Intellectual performance of children with maple syrup urine disease.
The intellectual performance of 22 children aged 3-16 years with maple syrup urine disease (MSUD) was assessed and compared to a group of early treated phenylketonuria (PKU) children and normal subjects matched by age, sex, nationality, and socio-economic status. All subjects were tested by one examiner only using the age related versions of the non-verbal Snijders-Oomen intelligence test. The mean IQ (+/- SD) score was 74 +/- 14 (range 50-103) in patients with MSUD, 101 +/- 12 (range 87-125) in early treated PKU patients, and 107 +/- 9 (range 90-122) in normal subjects. Intercorrelations indicated that length of time after birth that plasma leucine concentration remained > 1 mmol/l and quality of long-term metabolic control have important influences on IQ.
Hilliges
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C
Department of Paediatrics, Heinrich-Heine University, Düsseldorf, Federal Republic of Germany.
Awiszus
D
D
Wendel
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U
D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D007360:Intelligence; D007361:Intelligence Tests; D016018:Least-Squares Analysis; D007930:Leucine / Q000097:blood; D008297:Male; D008375:Maple Syrup Urine Disease / Q000097:blood / Q000150:complications / Q000523:psychology; D010661:Phenylketonurias
D007930:Leucine