• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

一名患有比尔斯综合征患者的分娩镇痛:管理挑战的病例报告

Labor Analgesia in a Patient With Beals Syndrome: A Case Report of Management Challenges.

作者信息

Laranjeira João, Katerenchuk Vasyl, Duarte Joana, Lérias-Cambeiro Muriel, Lança Filipa

机构信息

Anesthesiology, Unidade Local de Saúde de Santa Maria, Lisboa, PRT.

Anesthesiology, Unidade Local de Saúde da Arrábida, Setúbal, PRT.

出版信息

Cureus. 2025 Feb 19;17(2):e79302. doi: 10.7759/cureus.79302. eCollection 2025 Feb.

DOI:10.7759/cureus.79302
PMID:40125196
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11927799/
Abstract

Beals syndrome, also known as Beals-Hetch syndrome or congenital contractural arachnodactyly, is an autosomal dominantly inherited, rare connective tissue disorder characterized by flexion contractures, arachnodactyly, camptodactyly, severe kyphoscoliosis, and, less frequently, cardiovascular features. We describe the successful management of labor analgesia in a pregnant woman with Beals syndrome. During labor, a combined spinal/epidural technique was performed uneventfully, and intermittent top-ups were administered as needed at lower-than-usual volumes. Eutocic delivery occurred without complications, and both mother and baby were discharged three days later. Given the sparse literature about obstetric patients with Beals syndrome, we summarize the main anesthetic challenges and suggested approaches.

摘要

比尔斯综合征,也称为比尔斯 - 赫奇综合征或先天性挛缩性蜘蛛指症,是一种常染色体显性遗传的罕见结缔组织疾病,其特征为屈曲挛缩、蜘蛛指、手指屈曲畸形、严重脊柱后凸侧弯,以及较少见的心血管特征。我们描述了一名患有比尔斯综合征的孕妇分娩镇痛的成功管理。分娩期间,顺利实施了腰麻 - 硬膜外联合技术,并根据需要以低于常规的剂量进行间歇性追加给药。分娩顺利,无并发症发生,母婴于三天后出院。鉴于关于患有比尔斯综合征的产科患者的文献稀少,我们总结了主要的麻醉挑战及建议的处理方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65c9/11927799/ad80b2e9486e/cureus-0017-00000079302-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65c9/11927799/ad80b2e9486e/cureus-0017-00000079302-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65c9/11927799/ad80b2e9486e/cureus-0017-00000079302-i01.jpg

相似文献

1
Labor Analgesia in a Patient With Beals Syndrome: A Case Report of Management Challenges.一名患有比尔斯综合征患者的分娩镇痛:管理挑战的病例报告
Cureus. 2025 Feb 19;17(2):e79302. doi: 10.7759/cureus.79302. eCollection 2025 Feb.
2
Congenital contractural arachnodactyly (Beals syndrome).先天性挛缩性蜘蛛指(贝氏综合征)。
Orphanet J Rare Dis. 2006 Jun 1;1:20. doi: 10.1186/1750-1172-1-20.
3
Beals-Hecht syndrome (congenital contractural arachnodactyly) with additional craniospinal abnormality: a case report.伴有额外颅脊柱异常的比尔斯-赫希特综合征(先天性挛缩性蜘蛛指):一例报告。
J Pediatr Orthop B. 2015 May;24(3):226-9. doi: 10.1097/BPB.0000000000000121.
4
What's New in Neuraxial Labor Analgesia.椎管内分娩镇痛的新进展
Curr Anesthesiol Rep. 2021;11(3):340-347. doi: 10.1007/s40140-021-00453-6. Epub 2021 Aug 27.
5
[Beals-Hecht Syndrome: report of a neonatal case].[比尔斯-赫希特综合征:一例新生儿病例报告]
J Pediatr (Rio J). 1995 Nov-Dec;71(6):337-40. doi: 10.2223/jped.802.
6
Congenital contractural arachnodactyly (Beals-Hecht syndrome): a rare connective tissue disorder.先天性挛缩性蜘蛛指(Beals-Hecht 综合征):一种罕见的结缔组织疾病。
Wien Klin Wochenschr. 2013 May;125(9-10):288-90. doi: 10.1007/s00508-013-0358-7. Epub 2013 Apr 18.
7
[The Beals-Hecht syndrome (congenital contractural arachnodactyly) revealed in a neonate].[一名新生儿中发现的比尔斯-赫希特综合征(先天性挛缩性蜘蛛指症)]
Pediatrie. 1988;43(7):609-12.
8
Congenital contractural arachnodactyly (Beals syndrome).先天性挛缩性蜘蛛指(比尔斯综合征)。
J Med Genet. 1994 Aug;31(8):640-3. doi: 10.1136/jmg.31.8.640.
9
[Neuraxial analgesia in a parturient with the VACTERL association undergoing labor and vaginal delivery].[一名患有VACTERL综合征的产妇在分娩和阴道分娩时的椎管内镇痛]
Braz J Anesthesiol. 2018 Mar-Apr;68(2):205-208. doi: 10.1016/j.bjan.2016.05.002. Epub 2017 Jan 9.
10
Beals syndrome with middle and inner ear dysplasia and encephalocele: A case report and review of imaging findings.伴有中耳和内耳发育异常及脑膨出的比尔斯综合征:一例报告及影像学表现回顾
Int J Pediatr Otorhinolaryngol. 2019 Feb;117:26-29. doi: 10.1016/j.ijporl.2018.11.009. Epub 2018 Nov 10.

本文引用的文献

1
Neuraxial anaesthesia in the parturient with pre-existing structural spinal pathology.患有脊柱结构病变的产妇的椎管内麻醉。
BJA Educ. 2024 Oct;24(10):361-370. doi: 10.1016/j.bjae.2024.05.005. Epub 2024 Aug 1.
2
2022 American Society of Anesthesiologists Practice Guidelines for Management of the Difficult Airway.2022 年美国麻醉医师学会困难气道管理实践指南。
Anesthesiology. 2022 Jan 1;136(1):31-81. doi: 10.1097/ALN.0000000000004002.
3
A 36-Year-Old Female With Congenital Contractural Arachnodactyly and Pectus Excavatum Requiring Fourth-Time Redo Surgical Correction.
一名36岁患有先天性挛缩性蜘蛛指(趾)症和漏斗胸的女性,需进行第四次再次手术矫正。
Cureus. 2021 Jul 28;13(7):e16701. doi: 10.7759/cureus.16701. eCollection 2021 Jul.
4
Beals-Hecht syndrome (congenital contractural arachnodactyly) with additional craniospinal abnormality: a case report.伴有额外颅脊柱异常的比尔斯-赫希特综合征(先天性挛缩性蜘蛛指):一例报告。
J Pediatr Orthop B. 2015 May;24(3):226-9. doi: 10.1097/BPB.0000000000000121.
5
Successful difficult airway management in a child with Hecht-Beals syndrome.成功处理一名患有赫希特-比尔斯综合征儿童的困难气道
Indian J Anaesth. 2012 Nov;56(6):591-2. doi: 10.4103/0019-5049.104591.
6
Marfan's syndrome during pregnancy: anesthetic management of delivery in 16 consecutive patients.妊娠合并马凡综合征:16 例连续患者分娩的麻醉管理。
Anesth Analg. 2013 Feb;116(2):392-8. doi: 10.1213/ANE.0b013e3182768f78. Epub 2013 Jan 9.
7
Congenital contractural arachnodactyly (Beals syndrome).先天性挛缩性蜘蛛指(贝氏综合征)。
Orphanet J Rare Dis. 2006 Jun 1;1:20. doi: 10.1186/1750-1172-1-20.
8
Ten novel FBN2 mutations in congenital contractural arachnodactyly: delineation of the molecular pathogenesis and clinical phenotype.先天性挛缩性蜘蛛指畸形中的十个新型FBN2突变:分子发病机制和临床表型的描述
Hum Mutat. 2002 Jan;19(1):39-48. doi: 10.1002/humu.10017.
9
Anaesthetic management of two paediatric patients with Hecht-Beals syndrome.两名患有赫克特-比尔斯综合征的儿科患者的麻醉管理
Paediatr Anaesth. 1999;9(5):444-7. doi: 10.1046/j.1460-9592.1999.00381.x.
10
Congenital contractural arachnodactyly. A heritable disorder of connective tissue.先天性挛缩性蜘蛛指。一种结缔组织的遗传性疾病。
J Bone Joint Surg Am. 1971 Jul;53(5):987-93.