克里格勒-纳贾尔综合征的麻醉处理

克里格勒-纳贾尔综合征（crigler—Najjar syndrome）是由于二磷酸尿苷葡萄糖醛酸转移酶先天性缺乏，而导致血浆非结合胆红素增高的一种遗传性疾病。血浆内游离胆红素增高或血浆白蛋白减少均可以使病情恶化，甚至导致胆红素脑病和严重的脑损伤。这类患者麻醉处理的目标是防止发生血浆胆红素与血浆白蛋白摩尔比值失调，避免神经系统损伤。

Crigler-Najjar syndrome is a hereditary condition of unconjugated hyperbilirubinemia due to a deficiency of the enzyme, uridine diphosphate glucuronosyltransferase. Exacerbations of the disease can occur whenever there is either an increase in free serum bilirubin and/or a decrease in serum albumin. The exacerbations can lead to bilirubin encephalopathy and severe brain damage. The goal of anesthetic management in these patients is to prevent an imbalance in the serum bilirubin to serum albumin molar ratio, thereby avoiding neurologic sequelae