Schmidt Joachim, Kroeber Stefanie, Irouschek Andrea, Birkholz Torsten, Schroth Michael, Albrecht Sven
Department of Anesthesiology, University of Erlangen-Nuremberg, Germany.
Paediatr Anaesth. 2006 Mar;16(3):333-7. doi: 10.1111/j.1460-9592.2005.01695.x.
Ornithine transcarbamylase deficiency (OTCD) is the most common inborn error of the urea cycle. Several specific factors require care during anesthesia in patients with this condition to avoid metabolic decompensation with acute hyperammonemia and encephalopathy. We report monozygous twins with severe neonatal-onset OTCD undergoing general anesthesia twice each, with midazolam, s-ketamine, fentanyl and isoflurane in combination with surgical field infiltration with ropivacaine. Alternative pathway medication and high-caloric diet with 10% glucose solutions were continuously administered during the perioperative course. Both children were extubated within 10 min of the final suture, and their neurological state remained unchanged. Perioperatively, blood ammonia levels remained within the normal range.
鸟氨酸转氨甲酰酶缺乏症（OTCD）是尿素循环中最常见的先天性代谢缺陷。对于患有这种疾病的患者，麻醉期间需要注意几个特定因素，以避免因急性高氨血症和脑病导致代谢失代偿。我们报告了一对患有严重新生儿期OTCD的同卵双胞胎，每人接受了两次全身麻醉，使用了咪达唑仑、S-氯胺酮、芬太尼和异氟烷，并在手术区域浸润使用了罗哌卡因。在围手术期持续给予替代途径药物和含10%葡萄糖溶液的高热量饮食。两个孩子在最后一针缝合后10分钟内拔管，神经状态未发生变化。围手术期，血氨水平保持在正常范围内。
