Soothill J F, Harvey B A
Arch Dis Child. 1976 Feb;51(2):91-9. doi: 10.1136/adc.51.2.91.
Serum opsonization of yeasts for phagocytosis by normal polymorphonuclear leucocytes was defective in 11 of 43 children with unexplained frequent infections. The children had a range of infections, largely bacterial, and only 3 had diarrhoea and rash in infancy. A similar defect in at least 6 of the 9 mothers of these children (of either sex), with normal function in the fathers, suggests that the defect was primary and was transmitted by an unusual form of dominant inheritance. Four of 72 healthy adults and 1 of 11 children with unrelated disease showed similar defective function, but the incidence of the defect in the patients with frequent infection was significantly greater than this. The defective function can be corrected, in vitro and in vivo, by normal plasma at concentrations too low to be effective alone. This suggests that there is a defective factor rather than an inhibitor, and that different factors are limiting in normal and in defective plasma. Sera from affected members of the same family do not correct each other, but defective sera from different families usually do.
在43名不明原因频繁感染的儿童中,有11名儿童的血清对酵母进行调理以使其被正常多形核白细胞吞噬的功能存在缺陷。这些儿童有一系列感染,主要是细菌感染,只有3名儿童在婴儿期有腹泻和皮疹。这些儿童(无论性别)的9名母亲中至少有6名存在类似缺陷,而父亲功能正常,这表明该缺陷是原发性的,并且通过一种不寻常的显性遗传形式传递。72名健康成年人中有4名以及11名患无关疾病的儿童中有1名表现出类似的功能缺陷,但频繁感染患者中该缺陷的发生率明显高于此。这种功能缺陷在体外和体内都可以通过浓度过低而单独无效的正常血浆来纠正。这表明存在缺陷因子而非抑制剂,并且正常血浆和缺陷血浆中限制因素不同。同一家族受影响成员的血清不能相互纠正,但来自不同家族的缺陷血清通常可以。