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

作者: I Smith , M G Beasley , A E Ades

DOI: 10.1136/ADC.66.3.311

关键词:

摘要: A total of 599 children with phenylketonuria, who had been treated early, were followed up prospectively in order to examine the association between intellectual progress from 4 14 years age and control phenylalanine concentrations. The rose around 400 mumol/l during first four above 900 by 12 years. divided into two cohorts: cohort I comprised 224 born United Kingdom 1964 1971 II 375 1972 1978. In a previous study it was shown that these already mean intelligence quotient (IQ) over half standard deviation below general population norms, IQ fell linearly as average concentrations rose. Multiple regression used estimate size associations at later ages periods assessments, after controlling for control, social class, type factors relating diagnosis early management. For each 300 rise those aged 5 8 4-6 points. This compared 7-10 point fall similar phenylalanine. After disappeared but persisted significant 10 age, although smaller than

参考文章(14)
B. Cabalska, N. Duczyńska, J. Borzymowska, K. Zorska, A. Koślacz-Folga, K. Bożkowa, Termination of dietary treatment in phenylketonuria. European Journal of Pediatrics. ,vol. 126, pp. 253- 262 ,(1977) , 10.1007/BF00477051
I Smith, M E Lobascher, J E Stevenson, O H Wolff, H Schmidt, S Grubel-Kaiser, H Bickel, Effect of stopping low-phenylalanine diet on intellectual progress of children with phenylketonuria. BMJ. ,vol. 2, pp. 723- 726 ,(1978) , 10.1136/BMJ.2.6139.723
D. Villasana, I. J. Butler, J. C. Williams, S. M. Roongta, Neurological deterioration in adult phenylketonuria. Journal of Inherited Metabolic Disease. ,vol. 12, pp. 451- 457 ,(1989) , 10.1007/BF01802042
Isabel Smith, Martin G. Beasley, Otto H. Wolff, Anthony E. Ades, Behavior disturbance in 8-year-old children with early treated phenylketonuria The Journal of Pediatrics. ,vol. 112, pp. 403- 408 ,(1988) , 10.1016/S0022-3476(88)80320-2
I Smith, M G Beasley, A E Ades, Intelligence and quality of dietary treatment in phenylketonuria. Archives of Disease in Childhood. ,vol. 65, pp. 472- 478 ,(1990) , 10.1136/ADC.65.5.472
N. Malamud, Neuropathology of Phenylketonuria Journal of Neuropathology and Experimental Neurology. ,vol. 25, pp. 254- 268 ,(1966) , 10.1097/00005072-196604000-00006
Richard Koch, Collen G. Azen, Eva Gross Friedman, Malcolm L. Williamson, Preliminary report on the effects of diet discontinuation in PKU The Journal of Pediatrics. ,vol. 100, pp. 870- 875 ,(1982) , 10.1016/S0022-3476(82)80503-9
CHARLES M. POSER, LUDO VAN BOGAERT, NEUROPATHOLOGIC OBSERVATIONS IN PHENYLKETONURIA Brain. ,vol. 82, pp. 1- 9 ,(1959) , 10.1093/BRAIN/82.1.1-A
A.J Thompson, B.D Youl, B Kendall, A.J Lees, I Smith, D Brenton, G Rylance, D.C Davidson, Neurological deterioration in young adults with phenylketonuria The Lancet. ,vol. 336, pp. 602- 605 ,(1990) , 10.1016/0140-6736(90)93401-A