Dataset Information

Bone Health in Classic Galactosemia: Systematic Review and Meta-Analysis.

ABSTRACT: INTRODUCTION:Previous studies have reported an association between classic galactosemia (CG) and decreased bone mass. The primary objective of this systematic review with meta-analysis was to determine the extent of bone mineral density (BMD) Z-score reduction. Low BMD was defined as a Z-score ?-2 standard deviations (SD). The secondary objective was to evaluate other indicators of bone status through a descriptive analysis. METHODS:Systematic search strategies were developed by an experienced clinical librarian. Selection of relevant manuscripts, risk of bias assessment, and data extraction were performed independently by two investigators. RESULTS:Four studies were included in the meta-analysis. BMD Z-scores in children and adults with CG measured at the lumbar spine (LBMD; 4 studies; n? =?112), total hip (HBMD; 2 studies; n = 58), and femoral neck (FBMD; 2 studies; n?=?73) were assessed. Mean BMD Z-scores in the CG population were LBMD -0.70 (95% CI: -0.88, -0.52); HBMD -0.89 (95% CI: -1.14, -0.64); and FBMD -0.63 (95% CI -1.29, 0.02). Results from studies included in the descriptive analysis (n = 7) show that vitamin D levels were frequently in the low reference range, whereas serum calcium levels were within reference range. CONCLUSION:The mean BMD Z-score in the CG population is -0.7, which is lower than in the general population, though still within two SD of the reference mean of zero. This indicates that bone health is mildly affected in CG and that more patients, compared to the general population, are at risk for a BMD Z-score ?-2 SD. In conclusion, clinicians should ensure appropriate preventive and therapeutic measures for CG patients.

SUBMITTER: van Erven B

PROVIDER: S-EPMC5585100 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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Bone Health in Classic Galactosemia: Systematic Review and Meta-Analysis.

van Erven Britt B Welling Lindsey L van Calcar Sandra C SC Doulgeraki Artemis A Eyskens François F Gribben Joanna J Treacy Eileen P EP Vos Rein R Waisbren Susan E SE Rubio-Gozalbo M Estela ME Bosch Annet M AM

JIMD reports 20161220

<h4>Introduction</h4>Previous studies have reported an association between classic galactosemia (CG) and decreased bone mass. The primary objective of this systematic review with meta-analysis was to determine the extent of bone mineral density (BMD) Z-score reduction. Low BMD was defined as a Z-score ≤-2 standard deviations (SD). The secondary objective was to evaluate other indicators of bone status through a descriptive analysis.<h4>Methods</h4>Systematic search strategies were developed by a ...[more]

PMID: 27995581

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Project description:Patients with Phenylketonuria (PKU) reportedly have decreased bone mineral density (BMD). The primary aim of this study was to perform a systematic review and meta-analysis to determine the extent and significance of low BMD in early treated patients with PKU. Secondary aims were to assess other bone status indicators including bone turnover markers (BTM) and to define areas for future research. Two research teams (Amsterdam, Netherlands and Atlanta, USA) performed literature searches for articles reporting data on BMD, osteopenia and osteoporosis, BTM or other bone indicators in patients with PKU. Included articles were compared between research teams and assessed for quality and risk of bias. A total of 13 unique articles were included; 11/13 articles reported BMD including a total of 360 patients. Ten out of 11 articles found BMD was significantly lower in patients with PKU. Meta-analyses for total BMD (TBMD; 3 studies; n = 133), lumbar spine BMD (LBMD; 7 studies; n = 247), and femoral neck BMD (FBMD; 2 studies; n = 78) Z-scores were performed. Overall effect sizes were: TBMD -0.45 (95% CI -0.61, -0.28); LBMD -0.70 (95% CI -0.82, -0.57); FBMD -0.96 (95% CI -1.42, -0.49). Definitions of osteopenia and osteoporosis were highly heterogeneous between studies and did not align with World Health Organization standards and the International Society for Clinical Densitometry positions on BMD measurement. Despite individual study findings of low BMD indicating higher risk of osteoporosis, pooled available data suggest reduction in BMD is not clinically important when using standard definitions of low BMD. Results from studies evaluating BTM are inconclusive. Phenylalanine concentration, vitamin D, PTH, and nutrient intake do not correlate with BMD or BTM. We recommend forthcoming studies use standard definitions of low BMD to determine clinical implications of BMD Z-scores below 0, explore cause of low BMD in the subset of patients with low BMD for chronological age (Z-score < -2) and assess fracture risk in patients with PKU.

Project description:BackgroundCurrent evidence demonstrate that sugar-sweetened beverages (SSBs) and bone health are related; however, there has been only a few reviews on the link between SSBs and bone health. A systematic review and meta-analysis was performed to investigate the association between SSBs consumption and bone health in chidren and adults.MethodsRelevant studies of SSBs and bone health published up to 15 March 2021 were searched using PubMed, the Web of Science, Cochrane Library, and a reference search. A random-effects meta-analysis was conducted to estimate the standardized mean difference (SMD). Subgroup analyses were performed to identify whether effects were modified by age, sex, measured skeletal sites, type of SSBs, and SSBs intake questionnaire.ResultsTwenty-six publications including 124,691 participants were selected on the review. The results from this meta-analysis showed a significant inverse association between SSBs intake and bone mineral density (BMD) in adults (ES: -0.66, 95% CI: - 1.01, - 0.31, n = 4312). Eighteen of the 20 studies included in the qualitative-only review in children and adults supported the findings from the meta-analysis. When subgroup analysis was performed according to skeletal site, a large effect was found on whole body BMD (ES: -0.97, 95% CI: - 1.54, - 0.40). There was a moderate effect on BMD in females (ES: -0.50, 95% CI: - 0.87, - 0.13). There was a moderate or large effect on BMD in individuals aged under 50 years (under 30 years: ES: -0.57, 95% CI: - 0.97, - 0.17; 30 to 50 years: ES: -1.33, 95% CI: - 1.72, - 0.93). High consumption of carbonated beverages had a moderate effect on BMD (ES: -0.73, 95% CI: - 1.12, - 0.35).ConclusionThe meta-analysis showed that SSBs consumption such as carbonated beverages were inversely related to BMD in adults. Qualitative review supported the results of meta-analysis.Trial registrationThis review was registered in the PROSPERO database under identifier CRD42020164428 .

Dataset Information

Bone Health in Classic Galactosemia: Systematic Review and Meta-Analysis.

Publications

Bone Health in Classic Galactosemia: Systematic Review and Meta-Analysis.

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