Yang Ge, Xu Siyu, Mei Haibo, Zhu Guanghui, Liu Yaoxi, Tan Qian, Yu Hui
Department of Pediatric Orthopedics, The Hunan Children's Hospital, Changsha, China.
Department of Orthopedics, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.
Front Pediatr. 2022 May 9;10:859580. doi: 10.3389/fped.2022.859580. eCollection 2022.
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.
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.
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/cm) 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/cm). Multiple regression revealed that SOS, STI and QUI were statistically significant and negatively correlated with CPT Crawford classification.
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.
先天性胫骨假关节（CPT）是一种罕见且难以治疗的新生儿先天性疾病。我们之前的研究发现，CPT患儿血清来源的外泌体抑制骨形成。在本研究中，我们使用超声骨密度测定法检测住院的CPT患儿与非代谢性疾病患儿之间的骨强度差异，以确定CPT患儿的骨强度。
我院招募了37例平均年龄为3.14±1.81岁的CPT患儿以及40例平均年龄为3.32±2.66岁、因肱骨髁上骨折住院且无骨代谢疾病的患儿（对照组）。使用超声骨密度仪检查受试者的双侧跟骨。我们收集了宽带超声衰减（BUA）、声速（SOS）、定量超声指数（QUI）、骨强度指数（STI）和骨密度估计（BMDe）值。采用多变量回归分析定量超声测量差异与年龄、体重指数（BMI）、1型神经纤维瘤病（NF1）和CPT克劳福德分型之间的关联。计算组内相关系数（ICC）以评估评分者内和评分者间的一致性。
对CPT患儿（23例男孩和14例女孩）进行了74次跟骨扫描，对对照组（24例男孩和16例女孩）进行了80次跟骨扫描。CPT患儿的SOS（1368.75±136.78 m/s）、STI（7.2319±38.6525）、QUI（8.2532±56.1720）和BMDe（-0.0241±0.3552 g/cm）显著低于对照组（SOS：1416.02±66.15 m/s，STI：7.96±16.884，QUI：28.8299±25.461，BMDe：0.0180±0.1610 g/cm）。多元回归分析显示，SOS、STI和QUI具有统计学意义，且与CPT克劳福德分型呈负相关。
我们发现CPT组骨强度降低的发生率高于非骨代谢疾病组。这种现象与NF1无关，而与CPT克劳福德分型有关，这表明CPT克劳福德分型等级越高，骨强度越低，骨折风险越高。
