Literature DB >> 8828621

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

H Schmidt1, P Burgard, J Pietz, A Rupp.   

Abstract

The intellectual status and professional careers of 51 young adults with phenylketonuria whose treatment started before 3 months of age are described. Their mean IQ was 97 (SD = 16). Of the IQs, 4% were more than 2 SD below the norm. The distribution of types of schooling of the patients was comparable to that in the German population. The professional careers of nearly all the patients were according to their educational level. Within the sample the outcome was significantly correlated with phenylalanine (Phe) control, even when the patients' social background was statistically taken into account. The main influence of Phe on intelligence seems to occur during the first decade of life since IQ data remain stable even after Phe levels increased during adolescence.

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Year:  1996        PMID: 8828621     DOI: 10.1007/pl00014262

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  16 in total

1.  Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria.

Authors:  I Smith; M G Beasley; A E Ades
Journal:  Arch Dis Child       Date:  1991-03       Impact factor: 3.791

2.  Intellectual and school performances in early-treated classical PKU patients. The French collaborative study.

Authors:  J M Saudubray; F Rey; H Ogier; V Abadie; J P Farriaux; J Ghisolfi; P Guibaud; J Rey; M Vidailhet
Journal:  Eur J Pediatr       Date:  1987       Impact factor: 3.183

3.  The effects of diet discontinuation in children with phenylketonuria.

Authors:  R Koch; C G Azen; N Hurst; E G Friedman; K Fishler
Journal:  Eur J Pediatr       Date:  1987       Impact factor: 3.183

Review 4.  Diet termination in children with phenylketonuria: a review of psychological assessments used to determine outcome.

Authors:  S E Waisbren; R R Schnell; H L Levy
Journal:  J Inherit Metab Dis       Date:  1980       Impact factor: 4.982

5.  Effect of stopping low-phenylalanine diet on intellectual progress of children with phenylketonuria.

Authors:  I Smith; M E Lobascher; J E Stevenson; O H Wolff; H Schmidt; S Grubel-Kaiser; H Bickel
Journal:  Br Med J       Date:  1978-09-09

6.  School performance and intellectual outcome in adolescents with phenylketonuria.

Authors:  J Weglage; B Fünders; B Wilken; D Schubert; K Ullrich
Journal:  Acta Paediatr       Date:  1993 Jun-Jul       Impact factor: 2.299

7.  Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria.

Authors:  N A Holtzman; R A Kronmal; W van Doorninck; C Azen; R Koch
Journal:  N Engl J Med       Date:  1986-03-06       Impact factor: 91.245

8.  Intellectual development in 12-year-old children treated for phenylketonuria.

Authors:  C G Azen; R Koch; E G Friedman; S Berlow; J Coldwell; W Krause; R Matalon; E McCabe; M O'Flynn; R Peterson
Journal:  Am J Dis Child       Date:  1991-01

9.  Successful adjustment to society by adults with phenylketonuria.

Authors:  R Koch; M Yusin; K Fishler
Journal:  J Inherit Metab Dis       Date:  1985       Impact factor: 4.982

10.  Loss of intellectual function in children with phenylketonuria after relaxation of dietary phenylalanine restriction.

Authors:  M R Seashore; E Friedman; R A Novelly; V Bapat
Journal:  Pediatrics       Date:  1985-02       Impact factor: 7.124

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  10 in total

1.  Influence of knowledge of the disease on metabolic control in phenylketonuria.

Authors:  Jolita Bekhof; Francjan J van Spronsen; Matty R Crone; Margreet van Rijn; Carin G M Oudshoorn; Paul H Verkerk
Journal:  Eur J Pediatr       Date:  2003-04-08       Impact factor: 3.183

2.  Effect of genotype on changes in intelligence quotient after dietary relaxation in phenylketonuria and hyperphenylalaninaemia.

Authors:  L G Greeves; C C Patterson; D J Carson; R Thom; M C Wolfenden; J Zschocke; C A Graham; N C Nevin; E R Trimble
Journal:  Arch Dis Child       Date:  2000-03       Impact factor: 3.791

3.  Neuropsychological outcome of experimental manipulation of phenylalanine intake in treated phenylketonuria.

Authors:  P Griffiths; N Ward; A Harvie; F Cockburn
Journal:  J Inherit Metab Dis       Date:  1998-02       Impact factor: 4.982

4.  Transition of young adults with phenylketonuria from pediatric to adult care.

Authors:  Ulrike Mütze; Annika Roth; Johannes F W Weigel; Skadi Beblo; Christoph G Baerwald; Peter Bührdel; Wieland Kiess
Journal:  J Inherit Metab Dis       Date:  2011-02-09       Impact factor: 4.982

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

Authors:  F Rey; V Abadie; F Plainguet; J Rey
Journal:  Eur J Pediatr       Date:  1996-07       Impact factor: 3.183

Review 6.  The truth of treating patients with phenylketonuria after childhood: the need for a new guideline.

Authors:  F J van Spronsen; P Burgard
Journal:  J Inherit Metab Dis       Date:  2008-08-12       Impact factor: 4.982

7.  Social outcome in treated individuals with inherited metabolic disorders: UK study.

Authors:  M Bhat; C Haase; P J Lee
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.750

8.  High phenylalanine levels directly affect mood and sustained attention in adults with phenylketonuria: a randomised, double-blind, placebo-controlled, crossover trial.

Authors:  Amber E ten Hoedt; Leo M J de Sonneville; Baudouin Francois; Nienke M ter Horst; Mirian C H Janssen; M Estela Rubio-Gozalbo; Frits A Wijburg; Carla E M Hollak; Annet M Bosch
Journal:  J Inherit Metab Dis       Date:  2010-12-10       Impact factor: 4.982

9.  Parenting a child with phenylketonuria or galactosemia: implications for health-related quality of life.

Authors:  Amber E ten Hoedt; Heleen Maurice-Stam; Carolien C A Boelen; M Estela Rubio-Gozalbo; Francjan J van Spronsen; Frits A Wijburg; Annet M Bosch; Martha A Grootenhuis
Journal:  J Inherit Metab Dis       Date:  2011-02-03       Impact factor: 4.982

10.  Efficacy and safety of sapropterin dihydrochloride in patients with phenylketonuria: A meta-analysis of randomized controlled trials.

Authors:  Jinghan Qu; Ting Yang; Ente Wang; Min Li; Chaoyang Chen; Lingyun Ma; Ying Zhou; Yimin Cui
Journal:  Br J Clin Pharmacol       Date:  2019-03-18       Impact factor: 4.335

  10 in total

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