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一种非典型的哈勒曼-施特雷夫综合征。重点关注牙齿护理和鉴别诊断。

An atypical Hallermann-Streiff syndrome. Focus on dental care and differential diagnosis.

作者信息

Limeres Jacobo, Abeleira Maite, Tomás Inmaculada, Feijoo Javier F, Vilaboa Carlos, Diz Pedro

机构信息

Department of Special Needs, School of Medicine and Dentistry, Santiago de Compostela University, Santiago de Compostela, Spain.

出版信息

Quintessence Int. 2004 Jan;35(1):49-55.

Abstract

The Hallermann-Streiff syndrome (HSS) is a rare congenital disorder characterized by dyscephaly, birdlike facies, hypoplastic mandible, congenital cataracts, microphthalmia, hypotrichosis, skin atrophy, proportionate short stature, and dental anomalies. A case of a 29-year-old man with atypical HSS with neither cataracts, hair and skin alterations, nor short stature is reported, with special consideration to oral findings and dental management. Dental extractions, scaling, restorations, and endodontics were performed under local anesthesia. Later, orthodontic rehabilitation with fixed brackets was carried out. Finally, a removable partial denture for the maxillary arch was designed using transparent acrylic, and this also served as a retention splint. Young patients with HSS and other similar syndromes must be involved in personalized oral health prevention programs as early as possible. Despite numerous systemic anomalies, some of these patients may undergo conventional dental procedures under local anesthesia in the dental office.

摘要

哈勒曼-施特雷夫综合征(HSS)是一种罕见的先天性疾病,其特征为头颅畸形、鸟样面容、下颌发育不全、先天性白内障、小眼症、毛发稀少、皮肤萎缩、身材比例矮小和牙齿异常。本文报告了一例29岁非典型HSS男性病例,该患者既无白内障、毛发及皮肤改变,也无身材矮小情况,并特别关注口腔检查结果及牙齿治疗。在局部麻醉下进行了拔牙、洗牙、修复和根管治疗。之后,使用固定托槽进行正畸修复。最后,用上颌透明丙烯酸树脂设计了一副可摘局部义齿,它也起到了固位夹板的作用。患有HSS及其他类似综合征的年轻患者必须尽早参与个性化口腔健康预防计划。尽管存在众多全身异常,但其中一些患者在牙科诊所局部麻醉下仍可接受常规牙科治疗。

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