DRG Category: 642
Mean LOS: 4.9 days
Description MEDICAL: Inborn Errors of Metabolism
Hyperlipoproteinemia is a condition of increased lipoprotein particles (fats) in the blood caused by an increased rate of synthesis or a decreased rate of lipoprotein breakdown. Because lipoproteins transport triglycerides and cholesterol in the plasma, an increased level may cause pancreatitis and atherosclerosis.
Lipids are a mixed group of biochemical substances manufactured by the body or derived from metabolism of ingested substances. The plasma lipids (cholesterols, triglycerides, phospholipids, and free fatty acids) are derived from dietary sources and lipid synthesis. Cholesterol and triglycerides are implicated in atherogenesis. Hyperlipemia, also known as hyperlipidemia, occurs with elevated plasma cholesterol or triglyceride levels or both and is present in all hyperlipoproteinemias.
Hyperlipidemia, an elevation of serum cholesterol or triglycerides, can be primary or secondary to another underlying condition. Lipoprotein elevation, or hyperlipoproteinemia, is described by five specific types: types I, II, III, IV, and V (Table 3).
Types of Hyperlipoproteinemia
|TYPE||DEFINITION AND CAUSE||ASSESSMENT||LIPID LEVEL|
|I||Fat-induced hyperlipidemia or idiopathic familial hyperlipidemia, which is a rare condition caused by deficient or abnormal lipase; rare genetic disorder that is present in infancy||Recurrent attacks of severe abdominal pain after fat intake; malaise; anorexia||Triglycerides 1,00010,000+ mg/dL|
|II||Familial hyperbetalipoproteinemia and essential familial hypercholesterolemia because of deficient cell surface receptors||Chest pain from prematurely accelerated coronary artery disease; tendinous xanthomas (firm masses) on Achilles' tendons, tendons of hands and feet; juvenile corneal arcus (grayish ring around the cornea of the eye)||Elevated cholesterol and triglycerides, elevated low-density lipoproteins (LDL) and very low-density lipoproteins (VLDL)|
|III||Familial broad-beta disease xanthoma tuberosum caused by a deficient low-density lipoprotein receptor||Chest pain from early progression of atherosclerosis; xanthomas over elbows, knees, palms, and fingertips||Elevated cholesterol and triglycerides; elevated intermediate-density lipoproteins|
|IV||Endogenous hypertriglyceridemia and hyperbetalipoproteinemia with an idiopathic cause; often associated with obesity and diabetes||Chest pain from early progression of coronary artery disease; obesity, hypertension||Triglycerides < 1,000 mg/dL, normal cholesterol, elevated VLDL|
|V||Mixed hypertriglyceridemia from defective triglyceride clearance; often secondary to other disorders such as renal disease or obesity||Abdominal pain from pancreatitis; visual changes; xanthomas on arms and legs; enlarged liver and spleen||Triglycerides > 1,000 mg/dL, elevated cholesterol, normal LDL|
Hyperlipoproteinemia has been found in Diseases and Disorders
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