Hospital Universitario Virgen del Rocío, Spain.
Hospital Universitario Virgen del Rocío, Unidad de Metabolopatías, Sevilla, Spain.
Bone. 2021 Jun;147:115929. doi: 10.1016/j.bone.2021.115929. Epub 2021 Mar 16.
Cystic fibrosis (CF) bone disease (CFBD) has attracted considerable recent interest from researchers, although several aspects of CFBD pathophysiology remain poorly understood. The objective of this research was to investigate CFBD in children with CF and its relation to clinical and bone metabolism markers.
In a prospective observational study of 68 patients with CF and 63 healthy controls, we studied bone turnover biomarkers and bone mineral density (BMD). The biomarkers included osteocalcin, total-alkaline phosphatase, bone-alkaline phosphatase, N-terminal propeptide of type-1-procollagen, osteoprotegerin (OPG), interleukine-6, tumor necrosis factor alpha (TNF-α), type-1-collagen cross-linked C-telopeptide (CTX), parathormone (PTH), 25-vitamin D, 1,25-vitamin D, calcium and phosphorus. BMD was examined in lumbar spine, comparing two healthy Spanish populations. Two regression analyses were applied to any significant associations to evaluate predictors of BMD and of CF, expressed as odds ratios (OR) with 95% confidence intervals.
After adjusting for age, sex, and height Z-score, gains in BMD LS in children and adolescents (6-16 years) with CF were not less than in healthy reference population. Patients with CF showed significant associations with different bone turnover biomarkers. Age, gender, body mass index, PTH, CTX and OPG were significant predictors of BMD (R = 0.866, p < 0,001). Moreover, we found that PTH (OR = 1.070; 95% CI 1.019-1.123), and TNFα (OR = 2.173; 95% CI 1.514-3.118) were significantly linked to CF, and calcium (OR = 0.115; 95% CI 0.025-0.524), 1,25-vitamin D (OR = 0.979; 95% CI 0.962 0.996) and OPG (OR = 0.189; 95% CI 0.073-0.489) were significant reduced.
A normal bone mineral density along with altered remodeling was found in CF patients with a normal nutritional status and without acute lung disease.
囊性纤维化（CF）骨骼疾病（CFBD）最近引起了研究人员的极大兴趣，尽管 CFBD 病理生理学的几个方面仍未得到很好的理解。本研究的目的是研究 CF 儿童的 CFBD 及其与临床和骨代谢标志物的关系。
在一项对 68 例 CF 患者和 63 例健康对照者的前瞻性观察性研究中，我们研究了骨转换生物标志物和骨矿物质密度（BMD）。生物标志物包括骨钙素、总碱性磷酸酶、骨碱性磷酸酶、I 型前胶原 N 端前肽、骨保护素（OPG）、白细胞介素-6、肿瘤坏死因子-α（TNF-α）、I 型胶原交联 C 端肽（CTX）、甲状旁腺素（PTH）、25-维生素 D、1,25-维生素 D、钙和磷。比较了两个健康的西班牙人群，对腰椎进行了 BMD 检查。应用了两种回归分析来评估任何显著的相关性，以评估 BMD 和 CF 的预测因素，用比值比（OR）及其 95%置信区间表示。
在调整年龄、性别和身高 Z 分数后，6-16 岁 CF 儿童和青少年的腰椎骨密度（LS）增加并不低于健康参考人群。CF 患者与不同的骨转换生物标志物有显著关联。年龄、性别、体重指数、PTH、CTX 和 OPG 是 BMD 的显著预测因子（R=0.866，p<0.001）。此外，我们发现 PTH（OR=1.070；95%CI 1.019-1.123）和 TNFα（OR=2.173；95%CI 1.514-3.118）与 CF 显著相关，钙（OR=0.115；95%CI 0.025-0.524）、1,25-维生素 D（OR=0.979；95%CI 0.962-0.996）和 OPG（OR=0.189；95%CI 0.073-0.489）显著降低。
在营养状况正常且无急性肺部疾病的 CF 患者中，发现骨矿物质密度正常，同时存在重塑改变。
