Indications for inpatient emergency care:
(1) recurrent vomiting
(2) recurrent diarrhea
(3) reduced nutrient intake
(4) high fever
(5) neurologic deterioration
(6) failure of outpatient management
Protocol:
(1) Give intravenous glucose to provide sufficient energy supply, adjusted for age.
(2) Provide insulin if there is persistent hyperglycemia or glucosuria.
(3) Stop natural protein intake for 24 hours then reintroduce in stepwise fashion.
(4) Provide lysine-free amino acid maintenance if tolerated.
(5) Provide carnitine IV (100 mg per kg per day if < 4 years of age; 50-100 if 4-6 years; 30-50 if > 6 years).
(6) Antipyretics if body temperature > 38.5°C (acetaminophen, ibuprofen).
(7) Sodium bicarbonate can be given IV if the patient shows acidosis.
|
Age
|
Glucose in g per kg per day
|
|
< 1 year
|
12 to 15
|
|
1 to 3 years
|
10 to 12
|
|
3 to 6 years
|
8 to 10
|
|
> 6 years
|
age adjusted
|
