Bone health assessment in children with osteogenesis imperfecta from Bone health assessment in children with osteogenesis imperfecta from a pediatric endocrinology perspective a pediatric endocrinology perspective
Evaluation of skeletal health in children with OI should not rely solely on BMD measurements; vertebral fractures, biochemical markers, growth, and environmental factors should also be considered.
Evaluation of skeletal health in children with OI should not rely solely on BMD measurements; vertebral fractures, biochemical markers, growth, and environmental factors should also be considered.
Banu Turhan, N. Yaşar· Turkish Journal of Pediatric...· 0 citations
The findings suggest that the clinical role of TBS in the assessment of pediatric bone health remains to be established, and that larger prospective studies are needed to clarify the clinical value of TBS for fracture risk assessment in pediatric autoimmune gastrointestinal diseases.
Anna Łupińska, Sara Aszkiełowicz, Arkadiusz Zygmunt et al.· Nutrients· 0 citations
Metabolic factors, vitamin D levels, and functional limitations influence bone health and the development of secondary osteoporosis in children with CP and a significant negative correlation was found between GMFCS level and BMD.
Banu Turhan, N. Yaşar· Düzce Tıp Fakültesi Dergisi· 0 citations
The study indicates that lower BHI SDS values are associated with increased fracture risk in children with classical OI and indicates that BHI may represent a clinically useful complementary tool for fracture risk stratification in young children with OI, particularly where DXA is unavailable or technically limited, including infancy.
Ruggero Lanzafame, Alistair D. Calder, B. Crowe et al.· Journal of Clinical Endocrin...· 0 citations
Objectives
Type 1 diabetes (T1D) is a recognized risk factor for skeletal fragility in adults, but its impact on bone microstructure in children remains incompletely characterized. In particular, data on trabecular bone quality remain scarce. This study aimed to evaluate skeletal parameters in this group of children and examine their associations with clinical variables.
Methods
This retrospective cross-sectional study evaluated children with T1D at the Hong Kong Children's Hospital from June 2023 to December 2024. Skeletal parameters, including trabecular bone score (TBS), bone health index (BHI), bone mineral density (BMD) by dual-energy x-ray absorptiometry, and serum vitamin D and HbA1c levels, were evaluated alongside fracture history. A parent-reported questionnaire measured participation in physical activity. Correlations between bone parameters and clinical variables were analyzed.
Results
Sixty-eight children with T1D (male 42.6%, mean 12.7 ± 3.7 years, 44.1% prepubertal, mean HbA1c 7.3%) were included. A substantial proportion of children (19.6%) had low cortical bone density as reflected by BHI Z-score ≤ -2, whereas deficits in trabecular bone (TBS Z-score ≤ -2) were less common (4.8 %). Regression analysis confirmed that higher lean mass was associated with higher total body less head (TBLH), lumbar spine (LS) and TBS Z-scores (β=0.0001, p < 0.05), while disease duration had a negative association with these measurements (β=-0.1413; β =-0.1335, β=-0.2270, p < 0.05).
Conclusion
In this cohort of children with T1D, a pattern of skeletal involvement was observed, characterized by a high prevalence of low BHI Z-scores and a relatively low prevalence of low TBS Z-scores. This may suggest differential involvement of cortical bone with relative preservation of trabecular microarchitecture. Whether these structural differences translate into fracture risk remains unknown. Longitudinal studies are needed to elucidate the evolution of bone alterations over time and to identify determinants underlying skeletal vulnerability in childhood-onset diabetes. Higher lean mass was associated with higher bone mass and microarchitectural indices, whereas longer disease duration may have a negative association with these parameters. These findings highlight modifiable and disease-related factors that may influence bone health, warranting confirmation in larger controlled studies.
S. W. Poon, Jian-Fang Zhu, Gloria S. W. Pang et al.· Journal of Clinical Research...· 0 citations
Atypical femoral fractures (AFF) are rare fractures with characteristic radiographic features, most commonly associated with long-term bisphosphonate use. Their occurrence in patients with osteogenesis imperfecta (OI), a hereditary bone disease leading to bone fragility, is not well understood. In this study, 138 adults with genetically confirmed classical OI were screened for AFF. Five patients with AFF were identified and compared to an age- and treatment-matched adult OI cohort without AFF (n = 23). Demographical parameters, biochemical markers, bone mineral density (DXA), bone microarchitecture (HR-pQCT), and radiographs were analyzed. In addition, antiresorptive therapy and the duration of treatment were determined and compared. In the screened OI cohort, AFF prevalence was 3.6%. No significant differences were observed between groups regarding age, weight, height, BMI, fracture history, bone mineral density, or antiresorptive therapy exposure and duration. HR-pQCT showed no significant microarchitectural differences, although a trend toward higher cortical thickness was noted in AFF patients. AFF are a rare complication in adults with classical OI and appear to be multifactorial in origin. Our findings suggest that AFF are not exclusively related to antiresorptive therapy but may be influenced by disease-specific factors, particularly the underlying collagen defect, femoral deformities and altered biomechanics. Individualized management strategies are essential, and further studies are needed to clarify underlying mechanisms and best treatment options.
Mikolaj Bartosik, O. Windels, F. Barvencik et al.· Calcified Tissue Internation...· 0 citations
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