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作者对先天性非球形红细胞溶血性贫血(CNSHA)的两种变种——原发性丙酮酸激酶缺乏(PPKD)和继发性丙酮酸激酶缺乏(SDPKA,原发性红细胞ATP酶(Mg~(++))活力缺乏引起红细胞ATP水平增高和继发性丙酮酸激酶活力降低),进行红细胞动力学类型的对比研究。作者研究的四例CNSHA患者中,男二例(L.W.和K.W.),女二例(T.S.和O.M.),年龄19～65岁,其中一例(K.W.)为PPKD,另三例(O.M.,T.S.及L.W.)为SDPKA。所有四例患者均无肝胆疾病,红细胞渗透脆性,红细胞乙酰胆硷酯酶活力正常。Coomb’s试
The author of the two variants of congenital non-spherical hemolytic anemia (CNSHA) - primary pyruvate kinase deficiency (PPKD) and secondary pyruvate kinase deficiency (SDPKA, primary erythrocyte ATPase (Mg ~ ( ++)) Lack of vitality caused by elevated erythrocyte ATP levels and secondary pyruvate kinase activity decreased), the red blood cell kinetic types of comparative study. Among the four CNSHA patients studied by the authors, two were males (LW and KW) and two were females (TS and OM), aged 19-65 years. One patient (KW) was PPKD and the other three patients (OM, TS and LW ) Is SDPKA. All four patients had no hepatobiliary disease, fragile erythrocytes, and normal erythrocyte acetylcholinesterase activity. Coomb’s test