Precision errors and least significant changes in paediatric forearm measurements of bone density, mass, dimensions, mechanostat parameters and soft tissue composition by Stratec XCT-2000L.

OBJECTIVES: The peripheral quantitative computed tomography (pQCT) is gaining popularity in the field of paediatric densitometry, however, very little is known about the precision errors of this method in diseased children. The aim of the study was to evaluate the precision errors of bone density, mass, dimensions, strength, mechanostat parameters and soft tissue at the forearm in diseased children. METHODS: Stratec XCT 2000L apparatus was used. The measurement sites were 4% and 66% of the forearm length. The study group consisted of 60 patients (31 girls) aged 5,7-18,0 yrs. RESULTS: We observed week relationships between precision errors and body size with r from -0,37 to 0,28. Relative precision errors (CV%RMS) were from 0,85% for radius 66% cortical bone density to 3,82% for fat cross-sectional area to muscle cross-sectional area ratio. Least significant change (LSC) was from 2,73% to 10,59%, respectively. CONCLUSION: Presented study reveal pQCT method at the forearm in diseased children as relatively precise technique. The results may help with planning and interpretation of pQCT studies in diseased children.

Peripheral quantitative computed tomography of the lower leg in children and adolescents: bone densities, cross-sectional sizes and muscle distribution reference data.

OBJECTIVES: Peripheral quantitative computed tomography is utilised in increasing numbers of paediatric studies, however, very little is known about the reference limits for pQCT tibia measurements. The purpose of this study was to establish country-specific reference data for bone densities, cross-sectional sizes, strength and regional muscle distribution measured by pQCT in children and adolescents. METHODS: Stratec XCT 2000L apparatus was used. The measurement sites were 4%, 14%, 38% and 66% of the tibia length. The study group consisted of 222 participants (103 girls) aged 4,3-19,4 yrs. ANCOVA was used to assess the main determinants of pQCT outcomes. The LMS method was used to fit the percentile curves for each outcomes. RESULTS: Weight and age were the main determinants for most of the pQCT outcomes. Smoothed percentile curves were developed by age and by height for both sexes. CONCLUSION: In this study we present reference data for bone densities, cross-sectional size and strength as well as for regional muscle distribution measured by pQCT at certain sites of the lower leg to allow simple calculation of reliable Z scores.

Relationship between Bone Mineral Density and Selected Parameters of Calcium-Phosphate Economy with Dietary Management and Metabolic Control in Polish Pediatric Patients with Classical Homocystinuria-A Preliminary Study.

BACKGROUND: Classical homocystinuria (HCU) is an inborn defect of methionine metabolism caused by a deficiency of the enzyme cystathionine ß-synthase (CBS). The main symptoms of classical homocystinuria are lens subluxation, bone lesions, vascular disease and developmental delay/intellectual disability. The treatment method for HCU is a methionine-poor diet supplemented with amino acid preparations. The aim of the study was to examine the relationship of dietary factors, metabolic compensation and selected skeletal parameters in patients with HCU. METHODS: Bone mineral density measurements (DXA) were performed in pediatric patients with HCU, and blood levels of selected amino acids, minerals and vitamins, as well as dietary nutritional value, were analyzed. RESULTS: A total of 11 patients with HCU whose median age was 9.3 years were enrolled in the study. The median DXA total body less head of HCU patients was -0.4 z-score, and the lumbar spine was -1.4 z-score. Despite supplementation, calcium intake was below the age norm. Average vitamin D3 intake was in line with recommendations, but 36% of patients had reduced blood levels. Bone mineral density depended on blood levels of 25-hydroxyvitamin D, homocysteine and methionine, as well as on BMI, age and intake of natural protein (R2 = 98.5%, p = 0.015; R2 = 86.7%, p = 0.0049) and protein from an amino acid preparation (r = 0.69, p = 0.026). CONCLUSION: The results of the study indicate the need for regular densitometry in patients with HCU and also the use of additional calcium and vitamin D3 supplementation. It is also necessary to perform a comprehensive analysis of the diet and metabolic controls.

Bone Density, Geometry, and Mass by Peripheral Quantitative Computed Tomography and Bone Turnover Markers in Children with Diabetes Mellitus Type 1.

Background: The type 1 diabetes mellitus (T1DM) is a chronic systemic autoimmune-mediated disease characterised by the insulin deficiency and hyperglycaemia. Its deleterious effect on bones concerns not only bone mass, density, and fracture risk but also may involve the linear growth of long bones. Studies on the lower leg in children with T1DM by pQCT have generated conflicting results, and most of the studies published so far focused only on a selected features of the bone. An additional information about growth, modelling, and remodelling processes can be gathered by the bone turnover marker measurement. The objective of the study was to evaluate bone mineral density, mass, and geometry using peripheral quantitative computed tomography as well as bone turnover markers in the patients with type 1 diabetes mellitus. Material and Methods. Bone mineral density, mass, and geometry on the lower leg using peripheral quantitative computed tomography and serum osteocalcin (OC) and carboxyterminal cross-linked telopeptide of type 1 collagen (CTx) were measured in 35 adolescents with T1DM (15 girls) aged 12.3-17.9 yrs. The results were compared to age- and sex-adjusted reference values for healthy controls. Results: Both sexes reveal lower than zero Z-scores for lower leg 66% total cortical bone cross-sectional area to muscle cross-sectional area ratio (-0.97 ± 1.02, p = 0.002517 and -0.98 ± 1.40, p = 0.007050, respectively) while tibia 4% trabecular bone density Z-score was lowered in boys (-0.67 ± 1.20, p = 0.02259). In boys in Tanner stage 5 bone mass and dimensions were diminished in comparison to Tanner stages 3 and 4, while in girls, such a phenomenon was not observed. Similarly, bone formation and resorption were decreased in boys but not in girls. Consistently, bone turnover markers correlated positively with bone size, dimensions, and strength in boys only. Conclusions: T1DM patients revealed a decreased ratio of cortical bone area/muscle area, reflecting disturbed adaptation of the cortical shaft to the muscle force. When analyzing bone mass and dimensions, boys in Tanner stage 5 diverged from "less-mature" individuals, which may suggest that bone development in these individuals was impaired, affecting all three: mass, size, and strength. Noted in boys, suppressed bone metabolism may result in impairment of bone strength because of inadequate repair of microdamage and accumulation of microfractures.

The evaluation of consistency between body composition assessments in pediatric population using pencil beam and fan beam dual-energy x-ray absorptiometers.

The replacement of the old dual-energy X-ray absorptiometry system with a novel one should be preceded by a cross-calibration procedure. Therefore, the study was aimed at investigating the consistency of bone and body composition measures performed in pediatric population using pencil beam (DPX-L; GE Healthcare, GE Healthcare, Madison, WI) and fan beam (Prodigy; GE Healthcare, GE Healthcare, Madison, WI) densitometers. The study group consisted of 212 healthy children aged 4-18yr. Total body (TB) and lumbar spine (S) (L2-L4) measurements were performed using DPX-L and Prodigy during the same visit. Bland-Altman analysis, linear regressions, and paired t-test were performed to evaluate the consistency of measurements and to establish a cross-calibration equation. The average Prodigy values for TB and lumbar spine bone mineral density (BMD) and content (BMC) were 2.7%, 2.4% and 1.6%, 1.6% higher than those of DPX-L, respectively (p<0.0001). Prodigy-assessed bone area (BA) was lower by 1.4% for TBBA (p<0.0001) and 1.1% for SBA (p<0.001). Lean body mass (LBM) from Prodigy was higher by 6.9% (p<0.0001), whereas fat mass (FM) was lower by 8.4% compared with those from DPX-L (p<0.0001). Bland-Altman analyses revealed the effect of magnitude that was nonlinear (2nd degree polynomial) for TBBMD (r=0.32, p=0.001), TBBMC (r=0.51, p<0.0001), TBBA (r=0.34, p<0.0001), and LBM (r=0.56, p<0.0001), but not for FM (r=0.14, not significant [n.s.]). In contrast, in lumbar spine, the magnitude dependence was linear and significant for SBMC (r=0.46, p<0.0001) and SBA (r=0.34, p<0.0001) but not for SBMD (r=0.12, n.s.). Both skeletal and body composition variables assessed by DPX-L and Prodigy devices were highly correlated, showing R(2) values ranging from 0.976 for FM to 0.994 for SBMC. The results of this study document a necessity for implementation of calculated cross-calibration equations to transform DPX-L-based local pediatric references into a novel Prodigy system.

High-performance liquid chromatographic assay for cinnarizine in human plasma.

The high performance liquid chromatography for the determination of cinnarizine in human plasma is described. The procedure involves liquid-liquid extraction followed by reversed phase high-performance chromatographic analysis with fluorometric detection. The method was validated for accuracy, precision, specificity, linearity, sensitivity, recovery, and stability. No endogenous compounds were found to interfere. The absolute extraction recovery of cinnarizine and clocinizine (internal standard) from plasma samples were 97% and 89%, respectively. The linearity was assessed in the range 1-100 ng/mL. The intra-day and inter-day relative standard deviations were less than 10%, and the accuracy of the assay expressed by bias was in the range 0.14-2.37%. The method was proved to be suitable for human pharmacokinetic studies following single oral dose.

Analysis of nabumetone in human plasma by HPLC. Application to single dose pharmacokinetic studies.

A simple and sensitive high performance liquid chromatography method for the determination of nabumetone in human plasma is described. The procedure involves liquid-liquid extraction with ethyl acetate and reversed-phase chromatography with fluorimetric detection (excitation 230 nm, emission 356 nm). The chromatographic conditions and the extraction procedure gave a clean chromatogram for the compound. The limit of quantitation was established as 0.313 ng/ml and the calibration curve was linear up to 20 ng/ml. The within-day and between-day relative standard deviations were less than 10% and the accuracy of the assay was in the range of 99-104%. The suitability of the method is shown for pharmacokinetic studies.