胺反应性急性婴儿脑病变
Birendra Gurung1, Tsultem Doma Bhutia2,3, Subhash Chettri1,4
1Department of Pediatrics, Sir Thutob Namgyal Memorial Hospital, Gangtok, Sikkim, 737101, India.
Indian journal of pediatrics
|October 25, 2023
概括
胺治疗显著降低了患有急性非传染性脑病变的婴儿的死亡率,特别是双边基底腺干扰的婴儿. 这一发现突出了胺的含量.
科学领域:
- 儿科神经学 儿科神经学
- 营养神经科学 营养神经科学
背景情况:
- 急性非传染性脑病变是婴儿的关键病症,经常表现为多系统参与.
- 眼科和呼吸系统异常是常见的,以及神经学发现,如基底关节病变.
- 胺缺乏在这种情况中的作用需要进一步阐明.
研究的目的:
- 描述患有急性非传染性脑病变的婴儿的临床和研究发现.
- 为了确定胺治疗对该患者群的死亡率的影响.
主要方法:
- 对2019年10月至2021年3月期间入院的37名婴儿进行了回顾性研究.
- 分析临床数据,神经成像 (注意双边基底干涉) 和生化标志物.
- 在接受胺和不接受胺的患者之间比较死亡率.
主要成果:
- 患者的中位年龄为4个月,男性占主导 (62.2%).
- 75%的人在神经成像上显示了双边基底腺的参与;生物化学胺缺乏症在一个婴儿中得到证实.
- 接受胺治疗的11名患者中没有一个死亡,而26名患者中有20名 (病例死亡率为76.9%) 没有接受胺治疗. 胺使用与降低死亡率显著相关 (aOR 0.046,p=0.039).
结论:
- 胺治疗与急性非传染性脑病变的婴儿死亡率显著降低有关.
- 这种关联在双边基底腺干扰的情况下尤为明显.
- 提前给予胺可能是改善这些患者治疗结果的关键干预措施.
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