Skip to main content
SpringerLink
Log in

Evaluation of the classical methods for the diagnosis of type III hyperlipoproteinemia

Vergleichende Untersuchung der klassischen Methoden zur Diagnose der Typ III-Hyperlipoproteinämie

  • Originalien
  • Published:
Klinische Wochenschrift Aims and scope Submit manuscript

Zusammenfassung

Die familiäre Hyperlipoproteinämie Typ III ist gekennzeichnet durch 1) einen erhöhten Plasmaspiegel von Lipoproteinen sehr niederer Dichte (Verylowdensitylipoproteins, VLDL), welche ungewöhnlich cholesterinreich sind, und 2) durch Plasmalipoproteine mit einer Dichte zwischen der von VLLL und Lipoproteinen niederer Dichte (Lowdensitylipoproteins, LDL); diese Lipoproteine mit „Zwischendichte“ (Intermediatedensitylipoproteins, IDL oder Lp III) wandern elektrophoretisch langsamer als VLDL und werden als Verunreinigung in den in der Ultrazentrifuge flotierenden VLDL (Dichte <1006) gefunden. Allgemein wird die Diagnose der Hyperlipoproteinämie Typ III durch den Nachweis von Lipoproteinen gestellt, welche mit VLDL flotieren, aber elektrophoretisch mit beta-Globulinen wandern (“floating beta-lipoprotein”). Neuerlich wurde ein erhöhtes Verhältnis von VLDL-Cholesterin zu Plasmatriglyzeriden höher als 0,30 als diagnostisch für die Erkrankung beschrieben. Wir haben die beiden Methoden zur Diagnosestellung verglichen und kamen zu dem Schluß, daß die chemische Indexdefinition in der Diagnosestellung der Hyperlipoproteinämie Typ III verläßlicher ist.

Summary

Familial type III hyperlipoproteinemia is characterized by the presence of elevated plasma levels of very low density lipoproteins (VLDL) which contain an increased amount of cholesterol and by the presence of a significant amount of lipoproteins with an intermediate density between that of VLDL and low density lipoproteins (LDL); the intermediate density lipoproteins, designated IDL or Lp III, have a slower electrophoretic migration rate than VLDL, and are found in the ultracentrifugal top fraction as a contaminant. Classically, the diagnosis of type III is based on the demonstration of beta-migrating lipoproteins in the ultracentrifugal top fraction (density <1.006), thus “floating beta-lipoprotein”. More recently, it has been proposed that an elevated VLDL-cholesterol to triglyceride ratio is diagnostic of the disorder. In the present report, we have compared the two methods for their diagnostic value and have concluded that the chemical index definition is the more reliable method for the diagnosis of type III hyperlipoproteinemia.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Bartlett, G.R.: Phosphorus assay in column chromatography. J. Biol. Chem.234, 466–468 (1959)

    Google Scholar 

  2. Carlson, K., Carlson, L.A.: Composition of the behavior of very low density lipoproteins of type III hyperlipoproteinaemia on electrophoresis on paper and on agarose gel with a note on a late (slow) pre-β VLDL lipoprotein. Scand. J. Clin. Lab. Invest.35, 655–660 (1975)

    Google Scholar 

  3. Fredrickson, D.S., Levy, R.I.: Familial hyperlipoproteinemia, inThe Metabolic Basis of Inherited Disaases 3rd ed., J.B. Stanbury, J.B., Wyngarrden und D.S. Fredrickson, ed., New York, McGraw-Hill, pp. 545–614 (1972)

    Google Scholar 

  4. Fredrickson, D.S., Morganroth, J., Levy, R.I.: Type III hyperlipoproteinemia: an analysis of two contemporary definitions. Ann. Intern. Med.82, 150–157 (1975)

    Google Scholar 

  5. Godolphnin, W.J., Stinson, R.A.: Isoelectrofocusing of human plasma lipoproteins in polyacrylamide gels: diagnosis of type III hyperlipoproteinemia (“broad beta” disease). Clin. Chim. Acta56, 97–103 (1974)

    Google Scholar 

  6. Havel, R.J., Kane, J.P.: Primary dysbetalipoproteinemia: Predominance of a specific apoprotein species in triglyceride-rich lipoproteins. Proc. Natl. Acad. Sci. USA70, 2015–2019 (1973)

    Google Scholar 

  7. Havel, R.J.: Hyperlipoproteinemia: problems in diagnosis and challenges posed by the “type III” disorder. Ann. Intern. Med.82, 273–274 (1975)

    Google Scholar 

  8. Hazzard, W.R., Lindgren, F.T., Bierman, E.L.: Very low density lipoprotein subfractions in a subject with broad-beta disease (type III hyperlipoproteinemia) and a subject with endogenous lipemia (type IV): chemical composition and electrophoretic mobility. Biochim. Biophys. Acta202, 517–525 (1970)

    Google Scholar 

  9. Hazzard, W.R., Porte, D., Bierman, E.L.: Abnormal lipid composition of very low density lipoproteins in diagnosis of broad-beta disease (type III hyperlipoproteinemia). Metabolism21, 1009–1019 (1972)

    Google Scholar 

  10. Hazzard, W.R., Bierman, E.L.: Broad-β disease versus endogenous hypertriglyceridemia: levels and lipid composition of chylomicrons and very low density lipoproteins during fat-free feeding and alimentary lipemia. Metabolism24, 817–828 (1975)

    Google Scholar 

  11. Hazzard, W.R., O'Donnell, T.F., Lee, Y.L.: Borad-β disease (type III hyperlipoproteinemia) in a large kindred: evidence for a monogenic mechanism. Ann. Intern. Med.82, 141–149 (1975)

    Google Scholar 

  12. Hazzard, W.R., Bierman, E.L.: Delayed clearance of chylomicron remnants following vitamin A-containing oral fat loads in broad-β disease (type III hyperlipoproteinemia). Metabolism25, 777–801 (1976)

    Google Scholar 

  13. Lowry, O.H., Rosebrough, N.J., Fan, A.L., Randall, R.J.: Protein measurement with the Folin phenol reagent. J. Biol. Chem.193, 265–275 (1951)

    Google Scholar 

  14. Mahley, R.W., Weisgraber, K.H.: An electrophoretic method for the quantitative isolation of human and swine plasma lipoproteins. Biochemistry13, 1964–1969 (1974)

    Google Scholar 

  15. Manual of Laboratory Methods. Lipid Research Clinics Program, Chapel Hill, N.C., University of North Carolina (1972)

    Google Scholar 

  16. Masket, B.H., Levy, R.I., Fredrickson, D.S.: The use of polyacylamide gel electrophoresis in differentiating type III hyperlipoproteinemia. J. Lab. Clin. Med.81, 794–802 (1973)

    Google Scholar 

  17. Morganroth, J., Levy, R.I., Fredrickson, D.S.: The biochemical, clinical, and genetic features of type III hyperlipoproteinemia. Ann. Intern. Med.82, 158–174 (1975)

    Google Scholar 

  18. Patsch, J.R., Sailer, S., Braunsteiner, H.: Lipoproteins of the density 1.006–1.020 in the plasma of patients with type III hyperlipoproteinaemia in the postabsorptive state. Europ. J. Clin. Invest.5, 45–55 (1975)

    Google Scholar 

  19. Patsch, J.R., Sailer, S., Braunsteiner, H., Forte, T.: Electron microscopic characterization of lipoproteins from patients with familial type III hyperlipoproteinaemia. Europ. J. Clin. Invest.6, 307–310 (1976)

    Google Scholar 

  20. Patsch, J.R.: Type III hyperlipoproteinemia, inLow Density Lipoproteins C.E. Day, ed., New York, Plenum Press, pp. 197–227 (1976)

    Google Scholar 

  21. Quarfordt, S., Levy, R.I., Fredrickson, D.S.: On the lipoprotein abnormality in type III hyperlipoproteinemia. J. clin. Invest.50, 754–761 (1971)

    Google Scholar 

  22. Shepherd, J.: Diagnosis of dyslipoproteinaemaia by molecular sieve chromatography. Clin. Chim. Acta69, 161–173 (1976)

    Google Scholar 

  23. Shore, B., Shore, V.: An apolipoprotein preferentially enriched in cholesteryl ester-rich very low density lipoproteins. Biochem. Biophys. Res. Commun.58, 1–7 (1974)

    Google Scholar 

  24. Utermann, G., Jaeschke, M., Menzel, J.: Familial hyperlipoproteinemia type III: deficiency of a specific apolipoprotein (apoE-III) in the very-low-density lipoproteins. FEBS Letters56, 352–355 (1975)

    Google Scholar 

  25. Vessby, B., Carlson, L.A.: Conversion of type III hyperlipoproteinaemia to type IV hyperlipoproteinaemia by a fat-free, carbohydrate rich diet. Europ. J. clin. Invest.5, 359–364 (1975)

    Google Scholar 

  26. Vessby, B.: Studies on the serum lipoprotein composition in 50 year-old men. A suggestion of chemical criteria for diagnosis of hyperlipoproteinaemia type III (broad-β disease). Clin. Chim. Acta69, 29–42 (1976)

    Google Scholar 

  27. Wieland, H., Seidel, D.: Improved techniques for assessment of serum lipoprotein patterns. II. Rapid method for diagnosis of type III hyperlipoproteinemia without ultracentrifugation. Clin. Chem.19, 1139–1141 (1973)

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dept. of Medicine, Baylor College of Medicine Texas Medical Center, 77030, Houston, Texas, USA

    J. R. Patsch M.D. (Ass. Prof. of Experimental Medicine), R. L. Jackson & A. M. Gotto Jr.

Authors
  1. J. R. Patsch M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. R. L. Jackson
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. M. Gotto Jr.
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Patsch, J.R., Jackson, R.L. & Gotto, A.M. Evaluation of the classical methods for the diagnosis of type III hyperlipoproteinemia. Klin Wochenschr 55, 1025–1030 (1977). https://doi.org/10.1007/BF01489475

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01489475

Schlüsselwörter

Key words

Search

Navigation