芳香l -氨基酸脱羧酶缺乏症患儿的麻醉处理:1例报告。

IF 1.3 Q3 ANESTHESIOLOGY
Saudi Journal of Anaesthesia Pub Date : 2025-07-01 Epub Date: 2025-06-16 DOI:10.4103/sja.sja_627_24
Rayan Muawad, Abdullah AlDhuwaihy, Abdulrahman AlGhamdi, Ahmed Abdurazaq
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引用次数: 0

摘要

本报告讨论了一名患有芳香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疾病。患者接受了通气管插入和腺扁桃体切除术。与其他腺扁桃体切除术类似,该手术术后恶心和呕吐的风险较高,需要谨慎的管理策略。我们选择地塞米松作为主要止吐剂,限制阿片类药物使用单剂量芬太尼,同时也结合右美托咪定与酮咯酸和扑热息痛加强疼痛管理。本病例强调了AADC缺乏患者需要专门的麻醉方案,以提高安全性和结果,特别是解决恶心和呕吐的挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anesthetic management of a child with aromatic L-amino acid decarboxylase deficiency: A case report.

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.

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来源期刊
CiteScore
1.90
自引率
8.30%
发文量
141
审稿时长
36 weeks
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