ICD-10-CM Code E78.6

Lipoprotein deficiency

Version 2020 Billable Code

Valid for Submission

E78.6 is a billable code used to specify a medical diagnosis of lipoprotein deficiency. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code E78.6 might also be used to specify conditions or terms like abetalipoproteinemia, acanthocytosis, apo a-i giessen variant, apo a-i marburg variant, apo a-i milano variant, apo a-i variant fisheye-like syndrome, etc

ICD-10:E78.6
Short Description:Lipoprotein deficiency
Long Description:Lipoprotein deficiency

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 E78.6:

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.
  • Abetalipoproteinemia
  • Depressed HDL cholesterol
  • High-density lipoprotein deficiency
  • Hypoalphalipoproteinemia
  • Hypobetalipoproteinemia (familial)
  • Lecithin cholesterol acyltransferase deficiency
  • Tangier disease

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 E78.6 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:

  • Abetalipoproteinemia
  • Acanthocytosis
  • Apo A-I Giessen variant
  • Apo A-I Marburg variant
  • Apo A-I Milano variant
  • Apo A-I variant fisheye-like syndrome
  • ApoA-I Munster variant 1
  • ApoA-I Munster variant 2
  • ApoA-I Munster variant 3
  • Apolipoprotein A-I deficiency
  • Apolipoprotein A-I variant disorder
  • Ataxia co-occurrent and due to abetalipoproteinemia
  • Chorea acanthocytosis syndrome
  • Familial hypoalphalipoproteinemia
  • Familial hypobetalipoproteinemia
  • Familial hypobetalipoproteinemia - heterozygous form
  • Familial hypobetalipoproteinemia - homozygous form
  • Familial hypolipoproteinemia
  • Familial lipoprotein deficiency
  • Fish-eye disease
  • Hereditary acanthocytosis
  • High density lipoprotein deficiency
  • Hypoalphalipoproteinemia
  • Hypo-beta-lipoproteinemia
  • Hypocholesterolemia
  • Hypolipoproteinemia
  • Inborn error of lipoprotein metabolism
  • Lecithin cholesterol acyltransferase deficiency
  • Lipoprotein deficiency disorder
  • Phosphatidylcholine-sterol acyltransferase deficiency
  • Tangier disease

Clinical Information

  • HYPOALPHALIPOPROTEINEMIAS-. conditions with abnormally low levels of alpha lipoproteins high density lipoproteins in the blood. hypoalphalipoproteinemia can be associated with mutations in genes encoding apolipoprotein a i; lecithin cholesterol acyltransferase; and atp binding cassette transporters.

Convert E78.6 to ICD-9

  • 272.5 - Lipoprotein deficiencies

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Disorders of lipoprotein metabolism and other lipidemias (E78)

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

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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Abetalipoproteinemia Abetalipoproteinemia is an inherited disorder that impairs the normal absorption of fats and certain vitamins from the diet. Many of the signs and symptoms of abetalipoproteinemia result from a severe shortage (deficiency) of fat-soluble vitamins (vitamins A, E, and K). The signs and symptoms of this condition primarily affect the gastrointestinal system, eyes, nervous system, and blood.The first signs and symptoms of abetalipoproteinemia appear in infancy. They often include failure to gain weight and grow at the expected rate (failure to thrive); diarrhea; and fatty, foul-smelling stools (steatorrhea).As an individual with this condition ages, additional signs and symptoms include disturbances in nerve function that may lead to poor muscle coordination and difficulty with balance and movement (ataxia). They can also experience a loss of certain reflexes, impaired speech (dysarthria), tremors or other involuntary movements (motor tics), a loss of sensation in the extremities (peripheral neuropathy), or muscle weakness. The muscle problems can disrupt skeletal development, leading to an abnormally curved lower back (lordosis), a rounded upper back that also curves to the side (kyphoscoliosis), high-arched feet (pes cavus), or an inward- and upward-turning foot (clubfoot).Individuals with this condition may also develop an eye disorder called retinitis pigmentosa, in which breakdown of the light-sensitive layer (retina) at the back of the eye can cause vision loss. In individuals with abetalipoproteinemia, the retinitis pigmentosa can result in complete vision loss. People with abetalipoproteinemia may also have other eye problems, including involuntary eye movements (nystagmus), eyes that do not look in the same direction (strabismus), and weakness of the external muscles of the eye (ophthalmoplegia).Individuals with abetalipoproteinemia usually have a low number of red blood cells (anemia) with abnormally star-shaped red blood cells (acanthocytosis) and have difficulty forming blood clots, which can cause abnormal bleeding. In some cases, a condition called fatty liver develops, which can cause liver damage.
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Familial hypobetalipoproteinemia Familial hypobetalipoproteinemia (FHBL) is a disorder that impairs the body's ability to absorb and transport fats. This condition is characterized by low levels of a fat-like substance called cholesterol in the blood. The severity of signs and symptoms experienced by people with FHBL vary widely. The most mildly affected individuals have few problems with absorbing fats from the diet and no related signs and symptoms. Many individuals with FHBL develop an abnormal buildup of fats in the liver called hepatic steatosis or fatty liver. In more severely affected individuals, fatty liver may progress to chronic liver disease (cirrhosis). Individuals with severe FHBL have greater difficulty absorbing fats as well as fat-soluble vitamins such as vitamin E and vitamin A. This difficulty in fat absorption leads to excess fat in the feces (steatorrhea). In childhood, these digestive problems can result in an inability to grow or gain weight at the expected rate (failure to thrive).
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