Skip to main content
SpringerLink
Log in

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

  • Conference paper
Lipoprotein Metabolism and Atherogenesis

  • 473 Accesses

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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 54.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Illingworth R (1993) How effective is drug therapy in heterozygous familial hypercholesterolemia. Am J Cardiol 72: D54–D58.

    Article  Google Scholar 

  2. Bilheimer DW (1989) Portacaval shunt and liver transplantation in treatment of familial hypercholesterolemia

    Google Scholar 

  3. Grossman M, Raper SE, Kozarsky K, Stein EA, Engelhardt JF, Muller D, Lupien P-J, Wilson JM (1994) Successful ex vivo gene therapy directed to liver in a patient with familial hypercholesterolemia. Nature Genet 6: 335–341.

    Article  PubMed  CAS  Google Scholar 

  4. Yamamoto A, Kojima, S, Harada-Shiba M, Toyota, Y, Takamiya M, Tsushima M, Kishino B, Koga N, Tatami R (1995) Plasmapheresis for prevention and regression of cornary atherosclerosis. Ann NY Acad Sci 748: 429–440.

    Article  PubMed  CAS  Google Scholar 

  5. DeGenne JL, Toutaine R, Maunand B, Truffert J, Laudat P (1967) Forme homozygotes cutaneo-tendineuses de Xanthomatose hypercholesterolemique dans une observation familiale exemplaire. Essai de Plasmapherese a titre de traitment heroique. Bull Mem Soc Hop Paris 118: 1377–1402.

    Google Scholar 

  6. Thompson GR, Myant NB, Kilpatrick D, Oakley CM, Raphael MJ, Steiner RE (1980) Assessment of long-term plasma exchange for familial hypercholesterolemia. Brit Heart J 43: 680–688.

    Article  PubMed  CAS  Google Scholar 

  7. Yokoyama S, Hayashi R, Satani M, Yamamoto A (1985) Specific removal of low-density-lipoprotein by plasmapheresis in familial hypercholesterolemia. Arteriosclerosis 5: 613–622.

    Article  PubMed  CAS  Google Scholar 

  8. Yamamoto A, Yokoyama S, Kojima S, Kawaguchi A, Bosch T (1990) Comparison of double membrane filtration and affinity chromatographic techniques. In:Lock G (ed) Apheresis, Wiley-Liss, New York, pp 613–622.

    Google Scholar 

  9. Yokoyama S, Hayashi R, Kikkawa T, Tani N, Takada S, Hatanaka K, Yamamoto A (1984) Specific sorbent of apolipoprotein B-containing lipoproteins for plasmapheresis (LA-01); characterization and experimental use of hypercholesterolemia rabbits. Arteriosclosis 4: 276–282.

    Article  CAS  Google Scholar 

  10. Takada S, Tani N, Ohnishi M, Kau A, Narisada U (1988) Automated continuus low density lipoprotein absorption system. In: Oda T (ed) Therapeutic Plasmapheresis VII, ISAO Press, Cleveland, pp 454–459.

    Google Scholar 

  11. Daida H, Young JL, Yokoi H, Kanoh T, Ishiwata S, Nishikawa H, Takasu F, Kato H, Kusumi Y (1994) Prevention of restenosis after percutaneous transuminal coronary angioplasty by reducing lipoprotein (a) levels with low-density lipoprotein apheresis. Am J Cardiol 73: 1037–1040.

    Article  PubMed  CAS  Google Scholar 

  12. Armstrong VW, Windisch M, Wieland H, Fuchs C, Rieger J, Kostering H, Nebendahl K, Scheler F, Seidel D (1983) Selective continuous elimination of low-density lipoproteins with heparin at acidic pH. Trans Am Soc Artif Intern Organs 29: 323–327.

    PubMed  CAS  Google Scholar 

  13. Bosch T, Schmidt B, Blumenstein M, Gurland HJ (1993) Lipid apheresis by hemoperfusion: in vitro efficacy and ex vivo biocompatibility of a new low-density lipoprotein adsorber compatible with human whole blood. Artif Organs 17: 640–652.

    Article  PubMed  CAS  Google Scholar 

  14. Yamamoto A, Kawaguchi A, Harada-Shiba M, Tsushima M, Kojima S (1997) Apheresis technology for prevention and regression of atherosclerosis: An overview. Therapeutic Apheresis 1: 233–241.

    Article  PubMed  CAS  Google Scholar 

  15. Kawaguchi A, Miyatake K, Yutani C, Beppu S, Tsushima M, Yamamura T, Yamamoto A (1998) Hypercholesterolemic valvulopathy: Characteristic distribution of premature atherosclerosis in homozygous and heterozygous familial hypercholesterolemia. Am Heart J (in press).

    Google Scholar 

  16. Koga N (1997) The retardation of progression, stabilization, and regression of coronary and carotid atherosclerosis by low-density lipoprotein apheresis in patients with familial hypercholesterolemia. Therapeutic Apheresis 1: 260–270.

    Article  PubMed  CAS  Google Scholar 

  17. Keller C, Schmitz H, Theisen K, Zollner N (1986) Regression of valvular aortic stenosis due to homozygous familial hypercholesterolemia following plasmapheresis. Klin Wschr 64: 3338–3341.

    Article  Google Scholar 

  18. Yokoyama S, Yamamoto A, Hayashi R, Satani M (1987) LDL-apheresis: Potential procedure for prevention and regression of atheromatous vascular lesion. Jpn Circul J 51: 1116–1122.

    Article  CAS  Google Scholar 

  19. Hennerici M, Kleophas W, Gries FA (1991) Regression of carotid plaques during low density lipoprotein cholesterol elimination. Stroke 22: 989–992.

    Article  PubMed  CAS  Google Scholar 

  20. Borberg H (1997) Fifteen years experience with LDL-apheresis. In: Yamamoto A (ed) Therapeutic Plasmapheresis XVI Jpn Soc Apheresis, Tokyo, pp 56–62.

    Google Scholar 

  21. Thompson GR, Mahler VMG, Mattews S, Kitano Y, Neuwirth C, Shortt MB, Davies G, Rees A, Mir A, Prescott RJ, deFeyter P, Henderson A (1995) Familial hypercholesterolemia regression study: A randomized trial of low-density-lipoprotein apheresis. Lancet 345: 811–816.

    Article  PubMed  CAS  Google Scholar 

  22. Kroon AA, Aengevaeren WRM, van der Werf T, Uijen GJH, Reiber HC, Bruschke AVG, Stalenhoef AFH (1996) LDL-apheresis atherosclerosis regression study (LAARS). Effect of aggressive versus conventional lipid lowering treatment on coronary atherosclerosis. Circulation 93: 1826–1835.

    PubMed  CAS  Google Scholar 

  23. Harada-Shiba M, Yamamura T, Toyota Y, Tsushima M, Kojima S, Yamamoto A (1996) Rebound curve following LDL-apheresis reflects catabolic rate of plasma cholesterol and the synthetic rate of Lp(a). In Gotto AM Jr, Paoletti R, Smith LC, Catapano AL, Jackson AS (eds), Drugs Affecting Lipid Metabolism. Risk Factors and Future Direction,Kluwer, Dordrecht, pp 591–597.

    Chapter  Google Scholar 

  24. Pfohl M, Naoumova RP, Klass C, Knisel W, Jakober B, Risler R, Thompson GR (1994) Acute and chronic effects on cholesterol biosynthesis of LDL-apheresis with or without concomittant HMG-CoA reductase inhibitor. J Lipid Res 35: 1946–1955.

    PubMed  CAS  Google Scholar 

  25. Nawrocki JW, Weiss SR, Davidson MH, Sprecher DL, Schwartz SL, Lupien PJ, Jones PH, Harber HE, Black DM (1995) Reduction of LDL-cholesterol by 25% to 60% in patients with primary hypercholesterolemia by atorvastatin, new HMG-CoA reductase inhibitor. Arterioscl Thromb Vascul Biol 15: 678–682.

    Article  CAS  Google Scholar 

  26. Bisgaier CL, Essenburg AD, Auerbach BJ, Pape ME, Sekerke CS, Gee A, Woelle S, Newton RS (1997) Attenuation of plasma low density lipoprotein cholesterol by select 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors in mice devoid of low density lipoprotein receptors. J Lipid Res 38: 2502–2515.

    PubMed  CAS  Google Scholar 

  27. Marais AD, Naoumova RP, Firth JC, Penny C, Newwirth CKY, Thompson GR (1997) Decreased production of low density lipoprotein by atorvastatin after apheresis in homozygous familial hypercholesterolemia. J Lipid Res 38: 2071–2078.

    PubMed  CAS  Google Scholar 

  28. Yamamoto A for the Japan Atorvastatin/ LDL-apheresis group (1998) The effect of atorvastatin, a new HMG-CoA reductase inhibitor, and LDL-apheresis on homozygous familial hypercholesterolemia. XIII Int Symp on Drugs Affecting Lipid Metabolism (1998) Florence, May 30-June 3, Poster No 137.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. National Cardiovascular Center Research Institute, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan

    Akira Yamamoto, Akito Kawaguchi & Mariko Harada-Shiba

Authors
  1. Akira Yamamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Akito Kawaguchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Mariko Harada-Shiba
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Geriatric Medicine Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan

    Toru Kita M.D. (Professor) & Masayuki Yokode M.D. (Assistant Professor) (Professor) &  (Assistant Professor)

Rights and permissions

Reprints and permissions

Copyright information

© 2000 Springer-Verlag Tokyo

About this paper

Cite this paper

Yamamoto, A., Kawaguchi, A., Harada-Shiba, M. (2000). Long-term Effect of LDL-Apheresis on Familial Hypercholesterolemia Homozygotes. In: Kita, T., Yokode, M. (eds) Lipoprotein Metabolism and Atherogenesis. Springer, Tokyo. https://doi.org/10.1007/978-4-431-68424-4_3

Download citation

  • DOI: https://doi.org/10.1007/978-4-431-68424-4_3

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-68426-8

  • Online ISBN: 978-4-431-68424-4

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation