Skip to main content
SpringerLink
Log in

Homocystinuria: Kinetics and Distribution of 111In-Labelled Platelets

  • Chapter
Blood cells in nuclear medicine, part I

Part of the book series: Developments in Nuclear Medicine ((DNUM,volume 6))

  • 64 Accesses

Abstract

The pathogenesis of thromboembolism, a major cause of morbidity and mortality, in patients with homocystinuria due to cystathionine beta-synthase deficiency remains unclear. Two previous studies of platelet survival using the 51Cr label have produced conflicting results. In this study, we have investigated the kinetics and biodistribution of 111In-oxine labelled platelets in 11 normal volunteers and 12 homocystinuric patients, none of whom had clinical evidence of acute thrombosis at the time of the study. Six of the patients were pyridoxine resistant and had homocystinemia. There were no statistical differences in mean platelet survival times between pyridoxine responders and non-responders, and pyridoxine responders or non-responders and normals, regardless of whether a linear, exponential, weighted mean, or multiple-hit model was used to analyze the kinetic data. By the multiple-hit estimate model, which best fitted the kinetic data, the mean platelet survival time (MPS)for homocystinuric patients was 7.92 days (SEM = 0.30 days) compared with 8.40 days (SEM = 0.25 days) for the normals (p>0.1). Plasma homocystine levels had no apparent effect on mean platelet survival time.

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 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

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. Carson NAJ, Cusworth DC, Dent CE, Field CMB, Neill DW, Homocystinuria: A new inborn error of metabolism of methionine associated with mental deficiency. Arch. Dis. Child. 38: 425, 1962.

    Article  Google Scholar 

  2. Carson NAJ, Neill DW, Metabolic abnormalities detected in a survey of mentally backward individuals in Northern Ireland. Arch. Dis. Child. 37: 505, 1962.

    Article  PubMed  CAS  Google Scholar 

  3. Mudd SH, Finkestein JD, Irreverre F, Laster L, Homocystinuria: An enzymatic defect. Science 143: 1443, 1964.

    Article  PubMed  CAS  Google Scholar 

  4. Spaeth GL, Barber GW, Homocystinuria in a mentally retarded child and her normal cousin. Trans. Amer. Acad. Ophthalmol. Otolaryngologia 69: 912, 1965.

    CAS  Google Scholar 

  5. Schimke RN, McKusick VA, Hard T, Pollack AD, Homocystinuria: Studies of 20 families with 38 affected members. Jama 193: 711, 1965.

    PubMed  CAS  Google Scholar 

  6. McKusick VA, Homocystinuria. In: Heritable disorders of connective tissue. 4th ed. Mosby CV, St. Louis, pp 224–281, 1972.

    Google Scholar 

  7. Grieco AJ, Homocystinuria: Pathogenetic mechanisms. Amer. J. Med. Sci. 273: 120, 1977.

    Article  PubMed  CAS  Google Scholar 

  8. Mudd SH, Levy HL, Disorders of transsulfuration. In: The metabolic basis of inherited diseases. Stanbury JB, Wyngaarden JB, Fredrickson DS (eds), McGraw-Hill, New York, pp 458–503, 1978.

    Google Scholar 

  9. Valle D, Shashidar Pai G, Thomas GH, Pyeritz RE, Homocystinuria due to cystathionine beta-synthase deficiency: Clinical manifestations and therapy. Johns Hopk. Med. J. 146: 110, 1980.

    CAS  Google Scholar 

  10. Barber GW, Spaeth GL, The successful treatment of hcnocystinuria with pyridoxine. J. Pediat. 75–463, 1969.

    Google Scholar 

  11. Harker LA, Slichter SJ, Scott CR, Ross R, Homocystinuria: Vascular injury and arterial thrombosis. New Engl. J. Med. 291: 537, 1974.

    Article  PubMed  CAS  Google Scholar 

  12. Harker LA, Scott CR, Platelets in homocystinuria. New Engl. J. Med. 296: 818, 1977.

    PubMed  CAS  Google Scholar 

  13. Uhlemann ER, Tenpas JH, Lucky AW, Schulman JD, Mudd SH, Shulman NR, Platelet survival and morphology in homocystinuria due to cystationine synthase deficiency. New Engl. J. Med. 295: 1283, 1976.

    Article  PubMed  CAS  Google Scholar 

  14. Scheffel U, McIntyre PA, Evatt B, Dvornicky JA, Natarajan TK, Bolling DR, Murphy EA, Evaluation of Indium-111 as a new high photon yield gamma-emitting “physiological” platelet label. Johns Hopk. Med. J. 140: 285, 1977.

    CAS  Google Scholar 

  15. Scheffel U, Tsan MF, McIntyre PA, Labelling of human platelets with (111In) 8-hydroxyquinoline. J. nucl. Med. 20: 524, 1979.

    PubMed  CAS  Google Scholar 

  16. Scheffel U, Tsan MF, Mitchell TG, Camargo EE, Braine H, Ezekowitz MD, Nickoloff EL, Hill-Zobel RL, Murphy EA, McIntyre PA, 1111ndiumlabeled platelets in normal man. Kinetics, distribution and radiation dose estimations. J. nucl. Med. 23: 149, 1982.

    PubMed  CAS  Google Scholar 

  17. Hill-Zobel RL, Pyeritz RE, Scheffel N, Malpica O, Engin S, Camargo EE, Abbott M, Guilarte TR, Hill J, McIntyre PA, Murphy EA, Tsan MF, Kinetics and distribution of 111In-labeled platelets in patient with homocystinuria. New Engl. J. Med. 307: 781, 1982.

    Article  PubMed  CAS  Google Scholar 

  18. Uhlendorf BW, Conerly EB, Mudd SH, Honocystinuria: Studies in tissue culture. Pediat. Res. 7: 645, 1973.

    Article  PubMed  CAS  Google Scholar 

  19. Poole JR, Mudd SH, Conerly EB, Edward S, Homocystinuria due to cystathionine synthase deficiency. Studies of nitrogen balance and sulfur excretion. J. clin. Invest. 55: 1033, 1975.

    Article  PubMed  CAS  Google Scholar 

  20. Murphy EA, Francis ME, The estimation of blood platelet survival. II. The multiple-hit model. Thrombos. Diathes. haemorrh. (Stuttg.) 25: 53, 1971.

    CAS  Google Scholar 

  21. The Panel on Diagnostic Application of Radioisotopes in Haematology. International Committee for Standardization in Haematology. Recommended methods for radioisotope platelet survival studies. Blood 50: 1137, 1977.

    Google Scholar 

  22. Dixon WJ, Massey FJ, Introduction to statistical analysis. 2nd ed. McGraw Hill, New York, p 250, 1957.

    Google Scholar 

  23. Wright RR, Tono M, Pollycove M, Blood volume. Sem. nucl. Med. 5: 63, 1975.

    Article  CAS  Google Scholar 

  24. Nickoloff EL, The physics of left ventricular performance measurements with radioactive tracers. Ph.D. Thesis, Johns Hopk. University 1977.

    Google Scholar 

  25. Hill-Zobel RL, Scheffel U, McIntyre PA, Tsan MF, 111In-oxine labelled rabbit platelets: In vivo distribution and sites of destruction. Blood (in press).

    Google Scholar 

  26. Spackman H, Stein WH, Moore S, Automatic recording apparatus for use in the chromatography of amino acids. Anal. Chem. 30: 1190, 1958.

    Article  CAS  Google Scholar 

  27. Guilarte TR, McIntyre PA, Radiometric-microbiological assay of vitamin-B6: Analysis of plasma samples. J. Nutr. 111: 1891, 1981.

    Google Scholar 

  28. Chen NE, McIntyre PA, Wagner HN Jr, A radiometric microbiological method for vitamin B12 assay. J. nucl. Med. 18: 388, 1977.

    PubMed  CAS  Google Scholar 

  29. Chen ME, McIntyre PA, Kertcher JA, Measurement of folates in human plasma and erythrocytes by a radiometric microbiological method. J. nucl. Med. 19: 906, 1978.

    PubMed  CAS  Google Scholar 

  30. Fowler B, Kraus J, Packman S, Rosenburg LE, Homocystinuria. Evidence for 3 distinct classes of cystathionine beta-synthase mutants in cultured fibroblasts. J. clin.,Invest. 61–645, 1978.

    Google Scholar 

  31. Skovby F, Kraus J, Relich C, Rosenburg LE, Ingnunochemical studies on cultured fibroblasts from patients with homocystinuria due to cystathionine beta-synthase deficiency. Amer. J. hum. Genet. 34: 73, 1982.

    PubMed  CAS  Google Scholar 

  32. Hilden M, Brandt NJ, Nilsson IM, Schonheyder F, Investigations of coagulation and fibrinolysis in honocystinuria. Acta Med. Scand. 195: 533, 1974.

    Article  PubMed  CAS  Google Scholar 

  33. Bolling DR, Murphy EA, The estimation of blood platelet survival. VI. Evaluation of the graphical method. Johns Hopk. Med. J. 143: 25, 1978.

    CAS  Google Scholar 

Download references

Authors
  1. R. L. Hill-Zobel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. R. E. Pyeritz
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. U. Scheffel
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. O. Malpica
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Engin
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. E. E. Camargo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. M. Abbott
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. T. R. Guilarte
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. J. Hill
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. P. A. McIntyre
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. E. A. Murphy
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Min-Fu Tsan
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Internal Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands

    Max R. Hardeman PhD

  2. Department of Nuclear Medicine, Hôpital Saint Louis, Paris, France

    Yves Najean MD

Rights and permissions

Reprints and permissions

Copyright information

© 1984 Martinus Nijhoff Publishers, The Hague

About this chapter

Cite this chapter

Hill-Zobel, R.L. et al. (1984). Homocystinuria: Kinetics and Distribution of 111In-Labelled Platelets. In: Hardeman, M.R., Najean, Y. (eds) Blood cells in nuclear medicine, part I. Developments in Nuclear Medicine, vol 6. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-6027-5_20

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-6027-5_20

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-009-6029-9

  • Online ISBN: 978-94-009-6027-5

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation