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Volumn 158, Issue 1, 1999, Pages 46-54

Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997

Author keywords

Hyperphenylalaninaemia; Phenylalanine levels; Phenylketonuria; Treatment recommendations

Indexed keywords

PHENYLALANINE;

EID: 0033003339     PISSN: 03406199     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004310051008     Document Type: Article
Times cited : (128)

References (91)
  • 1
    • 0026949736 scopus 로고
    • Evolution intellectuelle après relâchement du régime à lâge de 5 ans dans la phénylcétonurie typique
    • Abadie V, Rey F, Plainguet F, Rey J (1992) Evolution intellectuelle après relâchement du régime à lâge de 5 ans dans la phénylcétonurie typique. Arch Fr Pediatr 49:773-778
    • (1992) Arch Fr Pediatr , vol.49 , pp. 773-778
    • Abadie, V.1    Rey, F.2    Plainguet, F.3    Rey, J.4
  • 2
    • 0029786951 scopus 로고    scopus 로고
    • Summary of findings from the United States Collaborative Study of children treated for phenylketonuria
    • Azen C, Koch R, Friedman E, Wenz E, Fishler K (1996) Summary of findings from the United States Collaborative Study of children treated for phenylketonuria. Eur J Pediatr 155 [Suppl 1]:S29-S32
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Azen, C.1    Koch, R.2    Friedman, E.3    Wenz, E.4    Fishler, K.5
  • 3
    • 0028269579 scopus 로고
    • Outcome of treatment in young adults with phenylketonuria detected by routine neonatal screening between 1964 and 1971
    • Beasley MG, Costello PM, Smith I (1994) Outcome of treatment in young adults with phenylketonuria detected by routine neonatal screening between 1964 and 1971. Q J Med 87:155-160
    • (1994) Q J Med , vol.87 , pp. 155-160
    • Beasley, M.G.1    Costello, P.M.2    Smith, I.3
  • 4
    • 0026056827 scopus 로고
    • Disturbed myelination in patients with treated hyperphenylalaninaemia: Evaluation with magnetic resonance imaging
    • Bick U, Fahrendorf G, Ludolph AC, Vassallo P, Weglage J, Ullrich K (1991) Disturbed myelination in patients with treated hyperphenylalaninaemia: evaluation with magnetic resonance imaging. Eur J Pediatr 150:185-189
    • (1991) Eur J Pediatr , vol.150 , pp. 185-189
    • Bick, U.1    Fahrendorf, G.2    Ludolph, A.C.3    Vassallo, P.4    Weglage, J.5    Ullrich, K.6
  • 5
    • 0027442717 scopus 로고
    • White matter abnormalities in patients with treated hyperphenylalaninemia: Magnetic resonance relaxometry and proton spectroscopy findings
    • Bick U, Ullrich K, Stöber U, Möller H, Schuierer G, Ludolph AC, Oberwitter C, Weglage J, Wendel U (1993) White matter abnormalities in patients with treated hyperphenylalaninemia: magnetic resonance relaxometry and proton spectroscopy findings. Eur J Pediatr 152:1012-1020
    • (1993) Eur J Pediatr , vol.152 , pp. 1012-1020
    • Bick, U.1    Ullrich, K.2    Stöber, U.3    Möller, H.4    Schuierer, G.5    Ludolph, A.C.6    Oberwitter, C.7    Weglage, J.8    Wendel, U.9
  • 6
    • 0023186649 scopus 로고
    • Phenylketonuria and sustained attention: The continuous performance test
    • Brunner RL, Berry HK (1987) Phenylketonuria and sustained attention: the Continuous Performance Test. Int J Clin Neuropsychol 9:68-70
    • (1987) Int J Clin Neuropsychol , vol.9 , pp. 68-70
    • Brunner, R.L.1    Berry, H.K.2
  • 7
    • 0020636194 scopus 로고
    • Early-treated phenylketonuria: Neuropsychologic consequences
    • Brunner RL, Jordan MK, Berry HK (1983) Early-treated phenylketonuria: neuropsychologic consequences. J Pediatr 102:831-835
    • (1983) J Pediatr , vol.102 , pp. 831-835
    • Brunner, R.L.1    Jordan, M.K.2    Berry, H.K.3
  • 8
    • 0023387816 scopus 로고
    • Phenylketonuria and complex spatial visualization: An analysis of information processing
    • Brunner RL, Berch DB, Berry H ( 1987) Phenylketonuria and complex spatial visualization: an analysis of information processing. Dev Med Child Neurol 29:460-468
    • (1987) Dev Med Child Neurol , vol.29 , pp. 460-468
    • Brunner, R.L.1    Berch, D.B.2    Berry, H.3
  • 9
    • 0028631183 scopus 로고
    • Psychopathology of patients treated early for phenylketonuria: Results of the German collaborative study of phenylketonuria
    • Burgard P, Armbruster M, Schmidt E, Rupp A (1994) Psychopathology of patients treated early for phenylketonuria: results of the German collaborative study of phenylketonuria. Acta Paediatr 83 [Suppl 407]:108-110
    • (1994) Acta Paediatr , vol.83 , Issue.SUPPL. 407 , pp. 108-110
    • Burgard, P.1    Armbruster, M.2    Schmidt, E.3    Rupp, A.4
  • 10
    • 0031018037 scopus 로고    scopus 로고
    • Neuropsychological functions of early treated patients with phenylketonuria on and off diet. Results of a cross-national and cross-sectional study
    • Burgard P, Rey F, Rupp A, Abadie V, Rey J (1997) Neuropsychological functions of early treated patients with phenylketonuria on and off diet. Results of a cross-national and cross-sectional study. Pediatr Res 41:368-374
    • (1997) Pediatr Res , vol.41 , pp. 368-374
    • Burgard, P.1    Rey, F.2    Rupp, A.3    Abadie, V.4    Rey, J.5
  • 11
    • 31344482675 scopus 로고    scopus 로고
    • Intellectual development of the patients of the German collaborative study of children treated for phenylketonuria
    • Burgard P, Schmidt E, Rupp A, Schneider W, Bremer HJ (1996) Intellectual development of the patients of the German collaborative study of children treated for phenylketonuria. Eur J Pediatr 155 [Suppl 1]:S33-S38
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Burgard, P.1    Schmidt, E.2    Rupp, A.3    Schneider, W.4    Bremer, H.J.5
  • 12
    • 0023627217 scopus 로고
    • Neuropsychological studies in adolescents with phenylketonuria returned to phenylalanine-restricted diets
    • Clarke JTR, Gates RD, Hogan SE, Barrett M, MacDonald GW (1987) Neuropsychological studies in adolescents with phenylketonuria returned to phenylalanine-restricted diets. Am J Ment Retard 92 (3):255-262
    • (1987) Am J Ment Retard , vol.92 , Issue.3 , pp. 255-262
    • Clarke, J.T.R.1    Gates, R.D.2    Hogan, S.E.3    Barrett, M.4    MacDonald, G.W.5
  • 14
    • 0029095727 scopus 로고
    • Magnetic resonance imaging in phenylketonuria: Reversal of cerebral white matter change
    • Cleary MA, Walter JH, Wraith JE, White F, Tyler K, Jenkins JPR (1995) Magnetic resonance imaging in phenylketonuria: reversal of cerebral white matter change. J Pediatr 127 (2):251-255
    • (1995) J Pediatr , vol.127 , Issue.2 , pp. 251-255
    • Cleary, M.A.1    Walter, J.H.2    Wraith, J.E.3    White, F.4    Tyler, K.5    Jenkins, J.P.R.6
  • 16
    • 0028631947 scopus 로고
    • Phenylalanine levels of 6-10 mg/dl may not be as benign as once thought
    • Diamond A (1994) Phenylalanine levels of 6-10 mg/dl may not be as benign as once thought. Acta Paediatr 83 [Suppl 407]:89-91
    • (1994) Acta Paediatr , vol.83 , Issue.SUPPL. 407 , pp. 89-91
    • Diamond, A.1
  • 17
    • 0346937769 scopus 로고    scopus 로고
    • Prefrontal cognitive deficits in children treated early and continuously for PKU
    • Diamond A, Prevor MB, Callendar G, Druin DP (1997). Prefrontal cognitive deficits in children treated early and continuously for PKU. Monogr Soc Res Child Dev 62 (4):1-208
    • (1997) Monogr Soc Res Child Dev , vol.62 , Issue.4 , pp. 1-208
    • Diamond, A.1    Prevor, M.B.2    Callendar, G.3    Druin, D.P.4
  • 18
    • 0022448037 scopus 로고
    • Neuropsychological functioning in treated phenylketonuria
    • Faust D, Libon D, Pueschel S (1986) Neuropsychological functioning in treated phenylketonuria. Int J Psychiatr Med 16:169-177
    • (1986) Int J Psychiatr Med , vol.16 , pp. 169-177
    • Faust, D.1    Libon, D.2    Pueschel, S.3
  • 20
    • 0028806360 scopus 로고
    • Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria
    • Griffiths P, Paterson L, Harvie A (1995) Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria. J Intellect Disabil Res 39 (5):365-372
    • (1995) J Intellect Disabil Res , vol.39 , Issue.5 , pp. 365-372
    • Griffiths, P.1    Paterson, L.2    Harvie, A.3
  • 21
    • 0001325347 scopus 로고
    • Inverse diurnal variation of serum phenylalanine and tyrosine in phenylketonuric children on low-phenylalanine diet
    • Allan JD, Holt KS, Ireland JT, Pollitt, RJ (eds) Enzymopenic anaemias, lysosomes and other papers. Livingstone, Edinburgh
    • Güttler F, Oleson ES, Wamberg E (1969) Inverse diurnal variation of serum phenylalanine and tyrosine in phenylketonuric children on low-phenylalanine diet. In: Allan JD, Holt KS, Ireland JT, Pollitt, RJ (eds) Enzymopenic anaemias, lysosomes and other papers. Proceedings of the Sixth Symposium of the Society for the Study of Inborn Errors of Metabolism. Livingstone, Edinburgh, pp 149-158
    • (1969) Proceedings of the Sixth Symposium of the Society for the Study of Inborn Errors of Metabolism , pp. 149-158
    • Güttler, F.1    Oleson, E.S.2    Wamberg, E.3
  • 23
    • 0023355001 scopus 로고
    • Use of restricted protein diet in the treatment of behavior disorder in a severely mentally retarded adult female phenylketonuric patient
    • Harper M, Reid AH (1987) Use of restricted protein diet in the treatment of behavior disorder in a severely mentally retarded adult female phenylketonuric patient. J Ment Defic Res 31:209-212
    • (1987) J Ment Defic Res , vol.31 , pp. 209-212
    • Harper, M.1    Reid, A.H.2
  • 24
    • 0029585416 scopus 로고
    • The use of a low phenylalanine diet in response to the challenging behaviour of a man with untreated phenylketonuria and profound learning disabilities
    • Harvey EL, Kirk SF (1995) The use of a low phenylalanine diet in response to the challenging behaviour of a man with untreated phenylketonuria and profound learning disabilities. J Intellect Disabil Res 39:520-526
    • (1995) J Intellect Disabil Res , vol.39 , pp. 520-526
    • Harvey, E.L.1    Kirk, S.F.2
  • 26
    • 0028795157 scopus 로고
    • Some reservations about clinical guidelines
    • Hopkins A (1995) Some reservations about clinical guidelines. Arch Dis Child 72:70-75
    • (1995) Arch Dis Child , vol.72 , pp. 70-75
    • Hopkins, A.1
  • 27
    • 0026607024 scopus 로고
    • The use of a low phenylalanine diet with amino acid supplement in the treatment of behavioral problems in a severely mentally retarded adult female with phenylketonuria
    • Hoskin RG, Sasitharan T, Howard R (1992) The use of a low phenylalanine diet with amino acid supplement in the treatment of behavioral problems in a severely mentally retarded adult female with phenylketonuria. J Intellect Dis Res 36:183-191
    • (1992) J Intellect Dis Res , vol.36 , pp. 183-191
    • Hoskin, R.G.1    Sasitharan, T.2    Howard, R.3
  • 28
    • 0018376668 scopus 로고
    • Synaptic density in human frontal cortex - Developmental changes and effects of aging
    • Huttenlocher P (1979) Synaptic density in human frontal cortex - developmental changes and effects of aging. Brain Res 163:195-205
    • (1979) Brain Res , vol.163 , pp. 195-205
    • Huttenlocher, P.1
  • 29
    • 0021905289 scopus 로고
    • Preliminary results for the oral administration of valine isoleucine and leucine for phenylketonuria
    • Jordan MK, Brunner RL, Hunt MM, Berry HK (1985) Preliminary results for the oral administration of valine isoleucine and leucine for Phenylketonuria. Dev Med Child Neurol 27:33-39
    • (1985) Dev Med Child Neurol , vol.27 , pp. 33-39
    • Jordan, M.K.1    Brunner, R.L.2    Hunt, M.M.3    Berry, H.K.4
  • 31
    • 0023617622 scopus 로고
    • The effects of diet discontinuation in children with phenylketonuria
    • Koch R, Azen CG, Hurst N, Friedman EG, Fishier K (1987) The effects of diet discontinuation in children with phenylketonuria. Eur J Pediatr 146 [Suppl 1]:A12-A16
    • (1987) Eur J Pediatr , vol.146 , Issue.SUPPL. 1
    • Koch, R.1    Azen, C.G.2    Hurst, N.3    Friedman, E.G.4    Fishier, K.5
  • 32
    • 0021990680 scopus 로고
    • Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man
    • Krause W, Halminski M, McDonald L, Dembure Ph, Salvo R, Freides D, Elsas L (1985) Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man. J Clin Invest 75:40-48
    • (1985) J Clin Invest , vol.75 , pp. 40-48
    • Krause, W.1    Halminski, M.2    McDonald, L.3    Dembure, Ph.4    Salvo, R.5    Freides, D.6    Elsas, L.7
  • 33
    • 0028029176 scopus 로고
    • Relation between phenylalanine hydroxylase genotypes and phenotypic parameters of diagnosis and treatment of hyperphenylalaninemic disorders
    • Lichter-Konecki U, Rupp A, Konecki DS, Trefz FK, Schmidt H, Burgard P (1994) Relation between phenylalanine hydroxylase genotypes and phenotypic parameters of diagnosis and treatment of hyperphenylalaninemic disorders. J Inherit Metab Dis 17:362-365
    • (1994) J Inherit Metab Dis , vol.17 , pp. 362-365
    • Lichter-Konecki, U.1    Rupp, A.2    Konecki, D.S.3    Trefz, F.K.4    Schmidt, H.5    Burgard, P.6
  • 34
    • 0021797643 scopus 로고
    • Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents
    • Lou HC, Güttler F, Lykkelund C, Bruhn P, Niederwieser A (1985) Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents. Eur J Pediatr 144:17-20
    • (1985) Eur J Pediatr , vol.144 , pp. 17-20
    • Lou, H.C.1    Güttler, F.2    Lykkelund, C.3    Bruhn, P.4    Niederwieser, A.5
  • 35
    • 0023225583 scopus 로고
    • Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria
    • Lou HC, Lykkelund C, Gerdes A-M, Udesen H, Bruhn P (1987) Increased vigilance and dopamine synthesis by large doses of tyrosine or phenylalanine restriction in phenylketonuria. Acta Paediatr Scand 76:560-565
    • (1987) Acta Paediatr Scand , vol.76 , pp. 560-565
    • Lou, H.C.1    Lykkelund, C.2    Gerdes, A.-M.3    Udesen, H.4    Bruhn, P.5
  • 37
    • 0026566711 scopus 로고
    • Neurological outcome in 22 treated adolescents with hyperphenylalaninemia
    • Ludolph AC, Ullrich K, Nedjat S, Masur H, Bick U (1992) Neurological outcome in 22 treated adolescents with hyperphenylalaninemia. Acta Neurol Scand 85:243-248
    • (1992) Acta Neurol Scand , vol.85 , pp. 243-248
    • Ludolph, A.C.1    Ullrich, K.2    Nedjat, S.3    Masur, H.4    Bick, U.5
  • 38
    • 31344482657 scopus 로고    scopus 로고
    • Studies of multimodal evoked potentials in treated phenylketonuria: The pattern of vulnerability
    • Ludolph AC, Vetter U, Ullrich K (1996) Studies of multimodal evoked potentials in treated phenylketonuria: the pattern of vulnerability. Eur J Pediatr 155 [Suppl 1]:S64-S68
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Ludolph, A.C.1    Vetter, U.2    Ullrich, K.3
  • 39
    • 0030048431 scopus 로고    scopus 로고
    • Factors affecting the variation in plasma phenylalanine in patients with phenylketonuria on diet
    • MacDonald A, Rylance G, Hall SK, Asplin D, Booth IW (1996) Factors affecting the variation in plasma phenylalanine in patients with phenylketonuria on diet. Arch Dis Child 74:412-417
    • (1996) Arch Dis Child , vol.74 , pp. 412-417
    • MacDonald, A.1    Rylance, G.2    Hall, S.K.3    Asplin, D.4    Booth, I.W.5
  • 40
    • 0017948814 scopus 로고
    • Effects of diet and behavior therapy on social and motor behavior of retarded phenylketonuric adults: An experimental analysis
    • Marholin D, Pohl RE, Stewart RM, Touchette PE, Townsend NM, Kolodny EH (1978) Effects of diet and behavior therapy on social and motor behavior of retarded phenylketonuric adults: an experimental analysis. Pediatr Res 12:179-187
    • (1978) Pediatr Res , vol.12 , pp. 179-187
    • Marholin, D.1    Pohl, R.E.2    Stewart, R.M.3    Touchette, P.E.4    Townsend, N.M.5    Kolodny, E.H.6
  • 42
    • 0027397393 scopus 로고
    • Phenylketonuria due to phenylalanine hydroxylase deficiency: An unfolding story
    • Medical Research Council Working Party on Phenylketonuria (1993) Phenylketonuria due to phenylalanine hydroxylase deficiency: an unfolding story. BMJ 306:115-119
    • (1993) BMJ , vol.306 , pp. 115-119
  • 43
    • 0027533685 scopus 로고
    • Recommendations on the dietary management of phenylketonuria
    • Medical Research Council Working Party on Phenylketonuria (1993) Recommendations on the dietary management of phenylketonuria. Arch Dis Child 68:426-427
    • (1993) Arch Dis Child , vol.68 , pp. 426-427
  • 44
    • 0019377180 scopus 로고
    • Linguistic development of children with phenylketonuria and normal intelligence
    • Melnick CR, Michals KK, Matalon R (1981) Linguistic development of children with phenylketonuria and normal intelligence. J Pediatr 98 (2):269-272
    • (1981) J Pediatr , vol.98 , Issue.2 , pp. 269-272
    • Melnick, C.R.1    Michals, K.K.2    Matalon, R.3
  • 45
    • 0029130812 scopus 로고
    • In-vivo NMR spectroscopy in patients with phenylketonuria: Changes of cerebral phenylalanine levels under dietary treatment
    • Möller HE, Vermathen P, Ullrich K, Weglage J, Koch H-G, Peters PE (1995) In-vivo NMR spectroscopy in patients with phenylketonuria: Changes of cerebral phenylalanine levels under dietary treatment. Neuropediatrics 26:199-202
    • (1995) Neuropediatrics , vol.26 , pp. 199-202
    • Möller, H.E.1    Vermathen, P.2    Ullrich, K.3    Weglage, J.4    Koch, H.-G.5    Peters, P.E.6
  • 46
    • 0344765524 scopus 로고    scopus 로고
    • Blood-brain barrier phenylalanine transport and individual vulnerability in phenylketonuria
    • (in press)
    • Möller HE, Weglage J, Wiederman D, Ullrich K (in press) Blood-brain barrier phenylalanine transport and individual vulnerability in phenylketonuria. J Cereb Blood Flow Metab, 1998, 18:1184-1191
    • (1998) J Cereb Blood Flow Metab , vol.18 , pp. 1184-1191
    • Möller, H.E.1    Weglage, J.2    Wiederman, D.3    Ullrich, K.4
  • 48
    • 0025352280 scopus 로고
    • Speech and language skills in children with early treated phenylketonuria
    • Ozanne AE, Krimmer H, Murdoch BE (1990) Speech and language skills in children with early treated phenylketonuria. Am J Ment Retard 94:625-632
    • (1990) Am J Ment Retard , vol.94 , pp. 625-632
    • Ozanne, A.E.1    Krimmer, H.2    Murdoch, B.E.3
  • 51
    • 0013614681 scopus 로고    scopus 로고
    • Phenylketonuria: An interdisciplinary survey of management over 40 years
    • Phenylketonuria: an interdisciplinary survey of management over 40 years (1996) Eur J Pediatr 155 [Suppl 1]:
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
  • 52
    • 0023926724 scopus 로고
    • Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early
    • Pietz J, Benninger Ch, Schmidt H, Scheffner D, Bickel H (1988) Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early. Eur J Pediatr 147:361-67
    • (1988) Eur J Pediatr , vol.147 , pp. 361-367
    • Pietz, J.1    Benninger, Ch.2    Schmidt, H.3    Scheffner, D.4    Bickel, H.5
  • 53
    • 0027500145 scopus 로고
    • EEGs in Phenylketonuria I: Follow-up to adulthood; II: Short-term diet-related changes in EEGs and cognitive function
    • Pietz J, Schmidt E, Matthis P, Kobialka B, Kutscha A, Sonneville L de (1993) EEGs in Phenylketonuria I: Follow-up to adulthood; II: Short-term diet-related changes in EEGs and cognitive function. Dev Med Child Neurol 35:54-64
    • (1993) Dev Med Child Neurol , vol.35 , pp. 54-64
    • Pietz, J.1    Schmidt, E.2    Matthis, P.3    Kobialka, B.4    Kutscha, A.5    De Sonneville, L.6
  • 54
    • 0028785556 scopus 로고
    • The dynamics of brain concentrations of phenylalanine and its clinical significance in patients with phenylketonuria determined by in vivo 1H magnetic-resonance spectroscopy
    • Pietz J, Kreis R, Boesch C, Penzien J, Rating D, Herschkowitz N (1995) The dynamics of brain concentrations of phenylalanine and its clinical significance in patients with phenylketonuria determined by in vivo 1H magnetic-resonance spectroscopy. Pediatr Res 38:657-663
    • (1995) Pediatr Res , vol.38 , pp. 657-663
    • Pietz, J.1    Kreis, R.2    Boesch, C.3    Penzien, J.4    Rating, D.5    Herschkowitz, N.6
  • 55
    • 0029954988 scopus 로고    scopus 로고
    • Phenylketonuria: Findings at MR imaging and localized in vivo H-1 MR spectroscopy of the brain in patients with early treatment
    • Pietz J, Kreis R, Schmidt H, Meyding-Lamade UK, Rupp A, Boesch C (1996) Phenylketonuria: findings at MR imaging and localized in vivo H-1 MR spectroscopy of the brain in patients with early treatment. Radiology 201:413-420
    • (1996) Radiology , vol.201 , pp. 413-420
    • Pietz, J.1    Kreis, R.2    Schmidt, H.3    Meyding-Lamade, U.K.4    Rupp, A.5    Boesch, C.6
  • 56
    • 31344482665 scopus 로고    scopus 로고
    • Magnetic resonance imaging of the brain in adolescents with phenylketonuria and in one case of 6-pyruvoyl tetrahydropteridine synthase deficiency
    • Pietz J, Meyding-Lamadé UK, Schmidt H (1996) Magnetic resonance imaging of the brain in adolescents with phenylketonuria and in one case of 6-pyruvoyl tetrahydropteridine synthase deficiency. Eur J Pediatr 155 [Suppl 1]:S69-S73
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Pietz, J.1    Meyding-Lamadé, U.K.2    Schmidt, H.3
  • 58
    • 0015221479 scopus 로고
    • The natural history of untreated phenylketonuria
    • Pitt D (1971) The natural history of untreated phenylketonuria. Med J Aust 58:378-383
    • (1971) Med J Aust , vol.58 , pp. 378-383
    • Pitt, D.1
  • 59
    • 0025745106 scopus 로고
    • The natural history of untreated phenylketonuria over 20 years
    • Pitt DB, Danks DM (1991) The natural history of untreated phenylketonuria over 20 years. J Paediatr Child Health 27:189-190
    • (1991) J Paediatr Child Health , vol.27 , pp. 189-190
    • Pitt, D.B.1    Danks, D.M.2
  • 60
    • 26944443956 scopus 로고    scopus 로고
    • Long-term follow up of patients with classical phenylketonuria after diet relaxation at 5 years of age
    • Rey F, Abadie V, Plainguet F, Rey J (1996) Long-term follow up of patients with classical phenylketonuria after diet relaxation at 5 years of age. Eur J Pediatr 155 [Suppl 1]:S39-S44
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Rey, F.1    Abadie, V.2    Plainguet, F.3    Rey, J.4
  • 61
    • 0029024835 scopus 로고
    • Comparison of different indices of dietary control in phenylketonuria
    • Rupp A, Burgard P (1995) Comparison of different indices of dietary control in phenylketonuria. Acta Paediatr 84:521-527
    • (1995) Acta Paediatr , vol.84 , pp. 521-527
    • Rupp, A.1    Burgard, P.2
  • 62
    • 0027985225 scopus 로고
    • Sustained attention in adult phenylketonuria: The influence of the concurrent phenylalanine-blood-level
    • Schmidt E, Rupp A, Burgard P, Pietz J, Weglage J, Sonneville L de (1994) Sustained attention in adult phenylketonuria: the influence of the concurrent phenylalanine-blood-level. J Clin Exp Neuropsychol 16:681-688
    • (1994) J Clin Exp Neuropsychol , vol.16 , pp. 681-688
    • Schmidt, E.1    Rupp, A.2    Burgard, P.3    Pietz, J.4    Weglage, J.5    De Sonneville, L.6
  • 63
    • 0029809096 scopus 로고    scopus 로고
    • Effects of concurrent phenylalanine levels on sustained attention and calculation speed in patients treated early for phenylketonuria (PKU)
    • Schmidt E, Burgard P, Rupp A (1996) Effects of concurrent phenylalanine levels on sustained attention and calculation speed in patients treated early for phenylketonuria (PKU). Eur J Pediatr 155 [Suppl 1]:S82-S186
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Schmidt, E.1    Burgard, P.2    Rupp, A.3
  • 64
    • 0029786709 scopus 로고    scopus 로고
    • Intelligence and professional career in young adults treated early for phenylketonuria
    • Schmidt H, Burgard P, Pietz J, Rupp A (1996) Intelligence and professional career in young adults treated early for phenylketonuria. Eur J Pediatr 155 [Suppl 1]:S97-S100
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Schmidt, H.1    Burgard, P.2    Pietz, J.3    Rupp, A.4
  • 66
    • 0021991742 scopus 로고
    • Loss of intellectual function in children with phenylketonuria after relaxation of dietary phenylalanine restriction
    • Seashore MR, Friedman E, Novelly RA, Bapat V (1985) Loss of intellectual function in children with phenylketonuria after relaxation of dietary phenylalanine restriction. Pediatr 75:226-232
    • (1985) Pediatr , vol.75 , pp. 226-232
    • Seashore, M.R.1    Friedman, E.2    Novelly, R.A.3    Bapat, V.4
  • 67
    • 0003073254 scopus 로고
    • The hyperphenylalaninemias
    • Lloyd JK Scriver CR (eds). Butterworth, London
    • Smith I (1985) The hyperphenylalaninemias In: Lloyd JK Scriver CR (eds) Genetic and metabolic disease in pediatrics. Butterworth, London, pp 166-209
    • (1985) Genetic and Metabolic Disease in Pediatrics , pp. 166-209
    • Smith, I.1
  • 68
    • 0024333597 scopus 로고
    • Intelligence and behavior in children with early treated phenylketonuria
    • Smith I, Beasley M (1989) Intelligence and behavior in children with early treated phenylketonuria. Eur J Clin Nutr 43:1-5
    • (1989) Eur J Clin Nutr , vol.43 , pp. 1-5
    • Smith, I.1    Beasley, M.2
  • 69
    • 0023840351 scopus 로고
    • Behavior disturbances in 8-year-old children with early treated phenylketonuria
    • Smith I, Beasley MG, Wolff OH, Ades AE (1988) Behavior disturbances in 8-year-old children with early treated phenylketonuria. J Pediatr 112:403-408
    • (1988) J Pediatr , vol.112 , pp. 403-408
    • Smith, I.1    Beasley, M.G.2    Wolff, O.H.3    Ades, A.E.4
  • 70
    • 0025346883 scopus 로고
    • Intelligence and quality of dietary treatment in phenylketonuria
    • Smith I, Beasley MG, Ades AE (1990) Intelligence and quality of dietary treatment in phenylketonuria. Arch Dis Child 65:472-478
    • (1990) Arch Dis Child , vol.65 , pp. 472-478
    • Smith, I.1    Beasley, M.G.2    Ades, A.E.3
  • 71
    • 0026087542 scopus 로고
    • Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria
    • Smith I, Beasley MG, Ades AE (1991) Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria. Arch Dis Child 65:311-316
    • (1991) Arch Dis Child , vol.65 , pp. 311-316
    • Smith, I.1    Beasley, M.G.2    Ades, A.E.3
  • 72
    • 0025062789 scopus 로고
    • Preliminary neuropsychological test results of the German phenylketonuria study
    • Sonneville LMJ de, Schmidt E, Michel U, Batzler U (1990) Preliminary neuropsychological test results of the German phenylketonuria study. Eur J Pediatr 149 [Suppl 1]:S39-S44
    • (1990) Eur J Pediatr , vol.149 , Issue.SUPPL. 1
    • De Sonneville, L.M.J.1    Schmidt, E.2    Michel, U.3    Batzler, U.4
  • 73
    • 0024991159 scopus 로고
    • Stellungnahme der arbeitsgemeinschaft für pädiatrische stoffwechselstörungen (APS) zur diätetischen behandlung der phenylketonurie
    • Stellungnahme der Arbeitsgemeinschaft für Pädiatrische Stoffwechselstörungen (APS) zur diätetischen Behandlung der Phenylketonurie (1990). Monatsschr Kinderheilkd 138:636
    • (1990) Monatsschr Kinderheilkd , vol.138 , pp. 636
  • 78
    • 0028868539 scopus 로고
    • Discordant phenylketonuria phenotypes in one family: The relationship between genotype and clinical outcome is a function of multiple effects
    • Tyfield LA, Zschocke J, Stephenson A, Cockburn F, Harvie A, Bidwell JL, Wood NA, Hunt LP (1995) Discordant phenylketonuria phenotypes in one family: the relationship between genotype and clinical outcome is a function of multiple effects. J Med Genet 32:867-870
    • (1995) J Med Genet , vol.32 , pp. 867-870
    • Tyfield, L.A.1    Zschocke, J.2    Stephenson, A.3    Cockburn, F.4    Harvie, A.5    Bidwell, J.L.6    Wood, N.A.7    Hunt, L.P.8
  • 80
    • 0027430130 scopus 로고
    • Plasma phenylalanine and tyrosine response to different nutritional conditions (fasting/postprandial) in patients with phenylketonuria: Effect of sampling timing
    • Van Spronsen FJ, van Rijn M, van Dijk T, Smit GPA, Reijngoud DJ, Berger R, Heymans HSA (1993) Plasma phenylalanine and tyrosine response to different nutritional conditions (fasting/postprandial) in patients with phenylketonuria: effect of sampling timing. Pediatrics 92:570-573
    • (1993) Pediatrics , vol.92 , pp. 570-573
    • Van Spronsen, F.J.1    Van Rijn, M.2    Van Dijk, T.3    Smit, G.P.A.4    Reijngoud, D.J.5    Berger, R.6    Heymans, H.S.A.7
  • 82
    • 0028609668 scopus 로고
    • Review of neuropsychological functioning in treated phenylketonuria: An information processing approach
    • Waisbren SE, Brown MJ, Sonneville LMJ de, Levy HL (1994) Review of neuropsychological functioning in treated phenylketonuria: an information processing approach. Acta Paediatr 83 [Suppl 407]:98-103
    • (1994) Acta Paediatr , vol.83 , Issue.SUPPL. 407 , pp. 98-103
    • Waisbren, S.E.1    Brown, M.J.2    De Sonneville, L.M.J.3    Levy, H.L.4
  • 84
    • 0027314636 scopus 로고
    • School performance and intellectual outcome in adolescents with phenylketonuria
    • Weglage J, Fünders B, Wilken B, Schubert D, Ullrich K (1993) School performance and intellectual outcome in adolescents with phenylketonuria. Acta Paediatr 81:582-586
    • (1993) Acta Paediatr , vol.81 , pp. 582-586
    • Weglage, J.1    Fünders, B.2    Wilken, B.3    Schubert, D.4    Ullrich, K.5
  • 85
    • 0028203239 scopus 로고
    • Personality characteristics in patients with phenylketonuria treated early
    • Weglage J, Rupp A, Schmidt E (1994) Personality characteristics in patients with phenylketonuria treated early. Pediatr Res 35:611-613
    • (1994) Pediatr Res , vol.35 , pp. 611-613
    • Weglage, J.1    Rupp, A.2    Schmidt, E.3
  • 86
    • 0030046062 scopus 로고    scopus 로고
    • Deficits in selective and sustained attention processes in early treated children with phenylketonuria - Result of impaired frontal lobe functions?
    • Weglage J, Pietsch M, Fünders B, Koch HG, Ullrich K (1996) Deficits in selective and sustained attention processes in early treated children with phenylketonuria - result of impaired frontal lobe functions? Eur J Pediatr 155:200-204
    • (1996) Eur J Pediatr , vol.155 , pp. 200-204
    • Weglage, J.1    Pietsch, M.2    Fünders, B.3    Koch, H.G.4    Ullrich, K.5
  • 88
    • 0029786186 scopus 로고    scopus 로고
    • Untreated non-Phenylketonuric-hyperphenylalaninemia: Intellectual and neurological outcome
    • Weglage J, Ullrich K, Pietsch M, Fünders B, Zaß R, Koch HG (1996) Untreated non-Phenylketonuric-hyperphenylalaninemia: intellectual and neurological outcome. Eur J Pediatr 155 [Suppl 1]:S26-S28
    • (1996) Eur J Pediatr , vol.155 , Issue.SUPPL. 1
    • Weglage, J.1    Ullrich, K.2    Pietsch, M.3    Fünders, B.4    Zaß, R.5    Koch, H.G.6
  • 89
    • 0025580566 scopus 로고
    • Neuropsychology of early-treated phenylketonuria: Specific executive function deficits
    • Welsh MC, Pennington BF, Oznoff S, Rouse B, McCabe ERB (1990) Neuropsychology of early-treated phenylketonuria: specific executive function deficits. Child Dev 61:1697-1713
    • (1990) Child Dev , vol.61 , pp. 1697-1713
    • Welsh, M.C.1    Pennington, B.F.2    Oznoff, S.3    Rouse, B.4    McCabe, E.R.B.5
  • 90
    • 0029070025 scopus 로고
    • Improvements in behaviour and physical manifestations in previously untreated adults with phenylketonuria using a phenylalanine-restricted diet: A national survey
    • Yannicelli S, Ryan A (1995) Improvements in behaviour and physical manifestations in previously untreated adults with phenylketonuria using a phenylalanine-restricted diet: a national survey. J Inherit Metab Dis 18:131-134
    • (1995) J Inherit Metab Dis , vol.18 , pp. 131-134
    • Yannicelli, S.1    Ryan, A.2
  • 91
    • 0029056452 scopus 로고
    • Editorial comment on phenylketonuria
    • Zetterström R (1995) Editorial comment on phenylketonuria Acta Pediatr 84:716-718
    • (1995) Acta Pediatr , vol.84 , pp. 716-718
    • Zetterström, R.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.