title: Body composition in young patients with galactose metabolic disorders: a preliminary report.
abstract: OBJECTIVE
  Body composition evaluation in patients with galactosemia and its variants.
  
  
  METHODS
  The body composition of young subjects with galactose metabolic disorders was analyzed with dual X-ray absorptiometry. The subjects were divided into the classic galactosemia (CG; n=14) group and the group with other galactose metabolic disorders (OGMD, e.g., epimerase deficiency; n=8).
  
  
  RESULTS
  In both groups, bone strength and bone mineral density Z-scores were normal. However, CG patients appeared sarcopenic (median muscle mass Z-score=-1.93), whereas OGMD subjects had low-normal muscle mass. In addition, approximately half of all patients were overweight. In CG patients, bone mass was strongly correlated to muscle mass (r=0.81), whereas in OGMD patients it was positively correlated to body mass index (r=0.78).
  
  
  CONCLUSIONS
  An imbalance between muscle and fat mass, especially in patients with classic galactosemia, was observed. Given the observed strong correlation between bone and muscle mass, more effective lifestyle counseling is needed.
authors.forename: Artemis
authors.initials: A
authors.lastname: Doulgeraki
authors.forename: Ioannis
authors.initials: I
authors.lastname: Monopolis
authors.forename: Domna
authors.initials: D
authors.lastname: Deligianni
authors.forename: Maria
authors.initials: M
authors.lastname: Kalogerakou
authors.forename: Kleopatra H
authors.initials: KH
authors.lastname: Schulpis
mesh_terms: D015502:Absorptiometry, Photon; D000293:Adolescent; D001823:Body Composition; D002239:Carbohydrate Metabolism, Inborn Errors / Q000000981:diagnostic imaging*; D002648:Child; D005260:Female; D005690:Galactose / Q000378:metabolism*; D006801:Humans; D008297:Male
chemical_list: D005690:Galactose