尿素循环障碍的肝移植治疗

尿素循环障碍（UCDs）是一组遗传性代谢病，是由于尿素合成和相关代谢途径的遗传缺陷引起的疾病，会导致高氨血症，临床上会出现一系列的表现，严重者昏迷、抽搐甚至死亡，当血氨急剧升高时会造成不可逆的神经系统损伤，肝脏移植是尿素循环障碍的治愈手段，应该在这些患者被导致神经系统损伤前尽早实施移植手术，从而改善预后。

Advances in the application of modern pediatric respiratory intervention techniques in tracheal development malformation

Tracheal malformation is rare compared with other respiratory diseases，but it can affect the growth and development of children，and even endanger life，which is an important content of diagnosis and treatment in respiratory department of children. With the wide application of bronchoscopy and airway reconstruction in pediatrics，the diagnosis rate of airway malformation in children is gradually increasing. In recent years，with the development of minimally invasive medicine of respiratory endoscope，the new broncho scopic intervention techniques have been gradually used in clinic，so children can get timely and effective diagnosis and treatment，and great progress has been made in treatment. This article briefly reviews the existing techniques to help clinicians apply these minimally invasive techniques.