The Japanese Journal of Clinical Dialysis Vol.27 No.12(3)

Theme Apheresis Update
Title Current state of apheresis therapy in dyslipidemia
Publish Date 2011/11
Author Noriyuki Iwamoto Division of Endocrinology and Metabolism, National Cerebral and Cardiovascular Center
Author Mariko Harada-Shiba Department of Molecular Innovation in Lipidology, National Cerebral and Cardiovascular Center Research Institute
[ Summary ] Low density lipoprotein (LDL) apheresis (LDL-A) was developed for treatment of homozygous familial hypercholesterolemia (FH) caused by a mutation of the LDL receptor related gene. Homozygous FH is characterized by very high levels of serum cholesterol (450-1,000 mg/dl), skin and tendon xanthomas and diseases such as coronary artery disease, aortic valve stenosis and supravalvular stenosis caused by premature atherosclerosis. Without any treatment, homozygous FH patients have poor prognoses. The prognosis for patients with homozygous FH has been improved due to early introduction of LDL-A treatment. Medical insurance has provided coverage not only for homozygous FH but also to for heterozygous FH having resistance to medication and coronary artery disease. Homozygous FH has been certified as "Tokutei-Shikkan", which means the patients can receive special financial support for medical expenses.
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