Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy

Abstract

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.

Extracorporeal Ammonia Clearance for Hyperammonemia in Critically Ill Patients: A Scoping Review

“…The protocol was registered in Open Science Framework (ID: osf.io/ dasxv). DOI: 10.1159/000512100 of its potential to induce irreversible neurological damage [3,4]. Immediate management aims to achieve a rapid reduction in blood ammonia levels to prevent cerebral edema and brain herniation [4].…”

Kidney Replacement Therapy in COVID-19 Induced Kidney Failure and Septic Shock: A Pediatric Continuous Renal Replacement Therapy [PCRRT] Position on Emergency Preparedness With Resource Allocation

“…It has also been demonstrated that convective modalities (such as CVVH and CVVHDF) are superior to diffusive modalities (CVVHD) due to the increased ultrafiltration rate and the higher sieving coefficient of the molecule in the convective mode, which further enhances the effect of cytokine removal (43). Thus, the Pediatric Continuous Renal Replacement Therapy (PCRRT) registry workgroup suggests high flow CVVHDF at 50 ml/kg/h for 12 h followed by step down CVVHDF at a dose of 25-30 ml/kg/h ( Table 4) (44). The provider may be able to incorporate the CytoSorb adsorber or oXiris filter into the CKRT circuit for higher clearance.…”

“…Thus, the Pediatric Continuous Renal Replacement Therapy (PCRRT) registry workgroup suggests high flow CVVHDF at 50 ml/kg/h for 12 h followed by step down CVVHDF at a dose of 25–30 ml/kg/h ( Table 4 ) ( 44 ). The provider may be able to incorporate the CytoSorb adsorber or oXiris filter into the CKRT circuit for higher clearance.…”

When do children need kidney replacement therapy?

“…Recent international guidelines recommend kidney replacement therapy in the most severely affected patients. 1 (see Box 2 ). Hyperammonaemia and certain toxins can also be managed with high clearance dialysis to ameliorate initial toxicity before maintenance therapies are established.…”

“…A diagnosis of OTC deficiency was subsequently made, and he went on to receive a successful liver transplant. Possible indications for KRT in treatment of hyperammonaemia 1 : Rapidly deteriorating neurological status, coma, or cerebral oedema Persistently high blood ammonia levels >400 μmol/L (681 μg/dL) refractory to non-kidney replacement therapy (NKRT) medical measures Rapid rise in ammonia levels >300 μmol/L (511 μg/dL) within a few hours that cannot be controlled via NKRT medical measures Alt-text: Box 2 …”