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  • Title: Rapid and accurate quantitation of Hb Bart's and Hb H using weak cation exchange high performance liquid chromatography: correlation with the alpha-thalassemia genotype.
    Author: Papassotiriou I, Traeger-Synodinos J, Vlachou C, Karagiorga M, Metaxotou A, Kanavakis E, Stamoulakatou A.
    Journal: Hemoglobin; 1999 Aug; 23(3):203-11. PubMed ID: 10490132.
    Abstract:
    The Bio-Rad Variant Hemoglobin testing system is an automated high performance liquid chromatography analyzer marketed with a beta-thalassemia short program to quantify Hbs F and A2, and assist in detecting Hbs A, S, D, C, and E. Although the two hemoglobins present in Hb H disease, Hb Bart's and Hb H, are separated by the system, they are not quantitated. In this study we modified the beta-thalassemia short program in order to facilitate quantitation of Hb Bart's and Hb H. Blood samples from 60 patients with Hb H disease, with various underlying genotypes, were studied. Analyses were performed on the day of blood collection or on hemolysates stored at -80 degrees C in cyanide or carbomonoxy forms. The mean sum of Hb Bart's and Hb H levels in all patients was found to be 12% (range 1.8-35%). Patients with nondeletional mutations (or association of alpha(0) deletion and nondeletional mutations) had notably higher Hb Bart's and Hb H levels when compared to patients with deletional genotypes.
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