...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Journal of paediatrics and child health >Utility of peritoneal dialysis in neonates affected by inborn errors of metabolism
【24h】

Utility of peritoneal dialysis in neonates affected by inborn errors of metabolism

机译：受先天性代谢错误影响的新生儿腹膜透析的效用

获取原文
获取原文并翻译 | 示例
   

获取外文期刊封面封底 >>

       
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Aim Some inborn errors of metabolism induce metabolic encephalopathy through accumulation of neurotoxic metabolites. Rapid elimination of these metabolites by peritoneal or extracorporeal dialysis is crucial to prevent neuronal damage or death. In this retrospective study, we evaluated the outcomes of nine neonates with metabolic crisis treated with peritoneal dialysis. Method Six neonates with hyperammonemic coma (four with organic acidemias, two with urea cycle disorders) and three with leucine accumulation due to maple syrup urine disease (MSUD) were managed with peritoneal dialysis in conjunction with dietary and pharmacological therapy. Results Three patients with organic acidemia survived. One of the patients was normal; others had moderate and severe neurological impairments. One neonate with organic acidemia and both neonates with urea cycle disorders died. Two of the three patients with MSUD survived without neurological impairment; the other had severe neurological damage and died at 9 months of age due to sepsis. Conclusion Theoretically, extracorporeal dialysis should be the first dialysis treatment of choice; however, this report demonstrates that peritoneal dialysis has a chance to prevent neurological damage in some patients. Therefore, in developing countries without extracorporeal dialysis opportunities, it can be still a life-saving procedure, if it is applied with skilled staff and standard procedures.
机译：目的某些先天性代谢错误会通过神经毒性代谢物的积累而诱发代谢性脑病。通过腹膜或体外透析快速消除这些代谢物对于预防神经元损伤或死亡至关重要。在这项回顾性研究中，我们评估了9名经腹膜透析治疗的代谢危重新生儿的结局。方法腹膜透析结合饮食和药物治疗，对6例新生儿高氨血症昏迷（4例有机酸血症，2例尿素循环异常）和3例因枫糖浆尿病（MSUD）导致亮氨酸蓄积的新生儿进行了腹膜透析治疗。结果3例有机酸血症患者存活。其中一名患者正常;其他人有中度和重度神经功能障碍。一名患有有机酸血症的新生儿和两名患有尿素循环异常的新生儿死亡。 MSUD的三名患者中有两名幸存，无神经功能障碍。另一个患有严重的神经系统损害，并因败血症在9个月大时死亡。结论从理论上讲，体外透析应该是首选的透析治疗方法。但是，该报告表明腹膜透析有机会预防某些患者的神经系统损害。因此，在没有体外透析机会的发展中国家，如果由熟练的工作人员和标准程序来实施，它仍然可以挽救生命。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号