ICD-10 Diagnosis Code E71.121

Propionic acidemia

Diagnosis Code E71.121

ICD-10: E71.121
Short Description: Propionic acidemia
Long Description: Propionic acidemia
This is the 2017 version of the ICD-10-CM diagnosis code E71.121

Code Classification
  • Endocrine, nutritional and metabolic diseases
    • 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.

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

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
  • Alkalosis
  • Lactic acid test
  • Metabolic acidosis
  • Metabolic neuropathies
  • Pseudohypoparathyroidism

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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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