Indications for outpatient therapy:
(1) generally well
(2) simple triggering event (mild infection, febrile reaction to vaccination, temperature < 38.5°C)
(3) able to tolerate diet
(4) no alarming symptoms (reduced level of consciousness, diarrhea, vomiting, hypotonia, dystonia, irritability)
Time period: 12 hours, with evaluation every 2 hours
Protocol:
(1) Give carbohydrates (maltodextrin) to provide sufficient energy supply (orally, by NG tube, by gastrostomy tube).
(2) Antipyretics if body temperature > 38.5°C (acetaminophen, ibuprofen).
(3) Reduce natural protein intake by > 50% or stop for 24-48 hours.
(4) Administer lysine-free amino acid mixture (AAM) if tolerated.
|
Age
|
Maltodextrin Solution
|
Volume in mL per day
|
|
<= 6 months
|
10% (w/v)
|
>= 150 mg/kg
|
|
6 to 12 months
|
12%
|
120 mg/kg
|
|
1 to 2 years
|
15%
|
100 mg/kg
|
|
2 to 6 years
|
20%
|
1,200-1,500 mL
|
If symptoms progress or if there is insufficient fluid intake, then admit to hospital.
If the issues resolve and child is stable, then gradually increase natural protein intake over 72 hours.
