蚕豆病病因发病原理探讨——Ⅵ.蚕豆病患者血清抑制凝集因子的观察

Studies on the Etiology and Pathogenesis of Favism. VI. Observations on an agglutinative Inhibition Factor in Patient's Serum

据报道,在蚕豆病多发区,红细胞葡糖-6-磷酸脱氢酶(G-6PD)缺乏者仅10.3～31.5％吃蚕豆后发病,而且这些病人尽管每年都吃蚕豆,也不是每年都发病(多数仅于幼年及老年期发病l～2次),说明本病的发生尚有其他因素参与。1974年,Nathan等发现蚕豆病人血清中缺乏一种抑制蚕豆液诱发红细胞凝集的因子,并认为这是与蚕豆病发生有关的另一种缺陷。1977～1980年。

It is proposed that there tad been a qualitative defect of IgA in favic serum related to the pathogenesis of favism (Nathan et al, 1974). To check this, a series of experiments have been carried out and the results are:1.Deficient of an agglutinative inhibition factor was proved in 90.4% pat-ients(47/52) and 8/8 newborns, but only 5.4% (6/101), 7.4% (2/27) and 1/5 in no rmal individuals with a history of favism (PH) and in the patient's first degree relatives respectively.2. When IgA (60mg/ml) was added to inhibition factor-deficient serum, agglutinative inhibition appeals in 5 out of 6,and slight agglutination in 1 out of 6 specimens.3. The average level of IgA in 13 favic serum was significantly than those of 24 normal and 23 PH, but the levels of IgG and IgM were similar in these groups.4.Determining the changes of agglutinative inhibition of patient's serum (inhibition factor deficient serum) various days after episodes reveal in general, that inhibition reappear by the 4th day (since adimision).Our findings suggest the deficiency is not genetic in nature, and it appears that IgA deficiency may probably play a role in the pathogenesis of favism.