Department of Dental Anesthesiology, Faculty of Dental Medicine and Graduate School of Dental Medicine, Hokkaido University, Hokkaido, Japan.
Anesth Prog. 2021 Oct 1;68(3):158-162. doi: 10.2344/anpr-68-02-04.
We report a case involving intravenous sedation for third molar extractions in a 32-year-old man with citrullinemia type I (CTLN1), a genetic disorder that affects the urea cycle. The patient was diagnosed with CTLN1 after he exhibited seizures soon after birth and was intellectually disabled because of persistent hyperammonemia, although his recent serum ammonia levels were fairly well controlled. We planned to minimize his preoperative fasting, continue his routine oral medications, and monitor his serum ammonia levels at least twice. Sedation with midazolam and a propofol infusion was planned to suppress his gag reflex and reduce protein hypercatabolism due to stress. Epinephrine-containing local anesthetics, which enhance protein catabolism, were avoided, replaced by plain lidocaine for blocks and prilocaine with felypressin for infiltration anesthesia. No significant elevation in ammonia levels was observed. In patients with CTLN1, sedation can be useful for preventing hyperammonemia. Patients who develop symptomatic hyperammonemia may require urgent/emergent treatment involving other medical specialists. Therefore, preoperative endocrinology consultation, perioperative monitoring of serum ammonia levels, and preemptively coordinating for appropriate care in the event hyperammonemia occurs should all be considered.
我们报告了一例涉及静脉镇静的病例，该患者为一名 32 岁男性，患有瓜氨酸血症 I 型（CTLN1），这是一种影响尿素循环的遗传性疾病。该患者出生后不久即出现癫痫发作，智力发育迟缓，被诊断为 CTLN1，因为持续性高氨血症，尽管他最近的血清氨水平控制得相当好。我们计划尽量减少他的术前禁食，继续他的常规口服药物，并至少监测两次他的血清氨水平。计划使用咪达唑仑和丙泊酚输注来抑制他的呕吐反射，并减少因应激引起的蛋白质过度分解代谢。避免使用含有肾上腺素的局部麻醉剂，因为它们会增强蛋白质分解代谢，而使用普通利多卡因进行阻滞，并用prilocaine 和 felypressin 进行浸润麻醉。未观察到氨水平显著升高。在 CTLN1 患者中，镇静可用于预防高氨血症。出现症状性高氨血症的患者可能需要紧急/紧急治疗，涉及其他医学专家。因此，应考虑术前内分泌学咨询、围手术期监测血清氨水平，并预先协调在发生高氨血症时进行适当的治疗。
