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Journal of Inherited Metabolic Disease

, Volume 28, Issue 6, pp 931–937 | Cite as

Body composition in children with galactosaemia

  • B. Panis
  • P. Ph. Forget
  • F. H. Nieman
  • M. J. P. G. van Kroonenburgh
  • M. E. RubioGozalbo
Article

Summary

Body composition in classical galactosaemia has not been studied. Patients with classical galactosaemia, an inherited disorder of galactose metabolism caused by deficiency of galactose-1-phosphate uridyltransferase (GALT, EC 2.7.7.10), might be at risk for an abnormal body composition because of intrinsic factors related to galactosaemia and/or diet-related factors. The aim of this study was to evaluate the body composition of children with classical galactosaemia. The studied population was a previously reported group of classical galactosaemia patients (13 male and 27 female, ages 3–17 years) with decreased height, weight, weight-for-height and insulin-like growth factor-I (IGF-I) Z-scores. Body composition data were obtained by dual-energy X-ray absorptiometry (DXA). In order to correct for height, fat mass (FM) and lean tissue mass (LTM) were divided by squared height. Mid-parental target height Z-scores were assessed and compared to actual height Z-scores. Linear and multiple regression analysis were done to investigate the relationship between body composition and IGF-I, dietary intake and growth data. We found decreased height Z-scores when compared to mid-parental target height Z-scores. Mean scores for FM and LTM (both adjusted for height) were decreased. LTM (adjusted for height) and height Z-score were correlated with IGF-I Z-score. FM (adjusted for height) was correlated with soy intake. No correlation was found between soy intake and IGF-I Z-score. In this limited group of patients, height is decreased and body composition is abnormal. The decreased levels of IGF-I and/or soy nutrition might play a role in these findings.

Keywords

Body Composition Galactose Intrinsic Factor Growth Data Limited Group 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© SSIEM and Springer 2005

Authors and Affiliations

  • B. Panis
    • 1
  • P. Ph. Forget
    • 2
  • F. H. Nieman
    • 3
  • M. J. P. G. van Kroonenburgh
    • 4
  • M. E. RubioGozalbo
    • 1
  1. 1.Dept. of Paediatrics and Laboratory of Inherited Metabolic DiseasesUniversity Hospital MaastrichtMaastrichtThe Netherlands
  2. 2.Department of PaediatricsUniversity Hospital MaastrichtMaastrichtThe Netherlands
  3. 3.Clinical Epidemiology and Technology Assessment (KEMTA)University Hospital MaastrichtMaastrichtThe Netherlands
  4. 4.Nuclear MedicineUniversity Hospital MaastrichtMaastrichtThe Netherlands

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