ICD-10 Diagnosis Code E71.0

Maple-syrup-urine disease

Diagnosis Code E71.0

ICD-10: E71.0
Short Description: Maple-syrup-urine disease
Long Description: Maple-syrup-urine disease
This is the 2017 version of the ICD-10-CM diagnosis code E71.0

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.

  • Classical maple syrup urine disease
  • Dihydrolipoamide dehydrogenase deficiency
  • Intermediate maple syrup urine disease
  • Intermittent maple syrup urine disease
  • Lactic acidosis
  • Maple syrup urine
  • Maple syrup urine disease
  • Maple syrup urine disease, multiple dehydrogenase form
  • Mild maple syrup urine disease
  • Thiamin-responsive maple syrup urine disease

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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Maple syrup urine disease Maple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants' urine and is also characterized by poor feeding, vomiting, lack of energy (lethargy), and developmental delay. If untreated, maple syrup urine disease can lead to seizures, coma, and death.Maple syrup urine disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy or childhood and are typically milder, but they still involve developmental delay and other health problems if not treated.
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