2024 ICD-10-CM Diagnosis Code E78.3

Approximate Synonyms

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
• Endogenous hyperlipidemia
• Endogenous hyperlipidemia
• Endogenous hyperlipidemia
• Familial apolipoprotein C-II deficiency
• Familial chylomicronemia syndrome
• Familial hyperchylomicronemia
• Familial hypertriglyceridemia
• Familial hypertriglyceridemia
• 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
• Hyperlipidemia with lipid deposition in skin
• Hyperlipidemia with lipid deposition in skin
• Hyperlipidemia with lipid deposition in skin
• Hyperlipidemia with lipid deposition in skin
• Hyperlipidemia with lipid deposition in skin
• Hyperlipoproteinemia
• Hyperlipoproteinemia, type I
• Lipidemia retinalis
• Primary acquired chylomicronemia
• Primary chylomicronemia
• Primary genetic hyperlipidemia
• Primary genetic hyperlipidemia
• Primary genetic hyperlipidemia
• Primary genetic hyperlipidemia
• Primary hypertriglyceridemia
• Primary hypertriglyceridemia
• Primary hypertriglyceridemia
• Primary hypertriglyceridemia
• Xanthoma due to abnormality of lipid metabolism
• Xanthoma due to primary chylomicronemia

Clinical Information

• 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.

• Hyperlipoproteinemia Type II

  a group of familial disorders characterized by elevated circulating cholesterol contained in either low-density lipoproteins alone or also in very-low-density lipoproteins (pre-beta lipoproteins).

• Hyperlipoproteinemia Type III

  an autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (idl or broad-beta-lipoprotein). idl has a cholesterol to triglycerides ratio greater than that of very-low-density lipoproteins. this disorder is due to mutation of apolipoproteins e, a receptor-binding component of vldl and chylomicrons, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.

• Hyperlipoproteinemia Type IV

  a hypertriglyceridemia disorder, often with autosomal dominant inheritance. it is characterized by the persistent elevations of plasma triglycerides, endogenously synthesized and contained predominantly in very-low-density lipoproteins (pre-beta lipoproteins). in contrast, the plasma cholesterol and phospholipids usually remain within normal limits.

• Hyperlipoproteinemia

  an elevated concentration of lipoproteins.

• Hyperlipoproteinemia, Type I|Familial Essential Hyperlipemia|Familial Hyperchylomicronemia|Hyperlipoproteinemia Type 1A|Lipoprotein Lipase Deficiency

  a genetic disorder of lipoprotein metabolism caused by mutations in the lpl and apolipoprotein (apo) c-ii genes. it is characterized by increased levels of chylomicrons and triglycerides in the blood.

• Hyperlipoproteinemia, Type I|Familial Essential Hyperlipemia|Familial Hyperchylomicronemia|Hyperlipoproteinemia Type 1A|Lipoprotein Lipase Deficiency

  a genetic condition caused by mutation(s) in the lpl gene, encoding lipoprotein lipase, resulting in reduced activity of the enzyme lipoprotein lipase.

• Hyperlipoproteinemia, Type II|Type II Hyperlipidemia

  an inheritable form of hyperlipidemia, in which there are excess lipids in the blood.

• Hyperlipoproteinemia, Type IIa|FH|Familial Hypercholesterolemia|Hyperlipidemia Type IIa|Type IIa Hyperlipidemia

  an autosomal dominant inherited disorder characterized by very high levels of low-density lipoprotein cholesterol (ldl-c) and total cholesterol in the blood. it is usually caused by mutations in the ldlr gene which is located on the short arm of chromosome 19.

• Hyperlipoproteinemia, Type IIb|Familial Combined Hyperlipidemia|Hyperlipidemia Type IIb|Type IIb Hyperlipidemia|Type IIb Hyperlipoproteinemia

  a disorder of lipoprotein metabolism characterized by high levels of cholesterol and triglycerides in the blood. it is caused by elevation of low density and very low density lipoproteins.

Convert E78.3 to ICD-9-CM

• ICD-9-CM Code: 272.3 - Hyperchylomicronemia

Patient Education

Cholesterol

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.

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

[Learn More in MedlinePlus]

Triglycerides

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 than you burn off, especially if you eat a lot of sugar
• Being overweight or 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

Category	Triglyceride Level
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.

[Learn More in MedlinePlus]

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).

[Learn More in MedlinePlus]

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.

[Learn More in MedlinePlus]

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.