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Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement
Rupesh Raina1,2, Jirair K Bedoyan3, Uta Lichter-Konecki4
1Department of Nephrology, Akron Children's Hospital, Akron, OH, USA. rraina@akronchildrens.org.
Insights
Hyperammonaemia in children can cause severe harm. This expert consensus provides recommendations for managing hyperammonaemia in pediatric patients using extracorporeal therapies like kidney replacement therapy (KRT).
Area of Science:
- Pediatric Nephrology
- Critical Care Medicine
- Metabolic Disorders
Background:
- Hyperammonaemia in children poses significant risks, including cerebral edema, neurological impairment, and mortality.
- Common causes in infants and children include urea cycle disorders and organic acidaemias.
- Limited research exists on extracorporeal therapies for neonatal and pediatric hyperammonaemia.
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.
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