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The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability.

Abstract

To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity.

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  • Authors+Show Affiliations

    ,

    Department of Pediatrics, University of California, San Francisco.

    , , , , ,

    Source

    Blood 72:6 1988 Dec pg 2056-9

    MeSH

    Anemia, Sickle Cell
    Body Water
    Cell Adhesion
    Erythrocyte Deformability
    Erythrocytes
    Hemoglobins
    Humans
    Pain

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    3196878

    Citation

    Lande, W M., et al. "The Incidence of Painful Crisis in Homozygous Sickle Cell Disease: Correlation With Red Cell Deformability." Blood, vol. 72, no. 6, 1988, pp. 2056-9.
    Lande WM, Andrews DL, Clark MR, et al. The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability. Blood. 1988;72(6):2056-9.
    Lande, W. M., Andrews, D. L., Clark, M. R., Braham, N. V., Black, D. M., Embury, S. H., & Mentzer, W. C. (1988). The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability. Blood, 72(6), pp. 2056-9.
    Lande WM, et al. The Incidence of Painful Crisis in Homozygous Sickle Cell Disease: Correlation With Red Cell Deformability. Blood. 1988;72(6):2056-9. PubMed PMID: 3196878.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability. AU - Lande,W M, AU - Andrews,D L, AU - Clark,M R, AU - Braham,N V, AU - Black,D M, AU - Embury,S H, AU - Mentzer,W C, PY - 1988/12/1/pubmed PY - 1988/12/1/medline PY - 1988/12/1/entrez SP - 2056 EP - 9 JF - Blood JO - Blood VL - 72 IS - 6 N2 - To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity. SN - 0006-4971 UR - https://www.unboundmedicine.com/medline/citation/3196878/The_incidence_of_painful_crisis_in_homozygous_sickle_cell_disease:_correlation_with_red_cell_deformability_ L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=3196878 DB - PRIME DP - Unbound Medicine ER -