Department of Nephrology, Akron Children's Hospital, Akron, OH, USA.
Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, OH, USA.
Nat Rev Nephrol. 2020 Aug;16(8):471-482. doi: 10.1038/s41581-020-0267-8. Epub 2020 Apr 8.
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.
高氨血症可导致严重后果，如脑水肿、严重神经功能障碍甚至死亡。在婴儿和儿童中，高氨血症的常见病因包括尿素循环障碍或有机酸血症。很少有研究评估体外治疗在新生儿和儿童高氨血症管理中的作用。此外，对于非肾脏替代治疗（NKRT）和肾脏替代治疗（KRT，包括腹膜透析、连续 KRT、血液透析和混合疗法）在新生儿和儿童高氨血症管理中的应用，缺乏共识指南。及时进行 KRT 和/或 NKRT 治疗至关重要，治疗方式取决于患者的血氨浓度和临床表现。本专家共识声明提出了儿科人群需要 KRT 治疗的高氨血症管理建议。需要进一步研究来加强这些建议。
