Shigematsu Yosuke, Hirano Satoko, Hata Ikue, Tanaka Yukie, Sudo Masakatsu, Sakura Nobuo, Tajima Tsuyoshi, Yamaguchi Seiji
Department of Health Science, School of Nursing, Fukui Medical University, Matsuoka-cho, 910-1193 Fukui, Japan.
J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):39-48. doi: 10.1016/s1570-0232(02)00077-6.
Electrospray tandem mass spectrometry was applied to detect a series of inherited metabolic disorders during a newborn-screening pilot study and a selective screening in Japan. In our mass screening of 102,200 newborns, five patients with propionic acidemia, two with methylmalonic acidemia, two with medium-chain acyl-CoA dehydrogenase deficiency, three with citrullinemia type II, and one with phenylketonuria were identified. In a selective screening of 164 patients with symptoms mainly related to hypoglycemia and/or hyperammonemia, 12 with fatty acid oxidation disorders and six with other disorders were found. The results indicated the importance of newborn screening using this technology in Japan.
在日本的一项新生儿筛查试点研究和选择性筛查中,应用电喷雾串联质谱法检测一系列遗传性代谢疾病。在对102200名新生儿的大规模筛查中,鉴定出5例丙酸血症患者、2例甲基丙二酸血症患者、2例中链酰基辅酶A脱氢酶缺乏症患者、3例Ⅱ型瓜氨酸血症患者和1例苯丙酮尿症患者。在对164例主要有低血糖和/或高氨血症相关症状患者的选择性筛查中,发现12例脂肪酸氧化障碍患者和6例其他疾病患者。结果表明,在日本使用该技术进行新生儿筛查具有重要意义。