ArticleOrphanet journal of rare diseases2013
Assessment of bone mineral density by dual energy x-ray absorptiometry in patients with mucopolysaccharidoses.
Article in Orphanet journal of rare diseases, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 37 citations in OpenAlex.
- Patient-driven surveys of mucopolysaccharidoses revealed patient-reported outcomes in the Japan MPS patient and family group.Orphanet journal of rare diseases · 2026Article
- Delayed diagnosis of mucopolysaccharidosis type I in a patient with spinopelvic instability, short stature, and skeletal dysplasia.JBMR plus · 2026Article
- Assessment of bone health and bone mineral density in patients with mucopolysaccharidosis receiving enzyme replacement therapy.BMC pediatrics · 2026Observational
- Femoral Structure and Biomechanical Characteristics in Sanfilippo Syndrome Type-B Mice.International journal of molecular sciences · 2023Article
- Mucopolysaccharidosis: What Pediatric Rheumatologists and Orthopedics Need to Know.Diagnostics (Basel, Switzerland) · 2022Review
- MPSI Manifestations and Treatment Outcome: Skeletal Focus.International journal of molecular sciences · 2022Review
- Evaluation of bone health in patients with mucopolysaccharidosis.Journal of bone and mineral metabolism · 2022Article
- Cervical spine involvement in pediatric mucopolysaccharidosis patients: Clinical features, early diagnosis, and surgical management.Frontiers in surgery · 2022Review
- Case Report: Diagnosis of Mucopolysaccharidosis Type IVA With Compound Heterozygous Galactosamine-6 Sulfatase Variants and Biopsy of Replaced Femoral Heads.Frontiers in pediatrics · 2022Article
- Non-cardiac Manifestations in Adult Patients With Mucopolysaccharidosis.Frontiers in cardiovascular medicine · 2022Review
- Progression of vertebral bone disease in mucopolysaccharidosis VII dogs from birth to skeletal maturity.Molecular genetics and metabolism · 2021Article
- Ultrastructural analysis of different skeletal cell types in mucopolysaccharidosis dogs at the onset of postnatal growth.Journal of anatomy · 2021Article
- Abnormal epiphyseal development in a feline model of Sandhoff disease.Journal of orthopaedic research : official publication of the Orthopaedic Research Society · 2020Article
- Failures of Endochondral Ossification in the Mucopolysaccharidoses.Current osteoporosis reports · 2020Review
- Review
- An At-Risk Population Screening Program for Mucopolysaccharidoses by Measuring Urinary Glycosaminoglycans in Taiwan.Diagnostics (Basel, Switzerland) · 2019Article
- Taiwan National Newborn Screening Program by Tandem Mass Spectrometry for Mucopolysaccharidoses Types I, II, and VI.The Journal of pediatrics · 2019Article
- The role of imaging in the skeletal involvement of mucopolysaccharidoses.Italian journal of pediatrics · 2018Review
- The relationships between urinary glycosaminoglycan levels and phenotypes of mucopolysaccharidoses.Molecular genetics & genomic medicine · 2018Article
- Liver-Directed Human Amniotic Epithelial Cell Transplantation Improves Systemic Disease Phenotype in Hurler Syndrome Mouse Model.Stem cells translational medicine · 2017Article
Corrections and comments
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Authors and funding
6 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPatients 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.
methodsDual 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).
resultsFor 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.
conclusionsThese findings and the follow-up data can be used to develop quality of care strategies for patients with MPS.
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