Gharavifard Mohammad, Sabzevari Alireza, Eslami Reza
Department of Anesthesiology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Surgical Oncology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.
Anesth Pain Med. 2014 Aug 13;4(3):e21791. doi: 10.5812/aapm.21791. eCollection 2014 Aug.
Citrullinemia is a defect in the urea cycle that causes ammonia to accumulate in the blood. We describe the anesthetic management of a patient with citrullinemia, who experienced an unexpected 10 day hospital admission.
We anesthetized a 3.5 year-old boy with citrullinemia who was scheduled for a dentistry procedure. Perioperative precautions included minimizing fasting period, hypothermia prevention, relieving anxiety and pain, perioperative infusion of D10W and benzoate sodium, as well as a pediatric endocrinology consultation. The operation lasted 4 hours and its course was uneventful. He had a delayed recovery from anesthesia and was discharged from hospital after 10 days.
General anesthesia and surgery can be a risk factor for exacerbating the course of the disease in patients with citrullinemia. It appears that administering short acting sedatives and analgesics in these patients would be of more benefit. Further studies are required to identify a safe method for anesthesia in citrullinemia.
瓜氨酸血症是尿素循环中的一种缺陷，会导致氨在血液中蓄积。我们描述了一名患有瓜氨酸血症的患者的麻醉管理情况，该患者意外住院10天。
我们为一名患有瓜氨酸血症的3.5岁男孩实施麻醉，该男孩计划进行牙科手术。围手术期预防措施包括尽量缩短禁食时间、预防体温过低、缓解焦虑和疼痛、围手术期输注10%葡萄糖溶液和苯甲酸钠，以及请儿科内分泌科会诊。手术持续了4小时，过程顺利。他麻醉苏醒延迟，10天后出院。
全身麻醉和手术可能是加重瓜氨酸血症患者病情的危险因素。在这些患者中使用短效镇静剂和镇痛药似乎更有益。需要进一步研究以确定瓜氨酸血症患者安全的麻醉方法。
