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Patterns of Arginine and Nitric Oxide in Patients With Sickle Cell Disease With Vaso-occlusive Crisis and Acute Chest Syndrome

Morris, Claudia R. M.D.; Kuypers, Frans A. Ph.D.; Larkin, Sandra B.S.; Vichinsky, Elliott P. M.D.; Styles, Lori A. M.D.

Journal of Pediatric Hematology/Oncology: November-December 2000 - Volume 22 - Issue 6 - p 515-520
Original Articles

Purpose Our objective was to evaluate l-arginine and nitric oxide metabolite (NOx) levels in children with sickle cell disease (SCD) at steady-state and during vaso-occlusive crisis (VOC). Because alterations in nitric oxide production may have an important role in the pathophysiology of SCD, our second aim was to determine if a relationship exists between these levels and vaso-occlusive crisis (VOC).

Patients and Methods Plasma l-arginine and serum NOx levels were examined in 36 patients with SCD with 39 episodes of VOC and 10 children with SCD at steady-state. Daily levels were obtained in children requiring hospitalization.

Results Steady-state l-arginine levels were normal in children with SCD. l-arginine levels were low, however, in children with VOC (37.4 ± 2.7 vs. 53.6 ± 4.6 μmol/L;P = 0.008) but returned to baseline during hospitalization. In contrast, NOx levels were normal at presentation but decreased during hospitalization for both patients with VOC and patients with acute chest syndrome (ACS) (21.1 ± 2.0, 17.4 ± 2.4, and 12.3 ± 1.6 μmol/L, respectively;P < 0.05). In the patients with VOC who had ACS develop, l-arginine decreased to the lowest levels at the time of the ACS diagnosis, correlating with decreasing NOx levels.

Conclusion These data suggest that there may be a relationship between the l-arginine–nitric oxide pathway and vaso-occlusion in SCD. Low arginine levels during VOC could reflect a state of acute substrate depletion that results in a decrease in nitric oxide production.

From the Department of Emergency Medicine (C.R.M.), Children's Hospital Oakland, Oakland, California, U.S.A.; Children's Hospital Oakland Research Institute (F.A.K., S.L.), Oakland, California, U.S.A.; Department of Hematology/Oncology (E.P.V., L.A.S.), Children's Hospital Oakland, Oakland, California, U.S.A.

Submitted for publication on October 22, 1999; accepted February 3, 2000.

Supported in part by the National Institute of Health grant M01 RR01271-19, HL-20985.

Address correspondence and reprint requests to Claudia R. Morris, M.D., Department of Emergency Medicine, Children's Hospital Oakland, 747 52nd Street, Oakland, CA 94609. E-mail:

© 2000 Lippincott Williams & Wilkins, Inc.