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不稳定血红蛋白病(UHb),是由于遗传缺陷所致血红蛋白肽链发生氨基酸替代或丢失,使血红蛋白分子稳定性降低,而引起溶血性贫血的一类少见的血红蛋白病,国外自Scott首次报告以来,已发现80余种UHb病,近年来国内已发现11种,多系个案或一个家系报告,我院自1979～1985年经血液门诊和住院确诊为UHb36例,说明木病并非少见,由于对本病认识不够,尤其Hb电泳无异常者更易忽略,而延误诊治,故将我院36例UHb病整理报告如下:临床资料一、一般资料:男女各18例,年龄3~75岁,<12岁4例,>50岁5例。籍贯四川19例,广东9例,浙江2例,山西6
Unstable hemoglobin disease (UHb) is due to genetic defects due to amino acid substitution or loss of hemoglobin peptide chain, reducing the stability of hemoglobin molecules, and hemolytic anemia caused by a rare class of hemoglobinopathies, since Scott’s first report, More than 80 kinds of UHb disease have been found. In recent years, 11 cases have been found in China, many cases have been reported or one pedigree. In our hospital from 1979 to 1985, 36 cases of UHb were diagnosed by blood clinics and inpatients, indicating that the disease is not uncommon. Disease is not enough understanding, in particular, Hb electrophoresis was more likely to be ignored, but delayed diagnosis and treatment, so the hospital 36 cases of UHb disease finishing report as follows: Clinical data First, the general information: 18 males and 18 females, aged 3 to 75 years old, <12 years old 4 cases,> 50 years old in 5 cases. 19 cases of native Sichuan, 9 cases of Guangdong, 2 cases of Zhejiang, Shanxi 6