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Axenfeld-Rieger 综合征:儿科患者的骨科和正畸管理:病例报告。

Axenfeld-Rieger syndrome: orthopedic and orthodontic management in a pediatric patient: a case report.

机构信息

Department of Clinical and Experimental Medicine, Università degli Studi di Foggia, Via Luigi Rovelli, 50, 71100, Foggia, Italy.

Department of Basic Medical Sciences, Neurosciences and Sensory Organs, "Aldo Moro" University of Bari, Piazza Giulio Cesare 11, 70124, Bari, Italy.

出版信息

Head Face Med. 2022 Jul 8;18(1):25. doi: 10.1186/s13005-022-00329-y.

Abstract

Axenfeld-Rieger Syndrome (ARS) is a rare autosomal dominant genetic disease with considerable expressive variability, characterized by ocular and non-ocular manifestations, cardiovascular, mild craniofacial abnormalities and dental malformations. Current data report an incidence of Xenfeld-Rieger syndrome in the population of 1: 200,000.The case described is that of a 14-year-old female patient whose ARS is suspected and investigated following a dental specialist visit for orthodontic reasons, acquired the patient's family and clinical data following a medical approach multidisciplinary, we proceed to the orthodontic involved the use of the Rapid Palatal Expander (RPE) and a fixed orthodontic treatment.The aim of this study is to report the case of the orthopaedic and orthodontic treatment in a patient affected by ARS and with facial dysmorphism and teeth anomalies associated to ocular anomalies.

摘要

Axenfeld-Rieger 综合征(ARS)是一种罕见的常染色体显性遗传疾病,具有相当大的表达变异性,其特征是眼部和非眼部表现、心血管、轻度颅面异常和牙齿畸形。目前的数据报告称,Xenfeld-Rieger 综合征在人群中的发病率为 1:200000。所描述的病例是一名 14 岁女性患者,因正畸原因就诊牙科专家后怀疑患有 ARS,并通过多学科医学方法获得了患者的家族和临床数据,我们开始进行正畸治疗,使用快速腭扩张器(RPE)和固定正畸治疗。本研究的目的是报告一例患有 ARS 且面部畸形和牙齿异常伴发眼部异常的患者的矫形和正畸治疗情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b5d0/9264492/febb126117d1/13005_2022_329_Fig1_HTML.jpg

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