错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Post-mortem diagnosis of CACT deficiency with a novel SLC25A20 variant

  • Hiromi Nyuzuki,
  • Aya Miura,
  • Takuma Yamamoto,
  • Akihide Koyama,
  • Kazuhisa Funayama,
  • Satoru Takami,
  • Saori Fujita,
  • Youko Kuriyama,
  • Hisakazu Takatsuka,
  • Hajime Nishio,
  • Takeshi Ikeuchi

摘要

Carnitine-acylcarnitine translocase deficiency is a severe neonatal metabolic disorder caused by SLC25A20 variants. We report a case of sudden neonatal death in which post-mortem CT revealed diffuse fatty liver, and subsequent genetic analysis identified compound heterozygous variants in SLC25A20, including a known variant, c.824G>A p.(Arg275Gln), and a novel nonsense variant, c.334C>T p.(Gln112Ter). These findings demonstrate the utility of post-mortem genetic analysis in determining the cause of sudden neonatal death.