丙酸血症是先天性有机酸代谢异常中较常见的疾患 ,多于新生儿或婴儿早期起病 ,死亡率、致残率极高 ,早期发现、合理治疗是挽救患儿、改善预后的关键[1 3] 。近年 ,通过合作研究 ,我科 6例丙酸血症患儿得以确诊 ,现就其临床、生化检查资料、诊断及治疗进行分析。对象和方法1 资 Propionic acidosis is a common disease of congenital organic acid metabolism, more than the early onset of newborns or infants, mortality, high morbidity, early detection and reasonable treatment is the key to save children and improve prognosis [1 3]. In recent years, through collaborative research, 6 cases of pediatric proprionicosis were diagnosed in our department, and their clinical, biochemical examination data, diagnosis and treatment were analyzed. Objects and methods 1 funding