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Related Experiment Videos

Body composition in children with galactosaemia.

B Panis1, P Ph Forget, F H Nieman

  • 1Dept. of Paediatrics and Laboratory of Inherited Metabolic Diseases, University Hospital Maastricht, Maastricht, The Netherlands.

Journal of Inherited Metabolic Disease
|January 26, 2006
PubMed
Summary

Children with classical galactosaemia have abnormal body composition and decreased height. Insulin-like growth factor-I (IGF-I) levels and soy intake may influence these findings in patients with galactose-1-phosphate uridyltransferase (GALT) deficiency.

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Area of Science:

  • Biochemistry
  • Pediatrics
  • Human Genetics

Background:

  • Classical galactosaemia is an inherited metabolic disorder caused by galactose-1-phosphate uridyltransferase (GALT) deficiency.
  • Patients may experience abnormal body composition due to disease-related factors and diet.
  • Previous studies have not focused on body composition in this population.

Purpose of the Study:

  • To evaluate the body composition of children diagnosed with classical galactosaemia.
  • To investigate potential correlations between body composition, growth parameters, and nutritional intake.

Main Methods:

  • Dual-energy X-ray absorptiometry (DXA) was used to assess body composition (fat mass and lean tissue mass).
  • Measurements were adjusted for height, and compared to mid-parental target height Z-scores.

Related Experiment Videos

  • Regression analysis explored relationships between body composition, insulin-like growth factor-I (IGF-I) Z-scores, and dietary intake (including soy).

    • Main Results:

    • Patients exhibited decreased height Z-scores compared to their mid-parental target height.
    • Adjusted fat mass (FM) and lean tissue mass (LTM) were found to be decreased.
    • LTM and height Z-scores correlated with IGF-I Z-scores, while FM correlated with soy intake.

    Conclusions:

    • Children with classical galactosaemia present with reduced height and altered body composition.
    • Decreased IGF-I levels and/or soy intake may contribute to these observed abnormalities.