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Long-term Effect of LDL-Apheresis on Familial Hypercholesterolemia Homozygotes

  • Akira Yamamoto
  • Akito Kawaguchi
  • Mariko Harada-Shiba
Conference paper

Summary

LDL-apheresis is the practical way of treatment for homozygous cases of familial hypercholesterolemia (FH). Long-term observations for 2–5 years have shown that atheromatous plaques can regress in major arteries including coronary arteries by repeating LDL-apheresis once a week or every two weeks. However, the observation for much longer time revealed that atherosclerotic vascular lesions relaps or progress with the involvement of the aortic valve being the most obstinate complication. Important problem is the severe rebound of cholesterol taking place after the apheresis and it is necessary to suppress this rebound as much as possible, so that we can keep the post-apheresis LDL-cholesterol levels in or near the optimal range as long as possible. It has been thought that statins are ineffective in patients with homozygous FH because of the lack of LDL-receptors. A recent trial carried out in South Africa showed atorvastatin being capable of suppressing cholesterol synthesis and useful in reducing the rebound in patients with homozygous FH under apheresis treatment. Another clinical trial carried out in Japan also showed the efficacy of the statin in such patients, suggesting that the statin is able to decrease cholesterol by inhibiting the synthesis of cholesterol and VLDL even in the absence of up-regulation of the LDL-receptor.

Keywords

Aortic Valve Aortic Regurgitation Familial Hypercholesterolemia Familial Hypercholesterolemia Atheromatous Plaque 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Tokyo 2000

Authors and Affiliations

  • Akira Yamamoto
    • 1
  • Akito Kawaguchi
    • 1
  • Mariko Harada-Shiba
    • 1
  1. 1.National Cardiovascular Center Research InstituteSuita, OsakaJapan

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