Table 4.

Acute Outpatient Management in Individuals with Arginase Deficiency

Manifestation/ConcernTreatmentConsideration/Other
Mildly ↑ catabolism 1
  • Carbohydrate supplementation orally or by feeding tube
  • ↓ natural protein intake 2
Trial of outpatient treatment at home for 12-48 hrs w/assessments for clinical changes 3
Fever Administration of antipyretics (acetaminophen, ibuprofen) if temperatures rises >38.5°C
Occasional vomiting Antiemetics
1.

Fever; vomiting, diarrhea, dehydration

2.

Some centers advocate reducing natural protein intake to zero or to 50% of the normal prescribed regimen for short periods (24-48 hours) in the outpatient setting during intercurrent illness.

3.

Alterations in mentation/alertness, fever, and enteral feeding tolerance with any new or evolving clinical features should be discussed with the designated center of expertise for inherited metabolic diseases.

From: Arginase Deficiency

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