Dataset Information

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

ABSTRACT: 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?

SUBMITTER: Lin HY

PROVIDER: S-EPMC3698009 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature

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

Lin Hsiang-Yu HY Shih Shou-Chuan SC Chuang Chih-Kuang CK Chen Ming-Ren MR Niu Dau-Ming DM Lin Shuan-Pei SP

Orphanet journal of rare diseases 20130511

<h4>Background</h4>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.<h4>Methods</h4>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 ...[more]

PMID: 23663302

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Project description:BackgroundBody composition assessment aids evaluation of energy stores and the impact of diseases and interventions on child growth. Current United States pediatric reference ranges from the National Health and Nutrition Examination Survey (NHANES) include 20% of children with obesity, body mass index of ≥95th percentile.ObjectivesThis study aimed to develop dual energy X-ray absorptiometry (DXA) based reference ranges in a diverse cohort with low-obesity prevalence from the Bone Mineral Density in Childhood Study (BMDCS).MethodsThis is a secondary analysis of a longitudinal, prospective, observational cohort. Healthy children (height and BMI within 3rd to 97th percentiles, ages 5-19 y at enrollment), from 5 United States centers were measured annually for ≤7 visits. Whole body scans were acquired using Hologic scanners. A subsample underwent repeat measurements to determine precision. We generated reference ranges for appendicular and total lean soft tissue mass index (LSTM Index), fat mass index (FMI), and other body composition measures. Resulting curves were compared to NHANES and across subgroups. Sex and age-specific equations were developed to adjust body composition Z-scores for height Z score.ResultsWe obtained 9846 scans of 2011 participants (51% female, 22% Black, 17% Hispanic, 48% White, 7% Asian/Pacific Islander, and 6% with obesity). Precision (percent coefficient of variation) ranged from 0.7% to 1.96%. Median and-2 standard deviation curves for BMDCS and NHANES were similar, but NHANES +2 standard deviation LSTM Index and FMI curves were distinctly greater than the respective BMDCS curves. Subgroup differences were more extreme for appendicular LSTM Index-Z (mean ± SD: Asian -0.52 ± 0.93 compared with Black 0.77 ± 0.87) than for FMI-Z (Hispanic 0.29 ± 0.98 compared with Black -0.14 ± 1.1) and were smaller for Z-scores adjusted for height Z-score.ConclusionsThese reference ranges add to sparse normative data regarding body composition in children and adolescents and are based on a cohort with an obesity prevalence similar to current BMI charts. Awareness of subgroup differences aids in interpreting results.

Project description:Introduction The WHO definition of osteoporosis, and published BMD (Bone Mineral Density) references ranges, do not consider differences in bone size. Because it is a two-dimensional technique, and cannot measure bone depth, aBMD (areal BMD) measured using DXA (Dual-energy X-Ray Absorptiometry) is affected by bone size variability. Mathematical models have been devised to correct aBMD for bone size, but these are confounded by variations in soft tissue surrounding bone. Confirmation of the actual quantitative effect on clinical results for patients requires precise changes in bone size and mineral density, but studies of humans and animals are limited by the inability to precisely control these in natural bones. Purpose The objectives of this experiment were to obtain precise, repeatable, quantitative data from sets of artificial vertebrae to confirm the dependence of aBMD on bone size in clinical practice, and to test the effect of applying corrections based on assumptions that the vertebrae were simple geometric shapes to produce corrected BMAD (Bone Mineral Apparent Density). Methods and materials Four sets of artificial bones, each set containing four cylinders of different diameters but identical in height, were constructed by casting a mixture of epoxy resin and calcium carbonate powder into a mould. The cylinders were considered to be artificial vertebrae L1 to L4 so that all four in a set may be tested in a single scan. The X-Ray attenuation of the material used was varied between the sets, to represent differences in BMD. Each set of vertebrae was inserted into a soft-tissue analogue and DXA scanned, in the anteroposterior position, with the GE Lunar Prodigy and the Hologic Discovery. Results The results verify the theoretical direct proportionality between aBMD and diameter, confirming that aBMD is significantly affected by bone size. Applying a BMAD correction, by assuming the vertebrae to be cylinders, reduced the effect of change in bone diameter by approximately two orders of magnitude to an insignificant level. Conclusion This experiment has confirmed that BMD measured using DXA, accepted in clinical practice as the “gold standard” means of diagnosing osteoporosis, could lead to misdiagnosis because it is significantly affected by differences in bone size. Graphical abstract Unlabelled Image Highlights • Dual-Energy X-Ray Absorptiometry is two-dimensional; results affected by bone size.• Those with small bones may be over-diagnosed; those with large bones under-diagnosed.• Experiment with artificial vertebrae confirms theory and previous clinical studies.

Project description:BackgroundFor bone health assessment, dual-energy X-ray absorptiometry (DEXA) is recommended to measure bone mineral content and areal bone mineral density (aBMD) in the lumbar spine. However, intermachine differences were not taken into account when developing these recommendations. According to the International Society of Clinical Densitometry (ISCD), phantom-based cross-calibration is adequate after replacing the DEXA system from a different manufacturer. For different DEXA equipment, individual calibration equations were found to be necessary to fit the observed values with the given densities.MethodsThe BMD European Spine Phantom (ESP) measurements (L1, L2, and L3) were assessed on 3 machines. We used the Welch test in the one-way analysis of variance (ANOVA) with a post-hoc Tamhane T2 test, linear regressions, and Bland-Altman analysis to assess the consistency of measurements and establish cross-calibration equations.ResultsThe coefficients of variation (CV)% of the phantom BMD values measured using the 3 systems were less than 3.0%. The 3 DEXA systems were highly correlated with BMD in the lumbar spine, with correlation values ranging from 0.933 to 0.984 (P<0.0001). The cross-calibration regression models of the ESP measurements yielded the highest prediction accuracies with the lowest prediction errors (the standard error of the estimate ranged from 0.004 to 0.008 g/cm2; P<0.0001). After the regression equations were applied, the differences in BMD values among the 3 systems were negligible. In addition, the Bland-Altman plot showed that almost all data points were within the 95% limits of agreement.ConclusionsA strong agreement for BMD measurement was established between the 3 DEXA systems. Cross-calibration equations for the lumbar spine BMD values need to be applied to transform the Hologic Discovery A or GE Lunar iDXA measurements into SONIALVISION SMIT measurements to comply with the ISCD standards for patient continuity of care in assessment during clinical diagnosis.

Dataset Information

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

Publications

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

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