Download PDF
Thromb Haemost 1971; 26(01): 015-021
DOI: 10.1055/s-0038-1653651
Originalarbeiten – Original Articles – Travaux Originaux
Schattauer GmbH

An Intrinsic Blood Platelet Abnormality in an Homocystinuric Boy, Corrected by Pyridoxine Administration

A. J Zweifler M. D.
1   The Department of Internal Medicine and Section of Pediatric Neurology, Department of Pediatrics University of Michigan Medical Center Ann Arbor, Michigan 48104
,
R. J Allen M. D.
1   The Department of Internal Medicine and Section of Pediatric Neurology, Department of Pediatrics University of Michigan Medical Center Ann Arbor, Michigan 48104
› Author Affiliations
This work was supported by a Training Grant from the Children’s Bureau, Department of Health Education and Welfare and by a Grant No. 2M01-FR-42 from the Clinical Research Centers Branch, Division of Research Resources, National Institutes of Health, and by a grant from the Michigan Heart Association.
Further Information

Publication History

Publication Date:
24 July 2018 (online)

Also available at
   Permissions and Reprints

Summary

Platelet aggregation in a boy with homocystinuria (HCU) was compared to that found in normal subjects. Response to adenosine diphosphate (ADP) was diminished while aggregation with collagen and adrenalin was normal. The impaired response to ADP was not corrected by resuspension of HCU platelets in normal plasma or by a period of therapy with a low protein diet, but it was enhanced promptly with pyridoxine 750 mg/day. The increase of platelet sensitivity to ADP during B6 administration could not be correlated with plasma concentrations of either homocystine or methionine.

 

  • References

  • 1 Barber G. W, Spaeth G. L. Pyridoxine therapy in homocystinuria. Lancet I: 337 1967;
    PubMedSearch in Google Scholar
  • 2 Cusworth D. G, Dent G. E. Homocystinuria. Brit. med. Bull 25: 42 1969;
    CrossrefPubMedSearch in Google Scholar
  • 3 McDonald L, Bray G, Field G, Love F, Davies B. Homocystinuria, thrombosis, and the blood-platelets. Lancet I: 745 1964;
    PubMedSearch in Google Scholar
  • 4 Moore S, Spackman D. H, Stein W. H. Chromatography of amino acids on sulfonated polystyrene resins - an improved system. Anal. Chem 30: 1185 1958;
    CrossrefPubMedSearch in Google Scholar
  • 5 Mudd S. H, Finkelstein J. D, Irreverre F, Laster L. Homocystinuria: An enzymatic defect. Science 243: 1443 1964;
    PubMedSearch in Google Scholar
  • 6 Mustard J. F, Hegardt B, Rowsell H. G, MacMillan R. L. Effect of adenosine nucleotides on platelet aggregation and clotting time. J. Lab. clin. Med 64: 548 1964;
    PubMedSearch in Google Scholar
  • 7 O’Brien J. R, Heywood J. B, Heady J. A. The quantitation of platelet aggregation induced by four compounds: A study in relation to myocardial infarction. Thrombos. Diathes. haemorrh. (Stuttg) 16: 752 1966;
    PubMedSearch in Google Scholar
  • 8 Schimke R. N, McKusick V. A, Huang T, Pollack A. D. Homocystinuria. Studies of 20 families with 38 affected members. JAMA 193: 711 1965;
    CrossrefPubMedSearch in Google Scholar
  • 9 Spaet T. H, Zucker M. B. Mechanism of platelet plug formation and role of adenosine diphosphate. Amer. J. Physiol 206: 1267 1964;
    PubMedSearch in Google Scholar
  • 10 Thomas D. P, Niewiarowski S, Ream V. J. Release of adenine nucleotides and platelet factor 4 from platelets of man and four other species. J. Lab. clin. Med 75: 607 1970;
    PubMedSearch in Google Scholar
  • 11 Zweifler A. J. Impairment of platelet function and thrombus growth in reserpine-treated rabbits. J. Lab. clin. Med 70: 16 1967;
    PubMedSearch in Google Scholar
  • 12 Zweifler A, Allen R. J. Abnormal platelet aggregation in patients with homocystinuria. Circulation 40 Suppl.III: 27 1969;
    PubMedSearch in Google Scholar