ICD-10-CM Code E71.121

Propionic acidemia

Version 2021 Billable Code

Valid for Submission

E71.121 is a billable code used to specify a medical diagnosis of propionic acidemia. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E71.121 might also be used to specify conditions or terms like deficiency of propionyl-coa carboxylase, propionic acidemia, propionic acidemia, type i, propionic acidemia, type ii, propionyl-coa carboxylase deficiency pcca complementation group, propionyl-coa carboxylase deficiency pccbc complementation group, etc

Short Description:Propionic acidemia
Long Description:Propionic acidemia

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E71.121 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Deficiency of propionyl-CoA carboxylase
  • Propionic acidemia
  • Propionic acidemia, type I
  • Propionic acidemia, type II
  • Propionyl-CoA carboxylase deficiency pccA complementation group
  • Propionyl-CoA carboxylase deficiency pccBC complementation group

Clinical Information

  • PROPIONIC ACIDEMIA-. autosomal recessive metabolic disorder caused by mutations in propionyl coa carboxylase genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia hyperglycemia lethargy vomiting hypotonia; and hepatomegaly. survivors of the neonatal onset propionic acidemia often show developmental retardation and intolerance to dietary proteins. late onset form of the disease shows mild mental and/or developmental retardation sometimes without metabolic acidemia.

Convert E71.121 to ICD-9

  • 270.3 - Bran-chain amin-acid dis (Approximate Flag)

Code Classification

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

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients

Metabolic Disorders

Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system 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, such as your liver, muscles, and body fat.

A metabolic disorder occurs when abnormal chemical reactions in your body disrupt this process. When this happens, you might have too much of some substances or too little of other ones that you need to stay healthy. There are different groups of disorders. Some affect the breakdown of amino acids, carbohydrates, or lipids. Another group, mitochondrial diseases, affects the parts of the cells that produce the energy.

You can develop a metabolic disorder when some organs, such as your liver or pancreas, become diseased or do not function normally. Diabetes is an example.

  • Acidosis (Medical Encyclopedia)
  • Alkalosis (Medical Encyclopedia)
  • Lactic acid test (Medical Encyclopedia)
  • Metabolic acidosis (Medical Encyclopedia)
  • Metabolic neuropathies (Medical Encyclopedia)
  • Pseudohypoparathyroidism (Medical Encyclopedia)

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Propionic acidemia Propionic acidemia is an inherited disorder in which the body is unable to process certain parts of proteins and lipids (fats) properly. It is classified as an organic acid disorder, which is a condition that leads to an abnormal buildup of particular acids known as organic acids. Abnormal levels of organic acids in the blood (organic acidemia), urine (organic aciduria), and tissues can be toxic and can cause serious health problems.In most cases, the features of propionic acidemia become apparent within a few days after birth. The initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These symptoms sometimes progress to more serious medical problems, including heart abnormalities, seizures, coma, and possibly death.Less commonly, the signs and symptoms of propionic acidemia appear during childhood and may come and go over time. Some affected children experience intellectual disability or delayed development. In children with this later-onset form of the condition, episodes of more serious health problems can be triggered by prolonged periods without food (fasting), fever, or infections.
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