[原著］Molecular Characterization and the Severity of Hemoglobin H Disease in Northern Thailand

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We investigated the molecular basis for Hb H disease in 41 Thai patients in Northern Thailand by restriction endonuclease analysis with alpha and zeta specific globin probes. The result showed that 36 cases (88%) were due to a combination of thalassemia 1, Southeast Asia type and non-deletion defect type. Five cases (12%) were of a deletion type with 3.7 kb deletion on one globin gene (rightward type). We did not find any leftward type (4.2kb) among these patients. In clinical exanimations, the patients with the non-deletion defect type of Hb H disease were more severe than the patients with deletion type.

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