Adjustment and intelligence among children with phenylketonuria in Sweden

被引:10
作者
Lundstedt, G [1 ]
Johansson, A
Melin, L
Alm, J
机构
[1] Huddinge Univ Hosp, Karolinska Inst, Dept Paediat, SE-14186 Stockholm, Sweden
[2] Huddinge Univ Hosp, Karolinska Inst, Dept Med Lab Sci & Technol, Ctr Inherited Metab Dis, SE-14186 Stockholm, Sweden
[3] Uppsala Univ, Dept Psychol, S-75148 Uppsala, Sweden
关键词
adjustment; compliance; intelligence; phenylketonuria;
D O I
10.1080/080352501317061558
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
In this investigation we present a recent survey of treatment effects among 8 to 19-y-old Swedish children and youths with phenylketonuria (PKU). The results from intelligence tests and a questionnaire comprising four scales, work capacity, social competence, and internalising and externalising problems, were used. Severity of disease, early treatment, and contemporary phenylalanine levels were assessed. The results showed that development of the patients' intelligence was normal. Adjustments were rated by the patients, their parents and their teachers, and the results were compared with those of a healthy reference group. The PKU patients did not differ from the reference group except for externalising problems judged by the children themselves. None of the patients with PKU showed signs of externalising problems in contrast to the reference group, where such behaviour was observed. Patients with severe PKU, however, showed less social competence compared with patients with a milder form of the disease, according to their own and their parents' ratings. Phenylalanine level in blood tests was in accordance with treatment norms, although the teenagers had higher levels of phenylalanine than the younger patients. Conclusion: Normal intelligence and adjustment is found among patients with PKU when plasma phenylalanine levels are within treatment norms.
引用
收藏
页码:1147 / 1152
页数:6
相关论文
共 30 条
[1]  
[Anonymous], 1990, Metaphors of mind: Conceptions of the nature of intelligence
[2]   Summary of findings from the United States Collaborative Study of children treated for phenylketonuria [J].
Azen, C ;
Koch, R ;
Friedman, E ;
Wenz, E ;
Fishler, K .
EUROPEAN JOURNAL OF PEDIATRICS, 1996, 155 :S29-S32
[3]   Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997 [J].
Burgard, P ;
Bremer, HJ ;
Bührdel, P ;
Clemens, PC ;
Mönch, E ;
Przyrembel, H ;
Trefz, FK ;
Ullrich, K .
EUROPEAN JOURNAL OF PEDIATRICS, 1999, 158 (01) :46-54
[4]   Intellectual development of the patients of the German Collaborative Study of children treated for phenylketonuria [J].
Burgard, P ;
Schmidt, E ;
Rupp, A ;
Schneider, W ;
Bremer, HJ .
EUROPEAN JOURNAL OF PEDIATRICS, 1996, 155 :S33-S38
[5]   PSYCHOPATHOLOGY OF PATIENTS TREATED EARLY FOR PHENYLKETONURIA - RESULTS OF THE GERMAN COLLABORATIVE STUDY OF PHENYLKETONURIA [J].
BURGARD, P ;
ARMBRUSTER, M ;
SCHMIDT, E ;
RUPP, A .
ACTA PAEDIATRICA, 1994, 83 :108-110
[6]  
BURGARD P, 1996, EUR J PEDIATR S1, V155, P53
[7]   Long-term follow-up of patients treated for phenylketonuria (PKU) - Results from the Prague PKU Center [J].
Cechak, P ;
Hejcmanova, L ;
Rupp, A .
EUROPEAN JOURNAL OF PEDIATRICS, 1996, 155 :S59-S63
[8]  
ELMAN JL, 1996, RETHINKING INNATENES, pCH1
[9]   Effect of genotype on changes in intelligence quotient after dietary relaxation in phenylketonuria and hyperphenylalaninaemia [J].
Greeves, LG ;
Patterson, CC ;
Carson, DJ ;
Thom, R ;
Wolfenden, MC ;
Zschocke, J ;
Graham, CA ;
Nevin, NC ;
Trimble, ER .
ARCHIVES OF DISEASE IN CHILDHOOD, 2000, 82 (03) :216-221
[10]  
Griffiths P, 1997, AM J MENT RETARD, V102, P27, DOI 10.1352/0895-8017(1997)102<0027:THICWC>2.0.CO