第09章氨基酸代谢资料.ppt

As part of a standard neonatal screen, an infant is diagnosed with a genetic defect in the enzyme phenylalanine hydroxylase. Defects in this enzyme can result in a condition known as phenylketonuria (PKU), which results from the toxic effects of phenylalanine derived phenylketones. Fortunately, this condition can be managed by regulating the amount of phenylalanine provided in the diet. Which of the following nonessential amino acids will need to be supplied in the diet of this infant? A. Alanine B. Aspartate C. Glycine D. Serine E. Tyrosine 氨基酸的重要含氮衍生物 目 录 嘌呤碱合成的元素来源 CO2 天冬氨酸 甲酰基 （一碳单位） 甘氨酸 甲酰基 （一碳单位） 谷氨酰胺 （酰胺基） 嘧啶合成的元素来源 + NO + O2 NADPH+H+ NADP+ 一氧化氮合酶 （NOS） 精氨酸 瓜氨酸 一氧化氮 目 录 氨基酸代谢库 食物蛋白质 消化吸收 组织 蛋白质 分解 体内合成氨基酸 (非必需氨基酸) α-酮酸 脱氨基作用 酮 体 氧化供能 糖 胺 类 脱羧基作用 氨 尿素 代谢转变 其它含氮化合物 (嘌呤、嘧啶等) 合成 目 录 总结：蛋白质 基本单位（AA） A 45-year-old male with history of hepatitis C and now cirrhosis of the liver is brought to the emergency center by family members for acute mental status changes. The family reports that the patient has been very disoriented and confused over the last few days and has been nauseated and vomiting blood. The family first noticed disturbances in his sleep pattern followed by alterations in his personality and mood. On examination, he is disoriented with evidence of icteric sclera. His abdomen is distended with a fluid wave appreciated. He has asterixis and hyperreflexia on neurologic exam. His urine drug screen and ethyl alcohol (EtOH) screen are both negative. A blood ammonia level was noted to be elevated, and all other tests have been normal. What is the most likely cause of the patient’s symptoms? What was the likely precipitating factor of the patient’s symptoms? 45岁男性患者，因急性精神状态改变就诊，家属陈述患者最近几天出现不知所措和糊涂disoriented and confused，恶心并呕血。家里人最初注意到患者睡眠方式改变，之后发现患者性格、情绪均有改变。患者有丙型肝炎和肝硬化病史10年。体格检查发现患者巩膜黄染，腹部膨隆，伴有液波震颤，神经系统检查发现扑翼样震颤，神经反射增强。血液检查发现患者血氨水平升高 患者出现上述症状最可能的原因是什么? 引起患者症状加重的因素可能是什么precipitating factor ?