hypolipoproteinemia

Hypolipoproteinemia 

Definition

Hypolipoproteinemia (or hypolipidemia) is the lack of fat in the blood.

Description

Although quite rare, hypolipoproteinemia is a serious condition. Blood absorbs fat from food in the intestine and transports it as a combined package with proteins and other chemicals like cholesterol. Much of the fat goes straight into the liver for processing. The cholesterol, a waste product, ends up in the bile. The proteins act as vessels, carrying the other chemicals around. These packages of fat, cholesterol, and proteins are called lipoproteins.

Causes and symptoms

Low blood fats can be the result of several diseases, or they can be a primary genetic disease with other associated abnormalities.

• Malnutrition is a lack of food, including fats, in the diet.
• Malabsorption is the inability of the bowel to absorb food, causing malnutrition.
• Anemia (too few red blood cells) and hyperthyroidism (too much thyroid hormone) also reduce blood fats.
• Rare genetic conditions called hypobetalipoproteinemia and abetalipoproteinemia cause malabsorption plus nerve, eye, and skin problems in early childhood.
• Tangier disease, causes only the cholesterol to be low. It also produces nerve and eye problems in children.

Symptoms are associated more closely with the cause rather than the actual low blood fats.

Diagnosis

Blood studies of the various fat particles help identify both the low and high fat diseases. These tests are often done after an overnight fast to prevent interference from fat just being absorbed from food. Fats and proteins are grouped together and described by density—high-density lipoproteins (HDL), low-density lipoproteins (LDL), and very low-density lipoproteins (VLDL). There are also much bigger particles called chylomicrons. Each contain different proportions of cholesterol, fats, and protein.

Treatment

Supplemental vitamin E helps children with the betalipoprotein deficiencies. There is no known treatment for Tangier disease. Treatment of the causes of the other forms of low blood fats reverses the condition.

Resources

Books

Ginsberg, Henry N., and Ira J. Goldberg. "Disorders Of Lipoprotein Metabolism." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.

Key terms

Cholesterol — A steroid alcohol found in animal cells and fluids.

Lipoprotein — Class of proteins that contain protein and lipid. The fundamental component of living cells.

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hypolipoproteinemia

[hī′pōlip′ōprō′tēnē′mē·ə]

Etymology: Gk, hypo + lipos, fat, proteios, first rank, haima, blood

a group of defects of lipoprotein metabolism that cause varying complexes of signs. Primary, or hereditary, hypolipoproteinemia factors include abnormal transport of triglycerides in the blood, low levels of high-density lipoproteins, and abnormal fat deposits in the body, especially in the kidneys and the liver. In some of the syndromes ocular, cardiovascular intestinal, and neurologic effects are also present. The condition also may be secondary to anemia, malabsorption syndromes, or malnutrition. Kinds of hypolipoproteinemia are abetalipoproteinemia, familial hypocalphalipoproteinemia, hypobetalipoproteinemia, lecithin-cholesterol acyltransferase deficiency, and Tangier disease. Also called hypolipidemia.

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hypolipoproteinemia

the presence of lipoproteins below normal concentrations in the blood.

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hypolipoproteinemia

A group of conditions characterized by ↓ lipoproteins, either acquired–eg, 2º to malabsorption, anemia, hyperthyroidism, or congenital