Table 5. [Acute Inpatient Treatment in Individuals with ASL Deficiency]. - GeneReviews®

Manifestation/ Concern	Treatment	Consideration/Other
Acute hyperammonemic episodes	Discontinue oral protein intake.
Supplement oral intake w/IV lipids, glucose, & insulin if needed (w/close monitoring of blood glucose) to promote anabolism.
IV nitrogen-scavenging therapy. A loading dose of 600 mg/kg L-arginine-HCL & 250 mg/kg each of sodium benzoate & sodium phenylacetate in 25-35 mL/kg of 10% dextrose solution given intravenously over a 90-min period is recommended, followed by a sustained IV infusion of 600 mg/kg L-arginine-HCL & 250 mg/kg each of sodium benzoate & sodium phenylacetate over a 24-hr period.	When available, plasma concentrations of ammonia-scavenging drugs should be monitored to avoid toxicity. In the absence of drug levels, a serum anion gap of >15 mEq/L & an anion gap that has risen >6 mEq/L could indicate drug accumulation & ↑ risk for toxicity.
Failure to ↓ ammonia levels w/medical therapy	Prompt institution of hemodialysis	Continuous arteriovenous hemodialysis or continuous venovenous hemodialysis w/flow rates >40-60 mL/min is optimal. Some centers use extracorporeal membrane oxygenation w/hemodialysis. Although this combination of techniques provides very high flow rates (170-200 mL/min) & rapidly reduces ammonia levels, morbidity is greater due to need for surgical vascular access. Nitrogen-scavenging therapy needs to be continued during hemodialysis. It is the authors' policy to continue nitrogen-scavenging therapy for 12-24 hrs after patient is stabilized & able to accept enteral feeds & medications [Author, personal observation].

Manifestation/
Concern

Treatment

Consideration/Other

Acute
hyperammonemic
episodes

Discontinue oral protein intake.

Supplement oral intake w/IV lipids, glucose, & insulin if needed (w/close monitoring of blood glucose) to promote anabolism.

IV nitrogen-scavenging therapy. A loading dose of 600 mg/kg L-arginine-HCL & 250 mg/kg each of sodium benzoate & sodium phenylacetate in 25-35 mL/kg of 10% dextrose solution given intravenously over a 90-min period is recommended, followed by a sustained IV infusion of 600 mg/kg L-arginine-HCL & 250 mg/kg each of sodium benzoate & sodium phenylacetate over a 24-hr period.

When available, plasma concentrations of ammonia-scavenging drugs should be monitored to avoid toxicity. In the absence of drug levels, a serum anion gap of >15 mEq/L & an anion gap that has risen >6 mEq/L could indicate drug accumulation & ↑ risk for toxicity.

Failure to ↓ ammonia levels w/medical therapy

Prompt institution of hemodialysis

Continuous arteriovenous hemodialysis or continuous venovenous hemodialysis w/flow rates >40-60 mL/min is optimal.
Some centers use extracorporeal membrane oxygenation w/hemodialysis.
Although this combination of techniques provides very high flow rates (170-200 mL/min) & rapidly reduces ammonia levels, morbidity is greater due to need for surgical vascular access.
Nitrogen-scavenging therapy needs to be continued during hemodialysis.
It is the authors' policy to continue nitrogen-scavenging therapy for 12-24 hrs after patient is stabilized & able to accept enteral feeds & medications [Author, personal observation].

From: Argininosuccinate Lyase Deficiency

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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