新生儿和婴儿的二次卡尼丁缺乏症需要为肠道阻塞进行手术 - - 一个被低估和管理不足的问题
Sheng-Yang Huang1,2,3, Chia-Man Chou1,2,3, Hou-Chuan Chen1
1Division of Pediatric Surgery, Department of Surgery, Taichung Veterans General Hospital, Taichung 407219, Taiwan.
Children (Basel, Switzerland)
|February 24, 2024
概括
卡尼丁缺乏与患有肠道阻塞的婴儿的长时间内有关. 通过MS/MS或尿液有机酸分析及卡尼丁替代疗法的早期诊断可以改善结果.
科学领域:
- 儿科手术 儿科手术
- 营养生物化学 营养生物化学
- 新生儿医学 新生儿医学
背景情况:
- 肠道阻塞是新生儿和婴儿的危急情况.
- 卡尼缺乏症是一种代谢障碍,具有潜在的全身影响.
- 在这个年龄段,肠道阻塞和肉素缺乏之间的关联需要进一步调查.
研究的目的:
- 为了调查患有肠道阻塞的婴儿中肉素缺乏症的流行情况.
- 确定卡尼丁缺乏对肠道阻塞的呈现和恢复的临床影响.
- 评估卡尼丁替代疗法在受影响的婴儿中的疗效.
主要方法:
- 对330名因肠道阻塞而接受手术的婴儿 (<6个月) 的医疗记录的回顾性审查.
- 对临床数据,并发症和病因学的分析.
- 在47名患者中,生物化学分析 (MS/MS或尿液有机酸) 检测卡尼丁缺乏.
主要成果:
- 在47名分析患者中,16名患者 (34.0%在可疑组,4.8%整体) 发现了卡尼丁缺乏症.
- 与没有 (平均10.8天) 的患者相比,卡尼丁缺乏症患者表现出显著延长的内脏 (平均41.7天).
- 在12名患者的卡尼丁替代疗法显示出潜在的益处,其中有一例轻度药物过敏.
结论:
- 卡尼丁缺乏是新生儿和婴儿肠道阻塞的一个重要,未被认可的因素.
- 建议使用MS/MS或尿液有机酸分析查卡尼丁缺乏症,对于患有长期内的婴儿.
- 卡尼丁替代疗法是一种可行的,对受影响的婴儿有潜在益处的治疗方法.
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