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Are Children Suffering From Congenital Pseudarthrosis of the Tibia Associated With Decreased Bone Strength?
Ge Yang1, Siyu Xu1, Haibo Mei1
1Department of Pediatric Orthopedics, The Hunan Children's Hospital, Changsha, China.
Insights
Congenital pseudarthrosis of the tibia (CPT) is linked to reduced bone strength in children. Higher CPT severity correlates with lower bone strength and increased fracture risk, independent of neurofibromatosis type 1.
Area of Science:
- Pediatric Orthopedics
- Biomedical Engineering
- Skeletal Diseases
Background:
- Congenital pseudarthrosis of the tibia (CPT) is a rare neonatal condition.
- Previous research indicated CPT-derived exosomes inhibit bone formation.
- This study investigates bone strength in children with CPT using ultrasound bone densitometry.
Purpose of the Study:
- To compare bone strength in children with CPT versus those with non-metabolic diseases.
- To identify factors associated with bone strength variations in CPT patients.
- To assess the relationship between CPT severity and bone strength.
Main Methods:
- Recruited 37 children with CPT and 40 controls (non-bone metabolic disease).
- Utilized ultrasonic bone densitometry to measure calcaneal broadband ultrasonic attenuation (BUA), speed of sound (SOS), quantitative ultrasound index (QUI), bone strength index (STI), and bone mineral density estimation (BMDe).
- Employed multivariable regression to analyze associations with age, BMI, NF1, and CPT Crawford type.
Main Results:
- CPT patients showed significantly lower SOS, STI, QUI, and BMDe compared to controls.
- Multiple regression analysis revealed negative correlations between SOS, STI, QUI, and CPT Crawford classification.
- Decreased bone strength incidence was higher in the CPT group.
Conclusions:
- Children with CPT exhibit reduced bone strength compared to controls.
- Bone strength reduction is linked to CPT severity (Crawford classification), not NF1.
- Higher CPT Crawford classification grades indicate lower bone strength and increased fracture risk.
Background:
Congenital pseudarthrosis of the tibia (CPT) is a rare and difficult-to-treat congenital disease in neonates. Our previous study found that exosomes derived from serum of children with CPT inhibit bone formation. In this study, we used ultrasound bone densitometry to detect the bone strength differences between hospitalized children with CPT and with non-metabolic diseases to determine the bone strength of children with CPT.
Methods:
A total of 37 children with CPT with a mean age of 3.14 ± 1.81 years and 40 hospitalized children with a mean age of 3.32 ± 2.66 years with supracondylar fracture of the humerus and without a bone metabolic disease (control group) were recruited in our hospital. The ultrasonic bone densitometer was used to examine the bilateral calcaneus of the subjects. We collected the broadband ultrasonic attenuation (BUA), speed of sound (SOS), quantitative ultrasound index (QUI), bone strength index (STI) and bone mineral density estimation (BMDe) values. Multivariable regression was used to examine the associations between quantitative ultrasound measurement differences and age, body mass index (BMI), neurofibromatosis type 1 (NF1) and CPT Crawford type. Intra-class correlation coefficient (ICC) was calculated to estimate intra- and inter-rater agreements.
Results:
74 calcaneus scans were taken from CPT patients (23 boys and 14 girls) and 80 calcaneus scans were taken from the control (24 boys and 16 girls). The CPT patients exhibited significantly lower SOS (1,368.75 ± 136.78 m/s), STI (7.2319 ± 38.6525), QUI (8.2532 ± 56.1720), and BMDe (-0.0241 ± 0.3552 g/cm3) than the control (SOS: 1,416.02 ± 66.15 m/s, STI: 7.96 ± 16.884, QUI: 28.8299 ± 25.461, BMDe: 0.0180 ± 0.1610 g/cm3). Multiple regression revealed that SOS, STI and QUI were statistically significant and negatively correlated with CPT Crawford classification.
Conclusions:
We found the incidence of decreased bone strength in CPT group was higher than that in the non-bone metabolic disease group. This phenomenon was not related to NF1 but related to CPT Crawford classification, which suggested that the higher the grade of the CPT Crawford classification, the lower the bone strength and the higher the risk of fracture.
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