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Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome.
J Pediatr Hematol Oncol 2000 Nov-Dec; 22(6):515-20JP

Abstract

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 micromol/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 micromol/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.

Authors+Show Affiliations

Department of Emergency Medicine, Children's Hospital Oakland, California 94609, USA. johnclaud@home.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

11132219

Citation

Morris, C R., et al. "Patterns of Arginine and Nitric Oxide in Patients With Sickle Cell Disease With Vaso-occlusive Crisis and Acute Chest Syndrome." Journal of Pediatric Hematology/oncology, vol. 22, no. 6, 2000, pp. 515-20.
Morris CR, Kuypers FA, Larkin S, et al. Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome. J Pediatr Hematol Oncol. 2000;22(6):515-20.
Morris, C. R., Kuypers, F. A., Larkin, S., Vichinsky, E. P., & Styles, L. A. (2000). Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome. Journal of Pediatric Hematology/oncology, 22(6), pp. 515-20.
Morris CR, et al. Patterns of Arginine and Nitric Oxide in Patients With Sickle Cell Disease With Vaso-occlusive Crisis and Acute Chest Syndrome. J Pediatr Hematol Oncol. 2000;22(6):515-20. PubMed PMID: 11132219.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome. AU - Morris,C R, AU - Kuypers,F A, AU - Larkin,S, AU - Vichinsky,E P, AU - Styles,L A, PY - 2000/12/29/pubmed PY - 2001/2/28/medline PY - 2000/12/29/entrez SP - 515 EP - 20 JF - Journal of pediatric hematology/oncology JO - J. Pediatr. Hematol. Oncol. VL - 22 IS - 6 N2 - 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 micromol/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 micromol/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. SN - 1077-4114 UR - https://www.unboundmedicine.com/medline/citation/11132219/Patterns_of_arginine_and_nitric_oxide_in_patients_with_sickle_cell_disease_with_vaso_occlusive_crisis_and_acute_chest_syndrome_ L2 - http://Insights.ovid.com/pubmed?pmid=11132219 DB - PRIME DP - Unbound Medicine ER -