一名患有大疱性表皮松解症和吉特曼综合征的青少年患者接受后路脊柱融合手术的麻醉挑战。

IF 0.4 Q4 PEDIATRICS Journal of pediatric genetics Pub Date : 2021-06-01 Epub Date: 2020-04-25 DOI:10.1055/s-0040-1710329

Edgar E Kiss, Neethu Chandran, Gijo Alex, Patrick Olomu

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摘要

脊柱侧凸的手术矫正是为了避免进展到心肺损害以及改善患者的整体生活质量。在这个病例报告中，我们提出了一个14岁的单纯大疱性表皮松解症和Gitelman综合征的女孩，她接受了脊柱侧凸的后路脊柱融合术。这一独特的合并症患者在接受复杂、高风险的外科手术时的围手术期计划和术中处理在以前的文献中没有记录。我们详细介绍了术前优化患者的步骤，以及独特的术中手术和麻醉注意事项，从而成功完成手术和恢复。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Anesthetic Challenges of an Adolescent Patient with Epidermolysis Bullosa and Gitelman's Syndrome Undergoing Posterior Spinal Fusion Surgery.

Surgical correction for scoliosis is undertaken to avoid progression to cardiopulmonary compromise as well as improve the patient's overall quality of life. In this case report, we presented a case of a 14-year-old girl with epidermolysis bullosa simplex and Gitelman's syndrome who underwent posterior spinal fusion for scoliosis. The perioperative planning and intraoperative management of a patient with this unique combination of comorbidities undergoing a complex, high-risk surgical procedure were not previously chronicled in the literature. We detailed the steps undertaken to optimize the patient prior to surgery and the unique intraoperative surgical and anesthetic considerations that led to a successful completion of the surgery and recovery.

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Journal of pediatric genetics PEDIATRICS-

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期刊介绍： The Journal of Pediatric Genetics is an English multidisciplinary peer-reviewed international journal publishing articles on all aspects of genetics in childhood and of the genetics of experimental models. These topics include clinical genetics, molecular genetics, biochemical genetics, medical genetics, dysmorphology, teratology, genetic counselling, genetic engineering, formal genetics, neuropsychiatric genetics, behavioral genetics, community genetics, cytogenetics, hereditary or syndromic cancer genetics, genetic mapping, reproductive genetics, fetal pathology and prenatal diagnosis, multiple congenital anomaly syndromes, and molecular embryology of birth defects. Journal of Pediatric Genetics provides an in-depth update on new subjects and current comprehensive coverage of the latest techniques used in the diagnosis of childhood genetics. Journal of Pediatric Genetics encourages submissions from all authors throughout the world. The following articles will be considered for publication: editorials, original and review articles, short report, rapid communications, case reports, letters to the editor, and book reviews. The aim of the journal is to share and disseminate knowledge between all disciplines in the field of pediatric genetics. This journal is a publication of the World Pediatric Society: http://www.worldpediatricsociety.org/ The Journal of Pediatric Genetics is available in print and online. Articles published ahead of print are available via the eFirst service on the Thieme E-Journals platform.