Reichert Christoph, Gölz Lina, Götz Werner, Wolf Michael, Deschner James, Jäger Andreas
Department of Orthodontics, Dental Hospital, University of Bonn, Welschnonnenstr. 17, 53111, Bonn, Germany,
J Orofac Orthop. 2014 Jul;75(4):251-63. doi: 10.1007/s00056-014-0216-x. Epub 2014 Jul 9.
The Hutchinson-Gilford progeria syndrome (HGPS) is an exceptionally rare medical disorder caused by mutations in the lamin A/C gene. Affected patients display typical features of premature aging. Beside general medical disorders, these patients have several specific features related to the craniofacial phenotype and the oral cavity. In this article, the dental and craniofacial characteristics of a 9-year-old girl with HGPS are presented. It is the first report addressing orthodontic tooth movement and microbiological features in a HGPS patient. We describe and discuss pathologic findings and provide a detailed histology of the teeth which had to be extracted during initial treatment.
哈钦森-吉尔福德早衰综合征(HGPS)是一种极为罕见的医学病症,由核纤层蛋白A/C基因的突变引起。受影响的患者表现出早衰的典型特征。除了一般的医学病症外,这些患者还有一些与颅面表型和口腔相关的特定特征。本文介绍了一名患有HGPS的9岁女孩的牙齿和颅面特征。这是首篇关于HGPS患者正畸牙齿移动和微生物特征的报告。我们描述并讨论了病理发现,并提供了在初始治疗期间必须拔除的牙齿的详细组织学情况。