Table 6c.

Classic Isovaleric Acidemia: Acute Inpatient Treatment

ManifestationTreatmentConsideration/Other
Increased catabolism (due to fever, perioperative/peri-interventional fasting periods, repeated vomiting/diarrhea)
  • Transient stop of protein intake
  • Intravenous glucose to restore anabolism
  • Normal saline for rehydration
  • Do not stop protein for >24 hours to avoid protein catabolism.
  • Glucose should be administered according to age, aiming to cover the gluconeogenesis rate of the liver (e.g., 12-15 g/kg/day in newborns).
  • Fluid intake is adjusted to age-dependent demands & should consider additional losses (e.g., vomiting, diarrhea).
New or evolving neurologic manifestations Adjust treatment after careful exam of secondary causes & following neurologic consultation & cranial MRI.
Metabolic acidosis Consider use of buffers (in case of life-threatening acidosis).
Hyperammonemia Nitrogen scavengersSodium benzoate: starting dose 250 mg/kg/day intravenously;, consider intravenous bolus or dose escalation in case of life-threatening hyperammonemia (usually rare).

From: Classic Isovaleric Acidemia

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