title: Body composition in children with galactosaemia.
abstract: 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.
authors.affiliation: Dept. of Paediatrics and Laboratory of Inherited Metabolic Diseases, University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: B
authors.initials: B
authors.lastname: Panis
authors.forename: P Ph
authors.initials: PP
authors.lastname: Forget
authors.forename: F H
authors.initials: FH
authors.lastname: Nieman
authors.forename: M J P G
authors.initials: MJ
authors.lastname: van Kroonenburgh
authors.forename: M E
authors.initials: ME
authors.lastname: Rubio-Gozalbo
mesh_terms: D015502:Absorptiometry, Photon; D000293:Adolescent; D001823:Body Composition; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D004032:Diet; D005260:Female; D005693:Galactosemias / Q000378:metabolism / Q000503:physiopathology*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008297:Male; D015233:Models, Statistical; D012044:Regression Analysis; D013025:Glycine max
chemical_list: D007334:Insulin-Like Growth Factor I