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Accumulation of ammonia in the blood may result in an acute life-threatening event in children. Patients typically present with lethargy, poor feeding, and hypotonia; if not managed efficiently, patients can develop seizures, coma, and eventually die. Management of hyperammonemia is difficult in children given the non-specific symptoms, age specific etiologies, and lack of consensus in the treatment plan. We systematically reviewed published literature to provide expert consensus panel recommendations for medical management and renal replacement therapy (RRT) in pediatric patients with hyperammonemia. Methods: PubMed/Medline, Embase and Cochrane database search was performed to include studies about hyperammonemia and RRT in children <18 years old. Two independent reviewers reviewed each title, abstract, and relevant full text articles. An expert panel of international pediatric nephrologists discussed medical management and RRT for hyperammonemia in children at a consensus conference to provide recommendations. Results: The initial search returned a total of 477 citations and 25 studies met the inclusion criteria. A total of 132 patients were included in these 25 studies and were treated with different dialysis modalities. Twenty-three heperammonemia patients were treated with peritoneal dialysis with 65% success rate, five were treated with intermittent hemodialysis with 100% success rate, 92 were treated with continuous renal replacement therapy (CRRT) with 60% success rate and three were treated by extracorporeal membrane oxygenation (ECMO) combined with CRRT and had 100% success rate. Conclusions: Expert panel recommendations were provided with regards to non-RRT (medical management), hemodialysis, peritoneal dialysis, CRRT, high dose CRRT, and hybrid therapy. CRRT was the first line dialysis modality of choice recommended for hyperammonemia management in children. Indications for RRT were variable among studies reviewed; RRT was recommended at ammonia blood level > 400mmol/L or in hemodynamically unstable children irrespective of blood ammonia level. More studies are needed to further strengthen these expert recommendations.

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