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血红蛋白(简称Hb)分子中某些氨基酸被替换或丢失,发生变构效应(Allosteric effect)使Hb分子变得不稳定,形成不稳定Hb(简称UHb)。后者可引起慢性溶血性贫血,具有红细胞内包涵体和暗褐色尿的特征。故称为“具有色素尿的包涵体贫血”(Inclusion-body anemia with pigmenturia)或“遗传性亨氏小体贫血”(Hereditary Heinz-body anemia)。自从Dacie等,介绍了检查UHb的特异试验——热变性试验以来,已发现了不少UHb,其中有的不形成包涵体,因此Carrell等把这一组贫血称为“不稳定血红蛋白溶血性贫血”(Unstable hemoglobin hemolytic anemia,简称UHHA)。最近Carrell报导了36种UHb,有26种UHHA的临床经过类似先天性非球形细胞性溶血性贫血,在服用某些药物时可以引起急性溶血和Hb尿(如Hb Zürich)。Hb K(?)ln的研究资料和病例报告为最多,有认为日本的Hb Ube I亦可能是Hb K(?)ln。其它一些UHb参见表1。
Some of the amino acids in the hemoglobin (Hb) molecule are replaced or lost, and the Allosteric effect causes the Hb molecule to become unstable and form unstable Hb (abbreviated as UHb). The latter can cause chronic hemolytic anemia, with the characteristics of intra-erythrocyte inclusions and dark brown urine. It is called “Inclusion-body anemia with pigmenturia” or “Hereditary Heinz-body anemia”. Since Dacie et al. Introduced the UHb-specific assay-thermal denaturation test, a number of UHb have been found, some of which do not form inclusion bodies, so Carrell et al. Refer to this group of anemias as “unstable hemoglobin hemolytic anemia ”(Unstable hemoglobin hemolytic anemia, referred to as UHHA). Carrell recently reported 36 UHb, 26 UHHA clinically similar congenital non-spherical hemolytic anemias, which can cause acute hemolysis and Hb urination (eg, Hb Zrich) when taking certain drugs. Hb K (?) Ln research data and case reports for the most, there is that the Japanese Hb Ube I may also be Hb K (?) Ln. See Table 1 for some other UHb.