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芳香族L-氨基酸脱羧酶缺乏症患儿的麻醉管理:一例报告

Anesthetic management of a child with aromatic L-amino acid decarboxylase deficiency: A case report.

作者信息

Muawad Rayan, AlDhuwaihy Abdullah, AlGhamdi Abdulrahman, Abdurazaq Ahmed

机构信息

Pediatric Anesthesia Department, King Abdullah Specialist Children Hospital, Ministry of National Guard Health Affairs (MNGHA), Riyadh, Saudi Arabia.

College of Medicine, King Saud University, Riyadh, Saudi Arabia.

出版信息

Saudi J Anaesth. 2025 Jul-Sep;19(3):425-427. doi: 10.4103/sja.sja_627_24. Epub 2025 Jun 16.

Abstract

This report discusses the anesthetic management of a 7-year-old child with aromatic L-amino acid decarboxylase (AADC) deficiency, a rare neurometabolic disorder. The patient underwent ventilation tube insertion and adenotonsillectomy. Similar to other adenotonsillectomy procedures, this surgery carries a higher risk of postoperative nausea and vomiting, necessitating a careful management strategy. We opted for dexamethasone as the primary antiemetic agent and limited opioid use to a single dose of fentanyl, while also incorporating dexmedetomidine for enhanced pain management alongside ketorolac and paracetamol. This case highlights the need for specialized anesthesia protocols for AADC deficiency patients to enhance safety and outcomes, particularly addressing the challenges of nausea and vomiting.

摘要

本报告讨论了一名患有芳香族L-氨基酸脱羧酶(AADC)缺乏症的7岁儿童的麻醉管理,这是一种罕见的神经代谢紊乱疾病。该患者接受了通气管插入术和腺样体扁桃体切除术。与其他腺样体扁桃体切除手术类似,该手术术后恶心和呕吐的风险更高,因此需要谨慎的管理策略。我们选择地塞米松作为主要的止吐药,并将阿片类药物的使用限制为单剂量芬太尼,同时还加入右美托咪定以加强疼痛管理,同时使用酮咯酸和对乙酰氨基酚。该病例强调了为AADC缺乏症患者制定专门麻醉方案以提高安全性和治疗效果的必要性,特别是应对恶心和呕吐的挑战。

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本文引用的文献

7
Aromatic amino acid decarboxylase deficiency in twins.
J Inherit Metab Dis. 1990;13(3):301-4. doi: 10.1007/BF01799380.

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