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鲁宾斯坦-泰比综合征患儿的麻醉管理

Anaesthetic Management of Children with Rubinstein-Taybi Syndrome.

作者信息

Karahan Mahmut Alp, Sert Hüseyin, Ayhan Zeliha, Ayhan Bülend

机构信息

Department of Anaesthesiology and Reanimation, Harran University School of Medicine, Şanlıurfa, Turkey.

出版信息

Turk J Anaesthesiol Reanim. 2016 Jun;44(3):152-4. doi: 10.5152/TJAR.2016.76992. Epub 2016 Jun 1.

Abstract

Rubinstein-Taybi syndrome (RTS) is a rare, autosomal dominant syndrome presenting with mental retardation and physical abnormalities, including broad thumbs, big and broad toes, short stature and craniofacial anomalies. Special attention was paid to the possibilities of difficult airway, aspiration pneumonia and cardiovascular dysfunction during anaesthesia. Micrognathia, retrognathia, broad nasal bridge, abnormally large or 'beak-shaped' nose, hypoplastic maxilla and small mouth-typical dysmorphic facial features are one of the biggest causes of the difficult airway in this syndrome. Approximately one-third of the affected individuals have a variety of congenital heart diseases. Recurrent respiratory infections are likely to be the result of microaspiration or gastro-oesophageal reflux in this syndrome. In this case report, we discussed the anaesthesia management of a child with RTS who underwent right endoscopic dacryocystorhinostomy.

摘要

鲁宾斯坦-泰比综合征(RTS)是一种罕见的常染色体显性综合征,表现为智力发育迟缓及身体异常,包括拇指宽大、脚趾大且宽、身材矮小以及颅面畸形。麻醉期间需特别关注困难气道、吸入性肺炎和心血管功能障碍的可能性。小颌畸形、下颌后缩、鼻梁宽、鼻子异常大或呈“喙状”、上颌发育不全和嘴巴小——典型的畸形面部特征是该综合征中困难气道的最大原因之一。约三分之一的受累个体患有各种先天性心脏病。在该综合征中,反复呼吸道感染可能是微量误吸或胃食管反流的结果。在本病例报告中,我们讨论了一名接受右侧内镜下泪囊鼻腔造口术的RTS患儿的麻醉管理。

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本文引用的文献

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Management of Anesthesia for Rubinstein-Taybi syndrome.鲁宾斯坦-泰比综合征的麻醉管理
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