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High-dose continuous renal replacement therapy for neonatal hyperammonemia.

Joann M Spinale¹, Benjamin L Laskin, Neal Sondheimer

¹Division of Nephrology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA. spinalej@email.chop.edu

Pediatric Nephrology (Berlin, Germany)

|March 9, 2013

Summary

High-dose continuous renal replacement therapy (CRRT) rapidly lowers ammonia levels in infants with hyperammonemia. This approach is crucial when medical therapy is insufficient for severe cases.

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Area of Science:

Neonatal Medicine
Pediatric Nephrology
Metabolic Disorders

Background:

Hyperammonemia in infants often presents with nonspecific symptoms, necessitating a high index of suspicion for diagnosis.
Renal replacement therapy (RRT) is indicated for ammonia concentrations exceeding 400 μmol/L, as medical management alone is insufficient for rapid clearance.
The optimal prescription for RRT in neonatal hyperammonemia is not well-established, with both hemodialysis and CRRT showing efficacy but differing risk profiles.

Observation:

Two neonates diagnosed with ornithine transcarbamylase deficiency and hyperammonemia were treated with high-dose CRRT.
The CRRT protocol utilized dialysis/replacement flow rates of 8,000 mL/h/1.73 m(2), which is fourfold higher than typical rates for acute kidney injury.

Findings:

High-dose CRRT rapidly decreased ammonia levels in both patients.
Ammonia concentrations reduced to below 400 μmol/L within 3 hours and below 100 μmol/L within 10 hours of initiating CRRT.

Implications:

A CRRT treatment algorithm is proposed to rapidly manage hyperammonemia in neonates.
Effective management requires multidisciplinary collaboration involving emergency, genetics, critical care, surgery, and nephrology teams.
This strategy offers a potential method for rapid ammonia reduction in critical neonatal cases.