Huisman T H, Reese A L, Gardiner M B, Wilson J B, Lam H, Reynolds A, Nagle S, Trowell P, Zeng Y T, Huang S Z, Sukumaran P K, Miwa S, Efremov G D, Petkov G, Sciarratta G V, Sansone G
Am J Hematol. 1983 Apr;14(2):133-48. doi: 10.1002/ajh.2830140205.
The gamma chain compositions of the fetal hemoglobins of 2453 newborn babies from East Asian countries (1350 babies), from Italy, Yugoslavia, Bulgaria, and Georgia (417 Caucasian babies), and 686 black babies from Georgia were determined by high pressure liquid chromatography. Unusual results for a limited number of babies were confirmed by chemical analyses, and were evaluated further by family studies. Statistical analyses indicated high gene frequencies for the A gamma T chain in Italian (f = 0.237), Yugoslavian and Bulgarian (f = 0.238), and white Georgia babies (f = 0.224), a lower frequency in Japan (f = 0.178), and India (f = 0.173), and particularly in mainland China (f = 0.079). The A gamma T gene frequency in normal (AA) Black babies was 0.102. When a beta S or beta C mutation was also present this frequency was greatly decreased, particularly in babies with the AC condition (f = 0.036). These results suggest the near absence of the A gamma T mutation on the chromosome also carrying the beta C determinant. Most babies had the expected G gamma values which vary between 60 and 80%, but several (mainly black) babies had higher values (between 80 and 90%), while one normal black baby had a G gamma value of (nearly) 100%. This condition may be a form of A gamma +1-thalassemia and has been discussed in detail elsewhere (Blood 58:491-500, 1981). Thirty-five clinically normal (mainly Chinese, Indian, and Japanese) babies had G gamma values of about 40%. Twenty-six babies had A gamma I values of about 60%, while the remaining nine babies had A gamma T and A gamma I chains in a ratio of either 1 to 2 or 1 to 1. Two additional newborns did not produce any G gamma chains, but had only A gamma I chains or A gamma T chains. Family studies failed to indicate a specific hematological abnormality. These unusual ratios between the G gamma and A gamma (either A gamma I or A gamma T) chains have led to speculations regarding possible genetic abnormalities present in these infants.
采用高压液相色谱法测定了来自东亚国家的2453名新生儿(1350名婴儿)、来自意大利、南斯拉夫、保加利亚和格鲁吉亚的417名高加索婴儿以及来自格鲁吉亚的686名黑人婴儿的胎儿血红蛋白的γ链组成。少数婴儿的异常结果通过化学分析得到证实,并通过家系研究作进一步评估。统计分析表明,意大利(f = 0.237)、南斯拉夫和保加利亚(f = 0.238)以及格鲁吉亚白人婴儿(f = 0.224)中AγT链的基因频率较高,日本(f = 0.178)和印度(f = 0.173)较低,中国大陆尤其低(f = 0.079)。正常(AA型)黑人婴儿中AγT基因频率为0.102。当同时存在βS或βC突变时,该频率会大幅下降,尤其是AC型婴儿(f = 0.036)。这些结果表明,在携带βC决定簇的染色体上几乎不存在AγT突变。大多数婴儿的Gγ值在60%至80%之间,符合预期,但有几个婴儿(主要是黑人)的值较高(在80%至90%之间),而一名正常黑人婴儿的Gγ值(几乎)为100%。这种情况可能是一种Aγ +1-地中海贫血形式,已在其他地方详细讨论过(《血液》58:491 - 500, 1981)。35名临床正常的婴儿(主要是中国、印度和日本婴儿)的Gγ值约为40%。26名婴儿的AγI值约为60%,其余9名婴儿的AγT链和AγI链比例为1比2或1比1。另外两名新生儿不产生任何Gγ链,只有AγI链或AγT链。家系研究未能表明存在特定的血液学异常。Gγ链与Aγ链(AγI或AγT)之间这些异常比例引发了对这些婴儿可能存在的基因异常的猜测。