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FAMILIAL HYPERCHOLESTEROLEMIA

Definition

Familial hypercholesterolemia (FH), hepatocyte apolipoprotein-B (apo-B) receptor as a result of a defined mutation in the LDL associated with decreased removal from the liver, autosomal is a dominant disease.

Rationale

Reduce blood LDL concentration to prevent atherosclerosis and the expression of adhesion molecules is reduced. This is the same atheroma plaque formation by reducing the size of xanthoma at the same time by preventing or regressing existing atheroma plaques and/or stabilizing angina pectoris and myocardial infarction prevents its development.

Process

TPD, DFPP, CF, Lipid Aferezi

Processed Volume

1-2 TPH

Frequency

1- every 2 weeks

Replacement Fluid

Albümin

Number and/or duration of applications

Target time-averaged cholesterol level 45-55%, LDL and lipoprotein A levels should be reduced by 40-60%. Treatment continued indefinitely and as described target by lowering time-averaged cholesterol and LDL levels frequency is adjusted so that the level is maintained.
Various patient inclusion criteria used around the world available. One of these criteria can be used.

FDA
  • Familial hypercholesterolemia refractory to medication and diet must be
  • Functional homozygous familial familial with LDL>500 mg/dl hypercholesterolemia
  • LDL>300 mg/dl; no known history of coronary diseasenon-functional heterozygous familial hypercholesterolemia patient
  • LDL>200 mg/dl; functional with a history of coronary disease heterozygous familial hypercholesterolemia patient
Germany
  • In patients with familial hypercholesterolemia in homozygous form,
  • Documented medical treatment for more than twelve months and LDL cholesterol sufficiently as a result of the maximum diet in patients with severe hypercholesterolemia who cannot be lowered,
  • Isolated, clinically and imaging documented Lp(a) level above 60mg/dl and LDL within normal limits progressive cardiovascular disease with cholesterol (coronary heart disease, peripheral artery occlusive disease, cerebrovascular diseases) patients
  • Therefore, by prioritizing the entire risk profile of the patient You need to determine the indications.
International Familial Hypercholesterolemia Management Panel
  • Lipids for patients with homozygous familial hypercholesterolemia apheresis is the standard treatment. Also, despite maximal treatment Symptomatic with LDL >160 mg/dl or less than 40% lowering heterozygous familial coronary heart disease additional that it should be used in patients with hypercholesterolemia is a recommendation.

    To prevent aortic stenosis in homozygous patients starting treatment at an early age (6-7 years) recommended. Familial hypercholesterolemia and conventional LDL, Lp(a), refractory to treatment or not receiving treatment patients can also be treated with lipid apheresis. Pregnancy and uteroplacental blood with very high LDL levels during lipid apheresis in expectant mothers whose flow is at risk can be used for successful termination of pregnancy. High LDL treatment target for high-risk patients is less than 100 mg/dl is recommended to be low.

***The information in this section is taken from the Republic of Turkey Ministry of Health National Apheresis Guidelines.

*THERAPEUTIC APHERESIS TREATMENTS ARE REIMBURSED IN THE HEALTH PRACTICE COMMUNIQUÉ (SUT).

*PLEASE CONSULT YOUR PHYSICIAN ABOUT TREATMENTS.