湖北籍正常人红细胞糖无氧酵解途径中8种关键酶活性正常参考值的建立及其临床应用

ESTABLISHMENT AND CLINICAL APPLICATION OF NORMAL ACTIVITY VALUES OF EIGHT IMPORTANT RED CELL ENZYMES IN THE EMBDEN-MEYERHOF PATHWAY IN NATIVE PEOPLE IN HUBEI PROVINCE

建立湖北籍正常人之红细胞糖无氧酵解途径（EMP）中8种关键酶的正常参考值并应用于临床。应用改装后的国产分光光度计，参照国际血液学标准委员会推荐的方法，进行红细胞EMP中8关种关键酶活性测定。结果和结论：（1）40例湖北籍汉族献血员（单位IU／gHb）：PK12·34±2.36，PFK10.95±2.65，PGK270±59.15，GPI66．54±10．97，HKI．29±0．36，ALD2．69±0．67，DPGM5．57±1．16，TPI2177±464。（2）首次发现纯湖北籍汉族遗传性PK缺陷症1例。（3）发现10例慢性再生障碍性贫血（CAA）和12例MDS中均存在获得性红细胞EMP酶病。（4）发现CAA和MDS－RA对红细胞EMP酶活性改变的影响是不相同的。（5）8例智力低下伴贫血者的8酶活性值均在正常范围内。

To establish the normal activity values of eight important red cell enzymes in the Embden-Meyerhof Pathway (EMP) in Hubei Province and to apply them for clinic. The methods recommended by international Committee for Standerdization in haematology and the spectrophotometer (751-GW type, made in China) were used to measure the activity values of eight important red cell enzymes in EMP. Results showed: (1)Normal values of 40 blood donors (IU/gHb):Pyruvate kinase (PK) 12. 341 2. 36, Phosphofructokinase (PFK) 10. 95 ±2. 65, Phosphoglycerate Kinase (PGK) 274± 59. 15, Glucosephosphate isomerase (GPI) 66. 54± 10. 97. Hexokinase (HK) 1. 29±0. 36, Aldolase (ALD) 2. 69±0. 67,Diphospboglycerate mutase (DPGM) 5. 57 ±1. 16, Triosephosphate isomerase (TPI) 2177 ± 464. (2) One patient of pure native of Hubei Province was first found to have hereditary PK deficiency. (3)It was found that the erythrocyte enzyme disorders in EMP in 10 patients with chronic aplastic anemia (CA) and in 12 patients with MDS were acquirde. (4)It was found that CAA was different from MDS-RA on effects of erythrocytic enzymopathy in EMP. (5)The activity values of eight enzymes from 8 patients with mental retardation were within normal limit.