Low bone mineral content and challenges in interpretation of dual-energy X-ray absorptiometry in children with mucopolysaccharidosis types I, II, and VI

Lynda E. Polgreen, William Thomas, Ellen Fung, David Viskochil, David A. Stevenson, Julia Steinberger, Paul J Orchard, Chester B Whitley, Kristine E Ensrud

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23 Scopus citations

Abstract

Osteoporosis has been described in animal models of mucopolysaccharidosis (MPS). Whether clinically significant osteoporosis is common among children with MPS is unknown. Therefore, cross-sectional data from whole body (WB; excluding head) and lumbar spine (LS) bone mineral density (BMD) compared with sex-, chronologic age-, and ethnicity-matched healthy individuals (Zage), height-for-age (HAZ) Z-score (ZHAZ) and bone mineral content (BMC) measured by dual-energy X-ray absorptiometry (DXA) in 40 children with MPS were analyzed. A subset of these children (n=24) was matched 1:3 by age and sex to a group of healthy children (n=72) for comparison of BMC adjusted for Tanner stage, race, lean body mass, height, and bone area. Low BMD Z-score was defined as Z-score of -2 or less. In children with MPS, 15% had low WB Zage and 48% had low LS Zage; 0% and 6% had low WB ZHAZ and low LS ZHAZ, respectively. Adjusted WB BMC was lower in MPS participants (p=0.009). In conclusion, children with MPS had deficits in WB BMC after adjustments for stature and bone area. HAZ adjustment underestimated bone deficits (i.e., overestimated WB BMD Z-scores) in children with MPS likely owing to their abnormal bone shape. The influence of severe short stature and bone geometry on DXA measurements must be considered in children with MPS to avoid unnecessary exposure to antiresorptive treatments.

Original languageEnglish (US)
Pages (from-to)200-206
Number of pages7
JournalJournal of Clinical Densitometry
Volume17
Issue number1
DOIs
StatePublished - Jan 2014

Bibliographical note

Funding Information:
This project was supported by grant number K23AR057789 from the National Institute of Arthritis and Musculoskeletal and Skin Diseases , U54NS065768 from the National Institute of Neurological Disorders and Stroke , 1UL1RR033183 from the National Center for Research Resources (NCRR), and by 8UL1TR000114 and UL1RR024131 from the National Center for Advancing Translational Sciences of the National Institutes of Health (NIH) to the University of Minnesota and to the Children's Hospital and Research Center, Oakland, respectively, Clinical and Translational Science Institutes (CTSI). Comparison data on healthy children were provided by JS from a project supported by grant number RO1CA113930-01A1 from the National Cancer Institute , M01-RR00400 from the NCCR, and the Children's Cancer Research Fund (JS). Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the CTSI or the NIH.

Keywords

  • Bone mineral content
  • Bone mineral density
  • Mucopolysaccharidoses
  • Osteoporosis
  • Skeletal dysplasia

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