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Intellectual performance of children with maple syrup urine disease

  • Metabolic Diseases
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Abstract

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.

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Abbreviations

MSUD:

maple syrup urine disease

PKU:

phenylketonuria

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Authors and Affiliations

  1. Division of Metabolism, Heinrich-Heine University, Moorenstrasse 5, W-4000, Düsseldorf, Germany

    C. Hilliges & U. Wendel

  2. Division of Psychology, Department of Paediatrics, Heinrich-Heine University, Moorenstrasse 5, W-4000, Düsseldorf, Germany

    D. Awiszus

Authors
  1. C. Hilliges
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  2. D. Awiszus
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  3. U. Wendel
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Hilliges, C., Awiszus, D. & Wendel, U. Intellectual performance of children with maple syrup urine disease. Eur J Pediatr 152, 144–147 (1993). https://doi.org/10.1007/BF02072492

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  • DOI: https://doi.org/10.1007/BF02072492

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