Table 9.

Acute Inpatient Treatment in Individuals with VLCAD Deficiency 1

ManifestationTreatmentConsideration/Other
↑ catabolism (due to fever, perioperative/peri-interventional fasting periods, repeated vomiting/diarrhea)Administer high-energy fluids (at least 10% IV dextrose) w/electrolytes at rate of ≥1.5x maintenance (minimum of 8 mg/kg/min of glucose). 2
  • Avoid use of L-carnitine during acute illness.
  • Avoid use of IV lipids.
  • A source of essential fatty acids should be provided after 7 days.
Cardiomyopathy /
Cardiac failure		Standard treatmentFluid amounts may require adjustment based on cardiac status w/o compromising caloric provisions.
RhabdomyolysisAmple hydration & alkalization of urineTo protect kidney function & prevent acute kidney failure secondary to myoglobinuria
1.

Inpatient emergency treatment should (1) take place at the closest medical facility, (2) be started without delay, and (3) be supervised by physicians and specialist dieticians at the responsible metabolic center, who should be contacted without delay.

2.

Total fluid volumes may need to be adjusted if the affected individual has cardiomyopathy; however, caloric provision should not be compromised. If necessary, decreasing IV fluid rates and increasing dextrose concentration (with supplemental insulin, if necessary, to avoid acute hyperglycemia) is required.

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

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