Mechanical loading of cranial joints minimizes the craniofacial phenotype in Crouzon syndrome.
Mechanical loading of cranial joints minimizes the craniofacial phenotype in Crouzon syndrome.
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DOI：
10.1038/s41598-022-13807-9
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发表时间：
2022-06-11
影响因子：
4.6
通讯作者：
Pauws, Erwin
中科院分区：
文献类型：
--
作者：
Moazen, Mehran;Hejazi, Mahbubeh;Savery, Dawn;Jones, Dominic;Marghoub, Arsalan;Alazmani, Ali;Pauws, Erwin
患有颅缝早闭综合征的儿童需要接受过多的手术干预，以解决颅缝过早融合引起的临床特征。虽然手术矫正是可靠的，但需要反复进行侵入性治疗，这给孩子和他们的家庭带来了沉重的负担。本研究探讨了一种非手术的替代方法，使用颅关节的机械负荷来预防或延迟与Crouzon综合征相关的颅面表型。我们在颅缝早闭发作前通过使用定制设计的装置对颅关节进行周期性机械负荷来治疗Crouzon综合征小鼠。施加于额骨的颅骨负荷部分恢复了正常的颅骨形态，显著减少了典型的短头外观。冠状缝和蝶骨间软骨结合的延迟闭合是其基础。这项研究为综合征性颅缝早闭提供了一种新的治疗选择，有可能成为替代、减少或改善所有颅缝早闭患者手术治疗的重要一步。
Children with syndromic forms of craniosynostosis undergo a plethora of surgical interventions to resolve the clinical features caused by the premature fusion of cranial sutures. While surgical correction is reliable, the need for repeated rounds of invasive treatment puts a heavy burden on the child and their family. This study explores a non-surgical alternative using mechanical loading of the cranial joints to prevent or delay craniofacial phenotypes associated with Crouzon syndrome. We treated Crouzon syndrome mice before the onset of craniosynostosis by cyclical mechanical loading of cranial joints using a custom designed set-up. Cranial loading applied to the frontal bone partially restores normal skull morphology, significantly reducing the typical brachycephalic appearance. This is underpinned by the delayed closure of the coronal suture and of the intersphenoidal synchondrosis. This study provides a novel treatment alternative for syndromic craniosynostosis which has the potential to be an important step towards replacing, reducing or refining the surgical treatment of all craniosynostosis patients.
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