Idolazzi Luca, Fassio Angelo, Tripi Gaia, Braga Vania, Viapiana Ombretta, Adami Giovanni, Rossini Maurizio, Gatti Davide
Rheumatology Unit, University of Verona, Verona, Italy.
Clin Rheumatol. 2017 Apr;36(4):925-928. doi: 10.1007/s10067-016-3497-1. Epub 2017 Jan 5.
Paget disease of bone is a chronic metabolic bone disorder characterized by increased bone resorption and new bone formation. The aim of this study is defining the role of inhibitors of canonical Wnt/b-catenin signaling pathway in patients with Paget disease of bone. Scarce and contrasting results have been reported in literature. We studied 40 patients (15 females and 25 males) with radiological and scintigraphic evidence of Paget disease of bone and 40 healthy subjects matched by age and sex. N-propeptide of type I collagen, C-terminal telopeptide of type I collagen, sclerostin, and Dickkopf-related protein 1 (DKK1) were evaluated by blood samples in our laboratory. As expected, mean serum levels of bone turnover markers (N-propeptide of type I collagen and C-terminal telopeptide of type I collagen) were significantly higher in the Paget disease of bone group compared with the control group. No difference was observed between groups in Dickkopf-1 and sclerostin. Dickkopf-1 and sclerostin were never correlated with each other or with bone turnover markers. Sclerostin was positively correlated with age. In conclusion, our results suggest that the regulators of the Wnt-β catenin pathway are not altered in patients with Paget disease of bone. The positive correlation we found between sclerostin and age in Paget disease of bone patients indicates that in comparative studies, sclerostin serum levels must be adjusted for age.
骨佩吉特病是一种慢性代谢性骨病，其特征为骨吸收增加和新骨形成。本研究的目的是确定经典Wnt/β-连环蛋白信号通路抑制剂在骨佩吉特病患者中的作用。文献报道的结果稀少且相互矛盾。我们研究了40例有骨佩吉特病影像学和骨闪烁显像证据的患者（15例女性和25例男性）以及40例年龄和性别匹配的健康受试者。在我们实验室通过血液样本评估了I型胶原N端前肽、I型胶原C端末肽、硬化蛋白和Dickkopf相关蛋白1（DKK1）。正如预期的那样，骨佩吉特病组的骨转换标志物（I型胶原N端前肽和I型胶原C端末肽）平均血清水平显著高于对照组。Dickkopf-1和硬化蛋白在两组之间未观察到差异。Dickkopf-1和硬化蛋白彼此之间或与骨转换标志物均无相关性。硬化蛋白与年龄呈正相关。总之，我们的结果表明，骨佩吉特病患者的Wnt-β连环蛋白途径调节因子未发生改变。我们在骨佩吉特病患者中发现的硬化蛋白与年龄之间的正相关表明，在比较研究中，必须对硬化蛋白血清水平进行年龄校正。
