Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results.
Author
Waugh, William H.; Daeschner, Charles W. III; Files, Beatrice A.; McConnell, Michael E.; Strandjord, Sarah E.
Abstract
L-Arginine may be a conditionally essential amino acid in children and adolescents with sickle cell disease, particularly as required substrate in the arginine-nitric oxide pathway for endogenous nitrovasodilation and vasoprotection. Vasoprotection by arginine is mediated partly by nitric oxide-induced inhibition of endothelial damage and inhibition of adhesion and activation of leukocytes. Activated leukocytes may trigger many of the complications, including vasoocclusive events and intimal hyperplasias. High blood leukocyte counts during steady states in the absence of infection are significant laboratory risk factors for adverse complications. L-Citrulline as precursor amino acid was given orally twice daily in daily doses of approximately 0.1 g/kg in a pilot Phase 11 clinical trial during steady states in four homozygous sickle cell disease subjects and one sickle cell-hemoglobin C disease patient (ages 10-1 8). There soon resulted dramatic improvements in symptoms of well-being, raised plasma arginine levels, and reductions in high total leukocyte and high segmented neutrophil counts toward or to within normal limits. Continued L-citrulline supplementation in compliant subjects continued to lessen symptomatology, to maintain plasma arginine concentrations greater than control levels, and to maintain nearly normal total leukocyte and neutrophil counts. Side effects or toxicity from citrulline were not experienced. Oral L-citrulline may portend very useful for palliative therapy in sickle cell disease. Placebo-controlled, long-term trials are now indicated. Originally published Journal of the National Medical Association, Vol. 93, No. 10, Oct 2001
Subject
Date
2001-10
Citation:
APA:
Waugh, William H., & Daeschner, Charles W. III, & Files, Beatrice A., & McConnell, Michael E., & Strandjord, Sarah E.. (October 2001).
Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results..
Journal of the National Medical Association,
93(10),
363-
371. Retrieved from
http://hdl.handle.net/10342/3137
MLA:
Waugh, William H., and Daeschner, Charles W. III, and Files, Beatrice A., and McConnell, Michael E., and Strandjord, Sarah E..
"Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results.". Journal of the National Medical Association.
93:10. (363-371),
October 2001.
April 24, 2024.
http://hdl.handle.net/10342/3137.
Chicago:
Waugh, William H. and Daeschner, Charles W. III and Files, Beatrice A. and McConnell, Michael E. and Strandjord, Sarah E.,
"Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results.," Journal of the National Medical Association 93, no.
10 (October 2001),
http://hdl.handle.net/10342/3137 (accessed
April 24, 2024).
AMA:
Waugh, William H., Daeschner, Charles W. III, Files, Beatrice A., McConnell, Michael E., Strandjord, Sarah E..
Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results.. Journal of the National Medical Association.
October 2001;
93(10):
363-371.
http://hdl.handle.net/10342/3137. Accessed
April 24, 2024.
Collections
Publisher
East Carolina University