Table 7.

Prevention of Secondary Manifestations in Individuals with Arginase Deficiency

Manifestation/
Situation		PreventionConsiderations/Other
Hyperammonemic
episodes 		Ongoing education of affected persons & caregivers re natural history, maintenance & emergency treatment, prognosis, & risks of acute encephalopathic crisesWritten protocols for maintenance & emergency treatment should be provided to parents, primary care providers/pediatricians, & teachers & school staff. 1, 2
Treatment protocols & provision of emergency letters or cards to incl guidance for care in the event of illnessEmergency letters/cards should be provided summarizing key information & principles of emergency treatment for arginase deficiency & containing contact information for the primary treating metabolic center.
MedicAlert® bracelets/pendants, or car seat stickers
Adequate supplies of specialized dietary products (protein-free formulas; medication required for maintenance & emergency treatment) should always be maintained at home.For any planned travel or vacations, consider contacting a center of expertise near the destination prior to travel dates.
1.

Essential information including written treatment protocols should be provided before inpatient emergency treatment may be needed.

2.

Parents or local hospitals should immediately inform the designated metabolic center if: (1) temperature is >38.5°C; (2) vomiting/diarrhea or other symptoms of intercurrent illness develop; or (3) new neurologic symptoms occur.

From: Arginase Deficiency

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