What is MCAD deficiency and why is it dangerous?

MCAD (medium-chain acyl-CoA dehydrogenase) deficiency prevents medium-chain fatty acid oxidation during fasting, causing hypoketotic hypoglycemia, encephalopathy, and sudden death if untreated.

How long can MCAD patients safely fast?

Safe fasting limits vary by age: newborns 4 hours, infants 6-8 hours, toddlers 8-10 hours, school-age 10-12 hours; illness protocols require dextrose supplementation if fasting exceeds these limits.

What acylcarnitine is elevated in MCAD deficiency?

C8 (octanoylcarnitine) is the hallmark elevation; C6, C8, C10, and C10:1 species are elevated. C8 >0.35 μmol/L on newborn screening is the typical cutoff.

What dietary fat restrictions are needed in LCHAD/VLCAD?

Long-chain FAO disorders require low long-chain fat diet with MCT oil supplementation; MCT bypasses the defective long-chain oxidation pathway and provides safe ketone production.

What is rhabdomyolysis risk in FAO disorders?

Exercise-induced rhabdomyolysis occurs particularly in VLCAD and CPT2 deficiency during prolonged exercise; patients need carbohydrate loading before exercise and avoidance of prolonged fasting.

Fatty Acid Oxidation Defect Risk Calculator

Assesses risk of metabolic crisis in fatty acid oxidation disorders (MCAD, LCHAD, VLCAD) based on acylcarnitine profile, fasting duration, illness severity, and provides safe fasting duration guidelines.

Use the full interactive calculator below, or

Related Calculators

Carnitine Deficiency

Medical Calculators

Metabolic Syndrome Risk

Medical Calculators

PA Monitor

Medical Calculators

Fatty Acid Oxidation Defect Risk Calculator

Related Calculators

Frequently Asked Questions

What is MCAD deficiency and why is it dangerous?

How long can MCAD patients safely fast?

What acylcarnitine is elevated in MCAD deficiency?

What dietary fat restrictions are needed in LCHAD/VLCAD?

What is rhabdomyolysis risk in FAO disorders?