Table 8.

Emergency Outpatient Treatment in Individuals with VLCAD Deficiency

ManifestationTreatmentConsiderations/Other
Mildly ↑ catabolism 1
  • Frequent high-carbohydrate feedings 2
  • Fasting duration time should be ↓ compared to when person is well.
  • Trial of outpatient treatment at home for up to 12 hrs
  • Reassessment frequently for clinical changes
FeverAdminister antipyretics (acetaminophen, ibuprofen) if temperature >38.5 °C.
Occasional vomitingAntiemetics 3
1.

Fever <38.5 °C (101 °F); enteral feeding is tolerated without recurrent vomiting or diarrhea; absence of neurologic symptoms (altered consciousness, irritability)

2.

Focusing on foods that will provide glucose polymers, or simple or complex carbohydrates [Van Calcar et al 2020]

3.

Some classes of antiemetics can be used safely on an occasional basis to temporarily improve enteral tolerance of food and beverages at home or during transfer to a hospital.

From: Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

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