ICD-10 Diagnosis Code E78.3


Diagnosis Code E78.3

ICD-10: E78.3
Short Description: Hyperchylomicronemia
Long Description: Hyperchylomicronemia
This is the 2017 version of the ICD-10-CM diagnosis code E78.3

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

Information for Medical Professionals

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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.
  • 272.3 - Hyperchylomicronemia

  • Chylomicron retention disease
  • Chylomicronemia syndrome
  • Endogenous hyperlipidemia
  • Familial apolipoprotein C-II deficiency
  • Familial hyperchylomicronemia
  • Familial hypertriglyceridemia
  • Familial hypertriglyceridemia
  • Familial lipoprotein lipase deficiency
  • Familial lipoprotein lipase deficiency with type I phenotype
  • Familial lipoprotein lipase deficiency with type V phenotype
  • Familial type 5 hyperlipoproteinemia
  • Fredrickson type I hyperlipoproteinemia
  • Hyperlipidemia with lipid deposition in skin
  • Hyperlipoproteinemia
  • Hyperlipoproteinemia
  • Lipidemia retinalis
  • Primary acquired chylomicronemia
  • Primary chylomicronemia
  • Primary genetic hyperlipidemia
  • Primary hypertriglyceridemia
  • Primary hypertriglyceridemia

Index of Diseases and Injuries
References found for the code E78.3 in the Index of Diseases and Injuries:

Information for Patients


Also called: HDL, Hypercholesterolemia, Hyperlipidemia, Hyperlipoproteinemia, LDL

Cholesterol is a waxy, fat-like substance that occurs naturally in all parts of the body. Your body needs some cholesterol to work properly. But if you have too much in your blood, it can combine with other substances in the blood and stick to the walls of your arteries. This is called plaque. Plaque can narrow your arteries or even block them.

High levels of cholesterol in the blood can increase your risk of heart disease. Your cholesterol levels tend to rise as you get older. There are usually no signs or symptoms that you have high blood cholesterol, but it can be detected with a blood test. You are likely to have high cholesterol if members of your family have it, if you are overweight or if you eat a lot of fatty foods.

You can lower your cholesterol by exercising more and eating more fruits and vegetables. You also may need to take medicine to lower your cholesterol.

NIH: National Heart, Lung, and Blood Institute

  • Cholesterol - drug treatment
  • Cholesterol and lifestyle
  • Cholesterol testing and results
  • Familial combined hyperlipidemia
  • Familial hypercholesterolemia
  • High blood cholesterol levels
  • How to take statins
  • Talk with Your Health Care Provider about High Cholesterol (Agency for Healthcare Research and Quality)
  • VLDL test

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Triglycerides are a type of fat found in your blood. Too much of this type of fat may raise the risk of coronary artery disease, especially in women.

A blood test measures your triglycerides along with your cholesterol. Normal triglyceride levels are below 150. Levels above 200 are high.

Factors that can raise your triglyceride level include

  • Being overweight
  • Lack of physical activity
  • Smoking
  • Excessive alcohol use
  • A very high carbohydrate diet
  • Certain diseases and medicines
  • Some genetic disorders

You may be able to lower your triglycerides with a combination of losing weight, diet, and exercise. You also may need to take medicine to lower your triglycerides.

NIH: National Heart, Lung, and Blood Institute

  • Familial hypertriglyceridemia
  • Fibrates
  • Triglyceride level
  • VLDL test

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Chylomicron retention disease Chylomicron retention disease is an inherited disorder that affects the absorption of dietary fats, cholesterol, and certain fat-soluble vitamins. As food is digested after a meal, molecules called chylomicrons are formed to carry fat and cholesterol from the intestine into the bloodstream. Chylomicrons are also necessary for the absorption of certain fat-soluble vitamins, such as vitamin E and vitamin D. A lack of chylomicron transport causes severely decreased absorption (malabsorption) of dietary fats and fat-soluble vitamins. Sufficient levels of fats, cholesterol, and vitamins are necessary for normal growth and development.The signs and symptoms of chylomicron retention disease 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; 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. Affected people may eventually develop decreased reflexes (hyporeflexia) and a decreased ability to feel vibrations.
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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.
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