ICD-10-CM Code E70.21

Tyrosinemia

Version 2021 Billable Code

Valid for Submission

E70.21 is a billable code used to specify a medical diagnosis of tyrosinemia. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E70.21 might also be used to specify conditions or terms like 4-hydroxyphenylpyruvate dioxygenase deficiency, classical phenylketonuria, clinical manifestation of enzyme deficiency, deficiency of fumarylacetoacetase, fumarylacetoacetase deficiency, chronic type, hereditary hypertyrosinemia, etc

ICD-10:E70.21
Short Description:Tyrosinemia
Long Description:Tyrosinemia

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code E70.21:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Hypertyrosinemia

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 E70.21 are found in the index:


Synonyms

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

  • 4-Hydroxyphenylpyruvate dioxygenase deficiency
  • Classical phenylketonuria
  • Clinical manifestation of enzyme deficiency
  • Deficiency of fumarylacetoacetase
  • Fumarylacetoacetase deficiency, chronic type
  • Hereditary hypertyrosinemia
  • Hypertyrosinemia
  • Hypertyrosinemia, Richner-Hanhart type
  • Persistent hyperphenylalaninemia
  • Persistent hyperphenylalaninemia AND tyrosinemia
  • Tyrosinemia
  • Tyrosinemia type I
  • Tyrosinemia type III
  • Tyrosinosis

Clinical Information

  • TYROSINEMIAS-. a group of disorders which have in common elevations of tyrosine in the blood and urine secondary to an enzyme deficiency. type i tyrosinemia features episodic weakness self mutilation hepatic necrosis renal tubular injury and seizures and is caused by a deficiency of the enzyme fumarylacetoacetase. type ii tyrosinemia features intellectual disability painful corneal ulcers and keratoses of the palms and plantar surfaces and is caused by a deficiency of the enzyme tyrosine transaminase. type iii tyrosinemia features intellectual disability and is caused by a deficiency of the enzyme 4 hydroxyphenylpyruvate dioxygenase. menkes textbook of child neurology 5th ed pp42 3

Convert E70.21 to ICD-9

  • 270.2 - Arom amin-acid metab NEC (Approximate Flag)

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Disorders of aromatic amino-acid metabolism (E70)

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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Tyrosinemia Tyrosinemia is a genetic disorder characterized by disruptions in the multistep process that breaks down the amino acid tyrosine, a building block of most proteins. If untreated, tyrosine and its byproducts build up in tissues and organs, which can lead to serious health problems.There are three types of tyrosinemia, which are each distinguished by their symptoms and genetic cause. Tyrosinemia type I, the most severe form of this disorder, is characterized by signs and symptoms that begin in the first few months of life. Affected infants fail to gain weight and grow at the expected rate (failure to thrive) due to poor food tolerance because high-protein foods lead to diarrhea and vomiting. Affected infants may also have yellowing of the skin and whites of the eyes (jaundice), a cabbage-like odor, and an increased tendency to bleed (particularly nosebleeds). Tyrosinemia type I can lead to liver and kidney failure, softening and weakening of the bones (rickets), and an increased risk of liver cancer (hepatocellular carcinoma). Some affected children have repeated neurologic crises that consist of changes in mental state, reduced sensation in the arms and legs (peripheral neuropathy), abdominal pain, and respiratory failure. These crises can last from 1 to 7 days. Untreated, children with tyrosinemia type I often do not survive past the age of 10.Tyrosinemia type II can affect the eyes, skin, and mental development. Signs and symptoms often begin in early childhood and include eye pain and redness, excessive tearing, abnormal sensitivity to light (photophobia), and thick, painful skin on the palms of their hands and soles of their feet (palmoplantar hyperkeratosis). About 50 percent of individuals with tyrosinemia type II have some degree of intellectual disability.Tyrosinemia type III is the rarest of the three types. The characteristic features of this type include intellectual disability, seizures, and periodic loss of balance and coordination (intermittent ataxia).About 10 percent of newborns have temporarily elevated levels of tyrosine (transient tyrosinemia). In these cases, the cause is not genetic. The most likely causes are vitamin C deficiency or immature liver enzymes due to premature birth.
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