title: Effect of calcium, vitamins K1 and D3 on bone in galactosemia.
abstract: Classical galactosemia is an inherited disorder of galactose metabolism. Recently, diminished bone mineral content (BMC) in children and adolescents has been found. The aim of this study was to evaluate the effect of calcium, vitamins K(1) and D(3) supplementation on bone in children with galactosemia. A 2-year randomized, double-blind, placebo-controlled clinical trial was undertaken in which 40 children with classical galactosemia (13 males and 27 females, aged 3-17 years) were included to receive daily either 750 mg calcium, 1.0 mg vitamin K(1) and 10.0 microg vitamin D(3) or placebo. BMC of femoral neck, lumbar spine and total body and body composition data were determined by dual energy X-ray absorptiometry (DXA) at baseline and after 1 and 2 years. Diet was assessed using a food frequency questionnaire and a 3-day food diary. Biochemical measurements were determined at baseline and after 1 and 2 years. In the children receiving treatment, carboxylated osteocalcin (cOC) concentration significantly increased (P < 0.001) and undercarboxylated osteocalcin (ucOC) concentration significantly decreased (P = 0.001) when compared to the children receiving placebo. Furthermore, there was a statistically significant increase in BMC of lumbar spine (P = 0.001), lean tissue mass (LTM: P = 0.016) and fat mass (FM: P = 0.014) in the treatment group when compared to the placebo group. The significant increase in cOC and decrease in ucOC concentration in the treatment group were present in prepubertal (P < 0.001 and P = 0.006 respectively) and pubertal children (P = 0.004 and P = 0.042 respectively). The significant increase in BMC of lumbar spine in the treatment group was present only in the prepubertal children (P = 0.015). Supplementation of calcium, vitamins K(1) and D(3) given in this dose (750 mg, 1.0 mg and 10.0 mug respectively) is likely to have a role in the treatment of BMC abnormalities in galactosemia.
authors.affiliation: Dept. of Pediatrics, University Hospital Maastricht, Maastricht, The Netherlands; Dept. of Laboratory Genetic Metabolic Diseases, University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Bianca
authors.initials: B
authors.lastname: Panis
authors.affiliation: Dept. of Biochemistry, University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Cees
authors.initials: C
authors.lastname: Vermeer
authors.affiliation: Dept. of Nuclear Medicine, University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Marinus J P G
authors.initials: MJPG
authors.lastname: van Kroonenburgh
authors.affiliation: Dept. of Clinical Epidemiology and Medical Technology Assessment (KEMTA), University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Fred H M
authors.initials: FHM
authors.lastname: Nieman
authors.affiliation: Dept. of Clinical Chemistry, University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Paul P C A
authors.initials: PPCA
authors.lastname: Menheere
authors.affiliation: Dept. of Laboratory Genetic Metabolic Diseases, University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Leo J
authors.initials: LJ
authors.lastname: Spaapen
authors.affiliation: Dept. of Pediatrics, University Hospital Maastricht, Maastricht, The Netherlands; Dept. of Laboratory Genetic Metabolic Diseases, University Hospital Maastricht, Maastricht, The Netherlands. Electronic address: mrub@paed.azm.nl.
authors.forename: M Estela
authors.initials: ME
authors.lastname: Rubio-Gozalbo
mesh_terms: D015502:Absorptiometry, Photon; D000293:Adolescent; D001823:Body Composition / Q000187:drug effects; D015519:Bone Density / Q000187:drug effects; D001842:Bone and Bones / Q000187:drug effects* / Q000378:metabolism; D002118:Calcium / Q000008:administration & dosage / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D019587:Dietary Supplements; D004305:Dose-Response Relationship, Drug; D004311:Double-Blind Method; D005260:Female; D005693:Galactosemias / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D008297:Male; D015675:Osteocalcin / Q000737:chemistry / Q000378:metabolism; D016896:Treatment Outcome; D014807:Vitamin D / Q000008:administration & dosage / Q000627:therapeutic use*; D010837:Vitamin K 1 / Q000008:administration & dosage / Q000627:therapeutic use*; D014815:Vitamins / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D014815:Vitamins; D015675:Osteocalcin; D014807:Vitamin D; D010837:Vitamin K 1; D002118:Calcium