Valid for Submission
E78.3 is a billable diagnosis code used to specify a medical diagnosis of hyperchylomicronemia. The code E78.3 is valid during the fiscal year 2021 from October 01, 2020 through September 30, 2021 for the submission of HIPAA-covered transactions.
The ICD-10-CM code E78.3 might also be used to specify conditions or terms like chylomicron retention disease, chylomicronemia syndrome, endogenous hyperlipidemia, familial apolipoprotein c-ii deficiency, familial hyperchylomicronemia , familial hypertriglyceridemia, etc.
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 E78.3:
Inclusion TermsInclusion 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.
- Chylomicron retention disease
- Fredrickson's hyperlipoproteinemia, type I or V
- Hyperlipidemia, group D
- Mixed hyperglyceridemia
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 E78.3 are found in the index:
- - Bürger-Grütz disease or syndrome - E78.3
- - Disease, diseased - See Also: Syndrome;
- - Prebetalipoproteinemia (acquired) (essential) (familial) (hereditary) (primary) (secondary) - E78.1
- - with chylomicronemia - E78.3
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Chylomicron retention disease
- Chylomicronemia syndrome
- Endogenous hyperlipidemia
- Familial apolipoprotein C-II deficiency
- Familial hyperchylomicronemia
- Familial hypertriglyceridemia
- Familial hypertriglyceridemia
- Familial lipoprotein lipase deficiency with type I phenotype
- Familial lipoprotein lipase deficiency with type V phenotype
- Familial type 5 hyperlipoproteinemia
- Hyperlipidemia with lipid deposition in skin
- Hyperlipoproteinemia, type I
- Lipidemia retinalis
- Primary acquired chylomicronemia
- Primary chylomicronemia
- Primary genetic hyperlipidemia
- HYPERLIPOPROTEINEMIA TYPE V-. a severe type of hyperlipidemia sometimes familial that is characterized by the elevation of both plasma chylomicrons and triglycerides contained in very low density lipoproteins. type v hyperlipoproteinemia is often associated with diabetes mellitus and is not caused by reduced lipoprotein lipase activity as in hyperlipoproteinemia type i .
- HYPERLIPOPROTEINEMIA TYPE I-. an inherited condition due to a deficiency of either lipoprotein lipase or apolipoprotein c ii a lipase activating protein. the lack of lipase activities results in inability to remove chylomicrons and triglycerides from the blood which has a creamy top layer after standing.
Convert E78.3 to ICD-9 Code
Information for Patients
Also called: Hypercholesterolemia, Hyperlipidemia, Hyperlipoproteinemia
What is cholesterol?
Cholesterol is a waxy, fat-like substance that's found in all the cells in your body. Your body needs some cholesterol to make hormones, vitamin D, and substances that help you digest foods. Your body makes all the cholesterol it needs. Cholesterol is also found in foods from animal sources, such as egg yolks, meat, and cheese.
If you have too much cholesterol in your blood, it can combine with other substances in the blood to form plaque. Plaque sticks to the walls of your arteries. This buildup of plaque is known as atherosclerosis. It can lead to coronary artery disease, where your coronary arteries become narrow or even blocked.
What are HDL, LDL, and VLDL?
HDL, LDL, and VLDL are lipoproteins. They are a combination of fat (lipid) and protein. The lipids need to be attached to the proteins so they can move through the blood. Different types of lipoproteins have different purposes:
- HDL stands for high-density lipoprotein. It is sometimes called "good" cholesterol because it carries cholesterol from other parts of your body back to your liver. Your liver then removes the cholesterol from your body.
- LDL stands for low-density lipoprotein. It is sometimes called "bad" cholesterol because a high LDL level leads to the buildup of plaque in your arteries.
- VLDL stands for very low-density lipoprotein. Some people also call VLDL a "bad" cholesterol because it too contributes to the buildup of plaque in your arteries. But VLDL and LDL are different; VLDL mainly carries triglycerides and LDL mainly carries cholesterol.
What causes high cholesterol?
The most common cause of high cholesterol is an unhealthy lifestyle. This can include
- Unhealthy eating habits, such as eating lots of bad fats. One type, saturated fat, is found in some meats, dairy products, chocolate, baked goods, and deep-fried and processed foods. Another type, trans fat, is in some fried and processed foods. Eating these fats can raise your LDL (bad) cholesterol.
- Lack of physical activity, with lots of sitting and little exercise. This lowers your HDL (good) cholesterol.
- Smoking, which lowers HDL cholesterol, especially in women. It also raises your LDL cholesterol.
Genetics may also cause people to have high cholesterol. For example, familial hypercholesterolemia (FH) is an inherited form of high cholesterol. Other medical conditions and certain medicines may also cause high cholesterol.
What can raise my risk of high cholesterol?
A variety of things can raise your risk for high cholesterol:
- Age. Your cholesterol levels tend to rise as you get older. Even though it is less common, younger people, including children and teens, can also have high cholesterol.
- Heredity. High blood cholesterol can run in families.
- Weight. Being overweight or having obesity raises your cholesterol level.
- Race. Certain races may have an increased risk of high cholesterol. For example, African Americans typically have higher HDL and LDL cholesterol levels than whites.
- Weight. Being overweight or having obesity raises your cholesterol level.
What health problems can high cholesterol cause?
If you have large deposits of plaque in your arteries, an area of plaque can rupture (break open). This can cause a blood clot to form on the surface of the plaque. If the clot becomes large enough, it can mostly or completely block blood flow in a coronary artery.
If the flow of oxygen-rich blood to your heart muscle is reduced or blocked, it can cause angina (chest pain) or a heart attack.
Plaque also can build up in other arteries in your body, including the arteries that bring oxygen-rich blood to your brain and limbs. This can lead to problems such as carotid artery disease, stroke, and peripheral arterial disease.
How is high cholesterol diagnosed?
There are usually no signs or symptoms that you have high cholesterol. There is a blood test to measure your cholesterol level. When and how often you should get this test depends on your age, risk factors, and family history. The general recommendations are:
For people who are age 19 or younger:
- The first test should be between ages 9 to 11
- Children should have the test again every 5 years
- Some children may have this test starting at age 2 if there is a family history of high blood cholesterol, heart attack, or stroke
For people who are age 20 or older:
- Younger adults should have the test every 5 years
- Men ages 45 to 65 and women ages 55 to 65 should have it every 1 to 2 years
How can I lower my cholesterol?
You can lower your cholesterol through heart-healthy lifestyle changes. They include a heart-healthy eating plan, weight management, and regular physical activity.
If the lifestyle changes alone do not lower your cholesterol enough, you may also need to take medicines. There are several types of cholesterol-lowering drugs available, including statins. If you take medicines to lower your cholesterol, you still should continue with the lifestyle changes.
Some people with familial hypercholesterolemia (FH) may receive a treatment called lipoprotein apheresis. This treatment uses a filtering machine to remove LDL cholesterol from the blood. Then the machine returns the rest of the blood back to the person.
NIH: National Heart, Lung, and Blood Institute
- Cholesterol and lifestyle (Medical Encyclopedia)
- Cholesterol testing and results (Medical Encyclopedia)
- Familial combined hyperlipidemia (Medical Encyclopedia)
- Familial hypercholesterolemia (Medical Encyclopedia)
- High cholesterol - children (Medical Encyclopedia)
What are triglycerides?
Triglycerides are a type of fat. They are the most common type of fat in your body. They come from foods, especially butter, oils, and other fats you eat. Triglycerides also come from extra calories. These are the calories that you eat, but your body does not need right away.Your body changes these extra calories into triglycerides, and stores them in fat cells. When your body needs energy, it releases the triglycerides. Your VLDL cholesterol particles carry the triglycerides to your tissues.
Having a high level of triglycerides can raise your risk of heart diseases, such as coronary artery disease.
What causes high triglycerides?
Factors that can raise your triglyceride level include
- Regularly eating more calories that you burn off, especially if you eat a lot of sugar
- Being overweight of having obesity
- Cigarette smoking
- Excessive alcohol use
- Certain medicines
- Some genetic disorders
- Thyroid diseases
- Poorly controlled type 2 diabetes
- Liver or kidney diseases
How are high triglycerides diagnosed?
There is a blood test that measures your triglycerides, along with your cholesterol. Triglyceride levels are measured in milligrams per deciliter (mg/dL). The guidelines for triglyceride levels are
|Normal||Less than 150mg/dL|
|Borderline high||150 to 199 mg/dL|
|High||200 to 499 mg/dL|
|Very high||500 mg/dL and above|
Levels above 150mg/dl may raise your risk for heart disease. A triglyceride level of 150 mg/dL or higher is also a risk factor for metabolic syndrome.
What are the treatments for high triglycerides?
You may be able to lower your triglyceride levels with lifestyle changes:
- Controlling your weight
- Regular physical activity
- Not smoking
- Limiting sugar and refined foods
- Limiting alcohol
- Switching from saturated fats to healthier fats
Some people will also need to take cholesterol medicines to lower their triglycerides.
- Familial hypertriglyceridemia (Medical Encyclopedia)
- Fibrates (Medical Encyclopedia)
- Triglyceride level (Medical Encyclopedia)
- VLDL test (Medical Encyclopedia)
Chylomicron retention disease Chylomicron retention disease is an inherited disorder that impairs the normal absorption of fats, cholesterol, and certain vitamins from food. The features of chylomicron retention disease primarily affect the gastrointestinal system and nervous system.Chylomicron retention disease begins in infancy or early childhood. Affected children have slow growth and weight gain, frequent (chronic) diarrhea, and foul-smelling stools (steatorrhea). They also have reduced blood cholesterol levels (hypocholesterolemia). Some individuals with chylomicron retention disease develop an abnormal buildup of fats in the liver called hepatic stenosis and can have an enlarged liver.Other features of chylomicron retention disease develop later in childhood and often impair the function of the nervous system. Affected people may develop decreased reflexes (hyporeflexia) and a decreased ability to sense vibrations. Rarely, affected individuals have heart abnormalities or muscle wasting (amyotrophy).
Familial lipoprotein lipase deficiency Familial lipoprotein lipase deficiency is an inherited condition that disrupts the normal breakdown of fats in the body, resulting in an increase of certain kinds of fats.People with familial lipoprotein lipase deficiency typically develop signs and symptoms before age 10, with one-quarter showing symptoms by age 1. The first symptom of this condition is usually abdominal pain, which can vary from mild to severe. The abdominal pain is often due to inflammation of the pancreas (pancreatitis). These episodes of pancreatitis begin as sudden (acute) attacks. If left untreated, pancreatitis can develop into a chronic condition that can damage the pancreas and, in rare cases, be life-threatening.Affected individuals may also have an enlarged liver and spleen (hepatosplenomegaly). The higher the levels of fat in the body, the larger the liver and spleen become. As fat levels rise, certain white blood cells called macrophages take in excess fat in an attempt to rid fat from the bloodstream. After taking in fat, the macrophages travel to the liver and spleen, where the fatty cells accumulate.Approximately half of individuals with familial lipoprotein lipase deficiency develop small yellow deposits of fat under the skin called eruptive xanthomas. These fat deposits most commonly appear on the trunk, buttocks, knees, and arms. Eruptive xanthomas are small (about 1 millimeter in diameter), but individual xanthomas can cluster together to form larger patches. They are generally not painful unless exposed to repeated friction or abrasion. Eruptive xanthomas begin to appear when fat intake increases and levels rise; the deposits disappear when fat intake slows and levels decrease.The blood of people with familial lipoprotein lipase deficiency can have a milky appearance due to its high fat content. When fat levels get very high in people with this condition, fats can accumulate in blood vessels in the tissue that lines the back of the eye (the retina). The fat buildup gives this tissue a pale pink appearance when examined (lipemia retinalis). This fat accumulation does not affect vision and will disappear once fats from the diet are reduced and levels in the body decrease.In people with familial lipoprotein lipase deficiency, increased fat levels can also cause neurological features, such as depression, memory loss, and mild intellectual decline (dementia). These problems are remedied when dietary fat levels normalize.