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Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy.
Nature Reviews Nephrology ( IF 41.5 ) Pub Date : 2020-04-08 , DOI: 10.1038/s41581-020-0267-8
Rupesh Raina 1, 2 , Jirair K Bedoyan 3 , Uta Lichter-Konecki 4 , Philippe Jouvet 5 , Stefano Picca 6 , Nicholas Ah Mew 7 , Marcel C Machado 8 , Ronith Chakraborty 2 , Meghana Vemuganti 9 , Manpreet K Grewal 10 , Timothy Bunchman 11 , Sidharth Kumar Sethi 12 , Vinod Krishnappa 2, 13 , Mignon McCulloch 14 , Khalid Alhasan 15 , Arvind Bagga 16 , Rajit K Basu 17 , Franz Schaefer 18 , Guido Filler 19 , Bradley A Warady 20
Affiliation  

Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.



中文翻译:

接受连续肾脏替代治疗的小儿高氨血症治疗共识指南。

小儿高氨血症可导致严重后果,包括脑水肿,严重的神经功能损害甚至死亡。在婴儿和儿童中,高氨血症的常见原因包括尿素循环紊乱或有机酸血症。很少有研究评估体外疗法在新生儿和儿童高氨血症管理中的作用。此外,缺乏使用非肾脏替代疗法(NKRT)和肾脏替代疗法(KRTs,包括腹膜透析,连续性KRT,血液透析和混合疗法)来治疗新生儿和儿童高氨血症的共识性指南。关键是及时使用KRT和/或NKRT进行治疗,其选择取决于氨浓度和患者的症状。该专家共识声明为小儿人群需要KRT的高氨血症的治疗提出了建议。需要加强研究以加强这些建议。

更新日期:2020-04-08
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