brouwer PK de Bree FJ van Sprang SK Wadman Low Serum Tyrosine in patients with phenylketonuria on dietary therapy Lancet 1 1977 1162
32. P Koepp KR Held Serum Tyrosine in patients with hyperphenylalaninemia Lancet 2 1977 92 93
33. E Kindt HA Lunde LR Gjessing S Halvorsen SO Lie Fasting plasma amino acid concentrations in PKU children on two different levels of protein intake Acta Paediatr Scand 77 1988 60 66
34. AM Nord L McCabe E RB McCabe Biochemical and nutritional status of children with hyperphenylalaninemia J Inher Metab Dis 11 1988 431 432
35. B Francios M Diels M de la Brassinne Iatrogenic skin lesions in PKU children due to low tyrosine intake J Inher Metab Dis 12 1989 333 334
36. FA Hommes K Matsuo On a possible mechanism of abnormal brain development in experimental hyperphenylalaninemia Neurochem Int 11 1987 1 10
37. E Schmidt A Rupp P Burgard J Pietz J Weglage L de Sommeville Sustained attention in adult phenylketonuria: The influence of the concurrent phenylalanine-blood level J Clin Exp Neuropsychol 16 1994 681 688
38. Arch Dis Child 68 1993 426 427
39. NA Peterson SN Shah E Raghupathy DE Rhoads Brain Res 272 1985 189 193
40. HC Lou Large doses of tryptophan & tyrosine as potential therapeutic alternative to dietary phenylalanine restriction in phenylketonuria Lancet 2 1985 150 151
41. HC Lou F Guttler C Lykkelund P Bruhl A Niederwieser Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents Eur J Pediatr 144 1985 17 20
42. C Lykkelund JB Nielsin HC Lou V Rasmussen AM Gerdes E Christensen F Guttler Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine Eur J Pediatr 148 1988 238 245
43. J TR Clarke RD Gates SE Hogan M Barrett GW MacDonald Neuropsychological studies on adolescents with phenylketonuria returned to phenylalanine restricted diets Am J Mental Retard 92 1987 255 262
44. FA Hommes JS Lee The control of 5-hydroxytryptamine and dopamine synthesis in the brain: A theoretical approach J Inher Metab Dis 13 1990 37 57
45. O Sheil N Duignan IP Saul ER Naughton Maternal phenylketonuria with increased tyrosine supplements J Inher Metab Dis 59 1986 231 233
46. MM Mazzoco S Yannicelli AM Nord W van Doorninck AJ Davidson-Mundt CL Green Cognition and tyrosine supplementation among school-aged children with phenylketonuria Am J Dis Child 146 1992 1261 1264
47. Pietz, J, Landwehr, R, Kutscha, A, Schmidt, H, de Sonneville, L. Trefz FK. Effect of high-dose tyosine supplementation on brain function in adults with phenylketonuria. J Pediatr127:936–943, 199.
48. ML Smith WB Hanley J TR Clarke P Klim W Schoonheyt V Austin DC Lehotay Randomized controlled trial of tyrosine supplementation on neuropsychological performance in phenylketonuria Arch Dis Child 78 1998 116 121
49. S Wasser KU Ettrich KD Schmidt H Theile Case studies of the effect of tyrosine administration in children with phenylketonuria on cognitive processes Klin Padiatr 204 1992 417 421
50. J Pietz R Kreis A Rupp E Mayatepek D Rating C Boesch HJ Bremer Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria J Clin Invest 103 1999 1117 1169
51. FJ Rohr D Lobbregt HL Levy Tyrosine supplementation in the treatment of maternal phenylketonuria Am J Clin Nutr 67 1998 473 476
52. FJ van Spronsen M van Rijn T van Dijk G PA Smit DJ Reijngoud R Berger H AS Heymans Plasma phenylalanine and tyrosine responses to different nutritional conditions fasting/postprandial) in patients with phenylketonuria: Effect of sample timing Pediatrics 92 1993 570 573
53. FJ van Spronsen T van Dijk PA Smit M van Rijn DJ Reijngould R Berger H AS Heymans Large daily fluctuations in plasma tyrosine in treated patients with phenylketonuria Am J Clin Nutr 64 1996 916 921
54. AM Gerdes JB Nielson H Lou F Guttler Plasma amino acids in phenylketonuric children treated either with phenylalanine-free amino acids or a protein hydrolysate Acta Paediatr Scand 79 1990 69 72