Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses

Orphanet Journal of Rare Diseases

Shuan-Pei Lin²  Dau-Ming Niu⁴  Ming-Ren Chen²  Chih-Kuang Chuang¹  Shou-Chuan Shih³  Hsiang-Yu Lin⁵ 

[1] Institute of Biotechnology, National Taipei University of Technology, Taipei, Taiwan;Department of Early Infant Care and Education, Mackay Medicine, Nursing and Management College, Taipei, Taiwan;Division of Gastroenterology, Department of Internal Medicine, Mackay Memorial Hospital, Taipei, Taiwan;Department of Pediatrics, Taipei Veterans General Hospital, Taipei, Taiwan;Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan

关键词: Mucopolysaccharidoses;    Enzyme replacement therapy;    Dual energy x-ray absorptiometry;    Bone mineral density;   

Others  :  863995
DOI  :  10.1186/1750-1172-8-71

 received in 2013-03-30, accepted in 2013-05-09,  发布年份 2013

【 摘 要 】

Background

Patients with mucopolysaccharidoses (MPS) are associated with poor bone growth and mineralization, however, information regarding the assessment of bone mineral density (BMD) in relation to age and treatment in this disorder is limited.

Methods

Dual energy x-ray absorptiometry (DXA) was performed in 30 patients with MPS (21 males and 9 females; 2 with MPS I, 12 with MPS II, 2 with MPS IIIB, 9 with MPS IVA, and 5 with MPS VI; median age, 10.8 years; age range, 5.0 years to 23.7 years; 26 patients were under 19 and 4 were above 19 years of age) to assess BMD of the lumbar spine (L1-L4), using the Hologic QDR 4500 system (Bedford, MA, USA).

Results

For 26 patients under 19 years of age, standard deviation scores (z scores) for height, weight, body mass index (BMI), and BMD were −4.53 ± 2.66, -1.15 ± 1.55, 0.74 ± 1.23, and −3.03 ± 1.62, respectively, and they were all negatively correlated with age (p < 0.05). However, after correction for height-for-age z score (HAZ), HAZ adjusted BMD z score was −0.7 ± 1.24. Eight patients (31%) had osteopenia (HAZ adjusted BMD z score < −1 and ≥ −2), and 4 patients (15%) had osteoporosis (HAZ adjusted BMD z score < −2). Of 8 patients with MPS I, II or VI who underwent follow-up DXA after receiving enzyme replacement therapy for 1.0 to 7.4 years, all showed increase in absolute BMD values.

Conclusions

These findings and the follow-up data can be used to develop quality of care strategies for patients with MPS.

【 授权许可】

   
2013 Lin et al.; licensee BioMed Central Ltd.

【 图 表 】

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