Annals of Hematology

, Volume 83, Issue 6, pp 371–375

Evidence for a metabolic shift of arginine metabolism in sickle cell disease

  • John-John B. Schnog
  • Esther H. Jager
  • Fey P. L. van der Dijs
  • Ashley J. Duits
  • Han Moshage
  • Fred D. Muskiet
  • Frits A. J. Muskiet
Original Article

DOI: 10.1007/s00277-004-0856-9

Cite this article as:
Schnog, JJ.B., Jager, E.H., van der Dijs, F.P.L. et al. Ann Hematol (2004) 83: 371. doi:10.1007/s00277-004-0856-9
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Abstract

Over the last few years, a pivotal role has been ascribed to reduced nitric oxide (NO) availability as a contributing factor to the vaso-occlusive process of sickle cell disease. We investigated whether arginine metabolism in sickle cell patients is different from healthy controls. Blood samples were drawn by venipuncture in the fasting state from 8 clinically asymptomatic HbSS patients and 14 race-matched HbAA controls. HbSS patients had decreased plasma arginine (p=0.001) and increased proline (p=0.015) levels as compared to controls. Ratios of arginine to ornithine (p<0.001), proline (p<0.001), glutamate (p=0.003), and citrulline (p=0.026) were lower in HbSS patients. There were significant correlations of ornithine (rs=−0.71, p=0.047), citrulline (rs=−0.79, p=0.021), arginine/ornithine (rs=0.93, p=0.001), and arginine/citrulline (rs=0.81, p=0.015) to hemoglobin and of arginine/proline (rs=−0.76, p=0.028) and citrulline (rs=0.71, p=0.048) to leukocyte counts. These data indicate that in clinically asymptomatic sickle cell patients increased arginine metabolism is shifted to the arginase pathway and that this seems to be more profound in patients with higher hemolytic rates and leukocyte counts.

Keywords

Sickle cell disease Arginine Nitric oxide Hemolysis Endothelium 

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • John-John B. Schnog
    • 1
    • 2
  • Esther H. Jager
    • 3
    • 4
  • Fey P. L. van der Dijs
    • 4
  • Ashley J. Duits
    • 2
  • Han Moshage
    • 5
  • Fred D. Muskiet
    • 6
  • Frits A. J. Muskiet
    • 3
  1. 1.Department of Internal Medicine (9B)Slotervaart HospitalAmsterdamThe Netherlands
  2. 2.Red Cross Blood Bank FoundationCuraçaoNetherlands Antilles
  3. 3.Department of Pathology and Laboratory MedicineGroningen University HospitalThe Netherlands
  4. 4.Analytical Diagnostic CenterCuraçaoNetherlands Antilles
  5. 5.Division of Gastroenterology and HepatologyGroningen University HospitalThe Netherlands
  6. 6.Department of PediatricsSt. Elisabeth HospitalCuraçaoNetherlands Antilles

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