Advertisement

Proposal for the prevention of osteoporosis in paediatric patients with classical galactosaemia

  • B. Panis
  • M. J. P. G. van Kroonenburgh
  • M. E. Rubio-Gozalbo
Short Report

Summary

Decreased bone mass in early childhood is an increasingly recognized problem in classical galactosaemia as in many other chronic diseases. Peak bone mass is reached in late adolescence; thus, increasing peak bone mass in childhood can prevent osteoporosis. Regular bone mass measurements and preventive treatment should begin in childhood. In the absence of evidence-based guidelines for identification and treatment of decreased bone mass in children, we provide a proposal based on our experience and the available literature. Dual-energy x-ray absorptiometry (DXA) should be used for bone mass assessment. Because cooperation is required, measurements can usually be performed from the age of 4 years. Interpretation of bone mass measurements is crucial for the diagnosis of osteopenia or osteoporosis. In children and adolescents, total body bone mineral content (BMC) as well as lean tissue mass (LTM) should be measured. Comparison of BMC corrected for LTM of the patient with the BMC corrected for LTM of healthy controls allows correction for the confounding effect of bone size. DXA should be repeated every two years in case of normal BMC, as this is the time window in which abnormalities become measurable. If BMC is between 0 and −ISD, lifestyle factors such as physical activity, intake of calcium and vitamins K and D and oestrogen supplementation (in girls) should be optimized. If BMC is below −ISD, we advise to start with supplementation of calcium, vitamin K1 and vitamin D3. DXA should be repeated yearly in case of BMC below 0 SD in order to identify deteriorations and improvements early.

Keywords

Osteoporosis Bone Mass Bone Mineral Content Peak Bone Mass Bone Size 
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.

Supplementary material

10545_2007_Article_676_ESM.pdf (246 kb)
(PDF 245 kb)

Copyright information

© SSIEM and Springer 2007

Authors and Affiliations

  • B. Panis
    • 1
  • M. J. P. G. van Kroonenburgh
    • 2
  • M. E. Rubio-Gozalbo
    • 1
  1. 1.Department of Paediatrics and Laboratory Genetic Metabolic DiseasesUniversity Hospital MaastrichtMaastrichtThe Netherlands
  2. 2.Department of Nuclear MedicineUniversity Hospital MaastrichtMaastrichtThe Netherlands

Personalised recommendations