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Anaesthetic Management of Children with Rubinstein-Taybi Syndrome.鲁宾斯坦-泰比综合征患儿的麻醉管理
Turk J Anaesthesiol Reanim. 2016 Jun;44(3):152-4. doi: 10.5152/TJAR.2016.76992. Epub 2016 Jun 1.
2
[General anesthesia for an infant with Rubinstein-Taybi syndrome].
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[Anesthesia in patient with Rubinstein-Taybi syndrome: case report.].
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Pulmonary hypertension due to obstructive sleep apnea in a child with Rubinstein-Taybi syndrome.一名患有鲁宾斯坦-泰比综合征的儿童因阻塞性睡眠呼吸暂停导致肺动脉高压。
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Prolonged paralysis with atracurium use in a patient with Rubinstein-Taybi syndrome.在一名患有鲁宾斯坦-泰比综合征的患者中,使用阿曲库铵后出现了长时间麻痹。
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本文引用的文献

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Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome.患有鲁宾斯坦-泰比综合征的儿童出现三尖瓣闭锁和肺动脉闭锁。
Ann Pediatr Cardiol. 2015 May-Aug;8(2):157-60. doi: 10.4103/0974-2069.154151.
2
Perioperative management of a patient of Rubinstein-Taybi syndrome with ovarian cyst for laparotomy.患有卵巢囊肿的鲁宾斯坦-泰比综合征患者行剖腹手术的围手术期管理。
J Anaesthesiol Clin Pharmacol. 2014 Jul;30(3):422-4. doi: 10.4103/0970-9185.137285.
3
Clinical and molecular characterization of Rubinstein-Taybi syndrome patients carrying distinct novel mutations of the EP300 gene.携带EP300基因不同新突变的鲁宾斯坦-泰比综合征患者的临床和分子特征
Clin Genet. 2015 Feb;87(2):148-54. doi: 10.1111/cge.12348. Epub 2014 Feb 17.
4
Successful use of air-Q intubating laryngeal airway after failed rapid sequence intubation in a child with Rubinstein-Taybi syndrome.在一名患有鲁宾斯坦-泰比综合征的儿童快速顺序插管失败后成功使用Air-Q插管型喉罩气道。
Indian J Anaesth. 2013 Mar;57(2):203-4. doi: 10.4103/0019-5049.111870.
5
Management of Anesthesia for Rubinstein-Taybi syndrome.鲁宾斯坦-泰比综合征的麻醉管理
Korean J Anesthesiol. 2012 Dec;63(6):571-2. doi: 10.4097/kjae.2012.63.6.571. Epub 2012 Dec 14.
6
Anesthetic management of children with Rubinstein-Taybi syndrome--case reports.鲁宾斯坦-泰比综合征患儿的麻醉管理——病例报告
Middle East J Anaesthesiol. 2011 Jun;21(2):309-12.
7
[Anesthesia in patient with Rubinstein-Taybi syndrome: case report.].
Rev Bras Anestesiol. 2005 Oct;55(5):546-51. doi: 10.1590/s0034-70942005000500010.
8
Dental treatment of a child with Rubinstein-Taybi syndrome.
Pediatr Dent. 2005 Sep-Oct;27(5):385-8.
9
Anesthetic management of a child with Rubinstein-Taybi syndrome.一名患有鲁宾斯坦-泰比综合征儿童的麻醉管理
Paediatr Anaesth. 2004 Jul;14(7):610-1. doi: 10.1111/j.1460-9592.2004.01314.x.
10
Broad thumbs and toes and facial abnormalities. A possible mental retardation syndrome.拇指和脚趾宽阔以及面部异常。一种可能的智力发育迟缓综合征。
Am J Dis Child. 1963 Jun;105:588-608. doi: 10.1001/archpedi.1963.02080040590010.

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.

DOI:10.5152/TJAR.2016.76992
PMID:27366579
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4925006/
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。