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Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome.

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.

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  • Authors+Show Affiliations

    ,

    Department of Emergency Medicine, Children's Hospital Oakland, California 94609, USA. johnclaud@home.com

    , , ,

    Source

    MeSH

    Acute Disease
    Adolescent
    Adult
    Anemia, Sickle Cell
    Arginine
    Biomarkers
    Blood Transfusion
    Child
    Child, Preschool
    Female
    Humans
    Infarction
    Male
    Nitric Oxide
    Pain
    Syndrome
    Thorax

    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 -