Journal
JOURNAL OF CLINICAL DENSITOMETRY
Volume 17, Issue 1, Pages 200-206Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.jocd.2013.03.004
Keywords
Bone mineral content; bone mineral density; mucopolysaccharidoses; osteoporosis; skeletal dysplasia
Categories
Funding
- National Institute of Arthritis and Musculoskeletal and Skin Diseases [K23AR057789]
- National Institute of Neurological Disorders and Stroke [U54NS065768]
- National Center for Research Resources (NCRR) [1UL1RR033183]
- National Center for Advancing Translational Sciences of the National Institutes of Health (NIH) [8UL1TR000114, UL1RR024131]
- National Cancer Institute [RO1CA113930-01A1]
- NCCR [M01-RR00400]
- Children's Cancer Research Fund
Ask authors/readers for more resources
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 (Z(age)), height-for-age (HAZ) Z-score (Z(HAZ)) 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 Z(age) and 48% had low LS Z(age); 0% and 6% had low WB Z(HAZ) and low LS Z(HAZ), 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available