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作者对先天性非球形红细胞溶血性贫血(CNSHA)的两种变异性——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,进行了红细胞动力学类型的对比研究。作者所研究的4例CNSHA患者中,男2(L.W.和K.W.),女2(T.S.和O.M.),年龄19~65岁,其中1例(K.W.)为PPKD,另3例(O.M.T.S.和L.W.)为SDPKA。所有4例患者的无肝胆疾患,红细胞渗透脆性,红细胞乙酰胆硷酯酶活力正常。
The author of two variants of congenital non-spherical red blood cell 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 type of erythrocyte dynamics were compared. The authors studied 4 CNSHA patients, male 2 (LW and KW), female 2 (TS and OM), aged 19 to 65 years old, 1 case (KW) PPKD, the other 3 cases (OMTS and LW) SDPKA. All 4 patients without hepatobiliary disease, red blood cell infiltration fragility, normal red blood cell acetylcholinesterase activity.