ICD-10 Diagnosis Code E78.6

Lipoprotein deficiency

Diagnosis Code E78.6

ICD-10: E78.6
Short Description: Lipoprotein deficiency
Long Description: Lipoprotein deficiency
This is the 2018 version of the ICD-10-CM diagnosis code E78.6

Valid for Submission
The code E78.6 is valid for submission for HIPAA-covered transactions.

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

Information for Medical Professionals

Convert to ICD-9 Additional informationCallout TooltipGeneral Equivalence Map
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.
  • 272.5 - Lipoprotein deficiencies

  • 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
  • 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

Index of Diseases and Injuries
References found for the code E78.6 in the Index of Diseases and Injuries:

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 affects the absorption of dietary fats, cholesterol, and fat-soluble vitamins. People affected by this disorder are not able to make certain lipoproteins, which are particles that carry fats and fat-like substances (such as cholesterol) in the blood. Specifically, people with abetalipoproteinemia are missing a group of lipoproteins called beta-lipoproteins. An inability to make beta-lipoproteins causes severely reduced absorption (malabsorption) of dietary fats and fat-soluble vitamins (vitamins A, D, E, and K) from the digestive tract into the bloodstream. Sufficient levels of fats, cholesterol, and vitamins are necessary for normal growth, development, and maintenance of the body's cells and tissues, particularly nerve cells and tissues in the eye.The signs and symptoms of abetalipoproteinemia appear in the first few months of life. They can include failure to gain weight and grow at the expected rate (failure to thrive); diarrhea; abnormal star-shaped red blood cells (acanthocytosis); and fatty, foul-smelling stools (steatorrhea). Other features of this disorder may develop later in childhood and often impair the function of the nervous system. Disturbances in nerve function may cause affected people to eventually develop poor muscle coordination and difficulty with balance and movement (ataxia). Individuals with this condition may also develop an eye disorder called retinitis pigmentosa, in which progressive degeneration of the light-sensitive layer (retina) at the back of the eye can cause vision loss. Adults in their thirties or forties may have increasing difficulty with balance and walking. Many of the signs and symptoms of abetalipoproteinemia result from a severe vitamin deficiency, especially a deficiency of vitamin E.
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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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