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Apheresis for Hypercholesterolemia

Source: www.cigna.com
Topic: Familial hypercholesterolemia
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Sort Desciption: Lipid apheresis is an invasive procedure that selectively removes LDL cholesterol and other lipoproteins from the plasma of refractory patients. ...

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Familial hypercholesterolemia (FH) is an inherited anomaly of low-density lipoprotein (LDL) receptors that was first described in 1973 by Goldstein et al. It affects up to 2% of the U.S. population. FH leads to extremely high blood levels of LDL cholesterol, ultimately resulting in early atherosclerotic disease. It is an autosomal-dominant trait that occurs in two forms: heterozygous (i.e., one mutant gene inherited) and homozygous (i.e., two mutant genes inherited). Heterozygous FH occurs in approximately one in 500 persons in the general population, more frequently in men than in women. Plasma cholesterol levels in heterozygous FH are generally in the range of 300â 500 mg/dL (Goldman, 2000). In contrast, the homozygous form of FH, although more severe, is extremely rare, occurring in approximately one case per million persons. Plasma cholesterol levels can exceed 800â 1000 mg/dL in homozygous FH (Goldman, 2000). Early diagnosis and lifelong treatment are essential to reduce the risk of cardiovascular disease, increase life expectancy and improve quality of life. Children with this disorder develop atherosclerotic disease very early in childhood, are at extreme risk of early coronary events and often die suddenly or experience acute myocardial infarction in the first decade of life.

Treatment strategies for FH include the following (Valente, 2001):
* LDL apheresis
* drug therapy
* diet therapy
* orthotopic liver transplant
* gene therapy (a more recent development)

Standard treatment for hypercholesterolemia includes lifestyle changes, diet and lipid-lowering medications. Although most patients with hypercholesterolemia respond to medical management, some patients with heterozygous FH and almost all patients with homozygous FH are refractory to treatment.

Lipid apheresis is an invasive procedure that selectively removes LDL cholesterol and other lipoproteins from the plasma of refractory patients. LDL apheresis involves separation of the patientâ s blood into cellular and plasma fractions, treatment of the plasma to remove the lipoprotein particles and recombination of the plasma and cells, followed by transfusion of the purified blood. The apheresis devices separate blood cells from plasma, using either a filter or a centrifuge. The plasma is then channeled through a purification column that selectively removes particles containing lipoproteins and triglycerides, without removing high-density lipoproteins. Treatment lasts three to four hours, during which time the LDL cholesterol concentration is reduced by 70â 80%. The procedure needs to be repeated approximately every two weeks in severe heterozygous FH and at seven- to 10-day intervals in the rare patient with homozygous FH (Illingworth, 2001).

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