E71.311
BillableMedium chain acyl CoA dehydrogenase deficiency
HCC Category Mapping
V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
A rare inherited metabolic disorder where the body lacks an enzyme needed to break down medium-chain fatty acids, potentially causing low blood sugar, liver problems, and muscle weakness.
Coding Tips
- •MCAD deficiency is one of the most common fatty acid oxidation disorders; ensure accurate documentation
- •Note any acute episodes or complications such as hypoglycemia or encephalopathy for complete coding
Clinical Significance
Medium chain acyl-CoA dehydrogenase deficiency (MCAD) is the most common inherited fatty acid oxidation disorder, detected through newborn screening in most states. Patients cannot oxidize medium-chain fatty acids during fasting, creating risk of hypoketotic hypoglycemia, hepatic dysfunction, and potentially fatal metabolic crises. With proper management including avoidance of prolonged fasting, prognosis is excellent.
Documentation Requirements
- ✓Document newborn screening results or confirmatory testing (acylcarnitine profile showing elevated C8), genetic mutation analysis (common A985G mutation), history of metabolic decompensation episodes, and current fasting avoidance protocol.
- ✓Record any emergency management plans.
Commonly Confused Codes
Code Hierarchy
└E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism└E71.3Disorders of fatty-acid metabolism└E71.31Disorders of fatty-acid oxidation└E71.311Medium chain acyl CoA dehydrogenase deficiency
└E71.311Medium chain acyl CoA dehydrogenase deficiency