Abstract
Objectives: This study aims to assess the feasibility of using hemofiltration for ammonia clearance in low body weight infants with an inborn error of metabolism.Design: A study of two cases.Setting: Quaternary pediatric hospital (Saint Louis Children's Hospital) NICU and PICU.Patients: Infants <6 months of age with an ICD-9 diagnosis of 270.6 (hyperammonemia).Interventions: Continuous renal replacement therapy (CRRT).Measurements and Main Results: We measure serum ammonia levels over time and the rate of ammonia clearance over time. Continuous renal replacement therapy was more effective than scavenger therapy alone (Ammonul™) for rapid removal of ammonia in low weight infants (as low as 2.5 kg).Conclusions: Continuous renal replacement therapy is technically feasible in low weight infants with severe hyperammonemia secondary to an inborn error of metabolism.
Highlights
Pediatric inborn errors of metabolism that cause hyperammonemia pose specific technical challenges in critical care management, especially in the newborn period when presenting in extremis
Both urea cycle defects (UCDs) or organic acidemias commonly present with hyperammonemia
Patients were enrolled with the following inclusion criteria: hospitalized infants
Summary
Pediatric inborn errors of metabolism that cause hyperammonemia pose specific technical challenges in critical care management, especially in the newborn period when presenting in extremis. During this newborn period, both urea cycle defects (UCDs) or organic acidemias commonly present with hyperammonemia. To a lesser extent, some patients with maple syrup urine disease and fatty acid oxidation disorders can have mildly elevated ammonia. Infants with these aforementioned diseases commonly present with decreased levels of consciousness and poor feeding, while up to 50% can present with seizures [1].
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