ICD-10 Diagnosis Code E71.39

Other disorders of fatty-acid metabolism

Diagnosis Code E71.39

ICD-10: E71.39
Short Description: Other disorders of fatty-acid metabolism
Long Description: Other disorders of fatty-acid metabolism
This is the 2018 version of the ICD-10-CM diagnosis code E71.39

Valid for Submission
The code E71.39 is valid for submission for HIPAA-covered transactions.

Code Classification
  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Disord of branched-chain amino-acid metab & fatty-acid metab (E71)

Information for Medical Professionals

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The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

  • Acetyl-CoA: carboxylase deficiency
  • Carnitine acylcarnitine translocase deficiency
  • Deficiency of 2,4-dienoyl-coenzyme A reductase
  • Deficiency of 2-hydroxyglutarate dehydrogenase
  • Deficiency of 3-hydroxybutyryl-coenzyme A epimerase
  • Deficiency of 3-hydroxyisobutyryl coenzyme A hydrolase
  • Deficiency of acetate-coenzyme A ligase
  • Deficiency of acetoacetyl-coenzyme A reductase
  • Deficiency of acetyl-CoA hydrolase
  • Deficiency of acetyl-coenzyme A acylase
  • Deficiency of acetyl-coenzyme A deacylase
  • Deficiency of carnitine acetyltransferase
  • Deficiency of coenzyme-A transferase
  • Deficiency of glutarate-CoA ligase
  • Deficiency of long-chain fatty-acyl-coenzyme A hydrolase
  • Deficiency of long-chain-fatty-acid-CoA ligase
  • Deficiency of malonyl-coenzyme A decarboxylase
  • Deficiency of palmitoyl-coenzyme A hydrolase
  • Long-chain fatty acid transport deficiency
  • Malonic aciduria

Information for Patients

Lipid Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues. If you have a metabolic disorder, something goes wrong with this process.

Lipid metabolism disorders, such as Gaucher disease and Tay-Sachs disease, involve lipids. Lipids are fats or fat-like substances. They include oils, fatty acids, waxes, and cholesterol. If you have one of these disorders, you may not have enough enzymes to break down lipids. Or the enzymes may not work properly and your body can't convert the fats into energy. They cause a harmful amount of lipids to build up in your body. Over time, that can damage your cells and tissues, especially in the brain, peripheral nervous system, liver, spleen, and bone marrow. Many of these disorders can be very serious, or sometimes even fatal.

These disorders are inherited. Newborn babies get screened for some of them, using blood tests. If there is a family history of one of these disorders, parents can get genetic testing to see whether they carry the gene. Other genetic tests can tell whether the fetus has the disorder or carries the gene for the disorder.

Enzyme replacement therapies can help with a few of these disorders. For others, there is no treatment. Medicines, blood transfusions, and other procedures may help with complications.

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