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马凡综合征——正畸视角

Marfan syndrome-an orthodontic perspective.

作者信息

Utreja Achint, Evans Carla A

机构信息

Department of Orthodontics, University of Illinois, Chicago, Chicago, IL, USA.

出版信息

Angle Orthod. 2009 Mar;79(2):394-400. doi: 10.2319/112707-558.1.

Abstract

Marfan syndrome is a heritable disorder of connective tissue that can affect the heart, blood vessels, lungs, eyes, bones, and ligaments. It is characterized by tall stature, elongated extremities, scoliosis, and a protruded or caved-in breastbone. Patients typically have a long, narrow face. A high-arched palate produced by a narrow maxilla and skeletal Class II malocclusion due to mandibular retrognathia are other common features. For a patient with no family history of the disorder, at least three body systems must be affected before a diagnosis can be made. Individuals affected by the syndrome routinely seek orthodontic treatment to correct the orofacial manifestations. In this report, the authors present the records of three patients with Marfan syndrome who were treated at a dental school. Two patients had severe periodontal disease in the absence of significant contributing local factors. The presentation of systemic symptoms and typical physical characteristics varied. The syndrome thus went unnoticed in one patient for many years. We discuss here the observed intraoral findings and the progress of orthodontic treatment to provide a brief overview of the challenges involved in treating such patients.

摘要

马凡氏综合征是一种遗传性结缔组织疾病,可影响心脏、血管、肺、眼睛、骨骼和韧带。其特征包括身材高大、四肢细长、脊柱侧弯以及胸骨前凸或凹陷。患者通常脸长且窄。上颌骨狭窄导致的高腭弓以及下颌后缩引起的骨骼Ⅱ类错牙合也是其他常见特征。对于无该疾病家族史的患者,在做出诊断前至少要有三个身体系统受到影响。受该综合征影响的个体通常会寻求正畸治疗来纠正口面部表现。在本报告中,作者展示了在一所牙科学院接受治疗的三名马凡氏综合征患者的记录。两名患者在没有显著局部促成因素的情况下患有严重的牙周疾病。全身症状和典型身体特征的表现各不相同。因此,该综合征在一名患者身上多年未被发现。我们在此讨论观察到的口腔内表现以及正畸治疗的进展,以简要概述治疗此类患者所涉及的挑战。

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