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重症联合免疫缺陷病好发于1岁以内婴幼儿,病情严重,死亡率高.近年来国内渐有个例报告.本文两例均因种痘引起全身性坏疽痘而死亡,经尸检证实.例1.男性,6月.种痘后一周,痘疹向四周蔓延扩大.液体渗出和坏死.继之出现弥散性痘疹,融合成片;全身有发热.住院后用新鲜血、抗痘苗人干血浆及抗生素等治疗无效死亡.患儿足月顺产,第六胎,出生后未曾患病.父母系表兄妹结婚,除第一胎健在外,其余四孩均在半岁左右死亡.实验室检查:白细胞8850,中性53%,淋巴47%.IgG363mg%,IgA36mg%,IgM55mg%,体外淋巴细胞转化率50%,血培养无菌生长.
Severe combined immunodeficiency disease occurs in infants and young children within 1 year of age, the condition is serious, the mortality rate is high .In recent years, there are a few cases reported .In this paper, two cases were all caused by acne causing systemic necrosis and confirmed by autopsy. Male, June. A week after the vaccination, the spread of pox to the surrounding expanded. Fluid exudation and necrosis. Followed by diffuse smallpox, fusion into tablets; body has fever. After hospitalization with fresh blood, anti-vaccinia human dry plasma And antibiotics and other treatment died.Patients with full-term birth, the sixth child, no risk after birth.Parents were cousins married, in addition to the first birth of a healthy baby, the remaining four children died in about six months old.Laboratory tests: Leukocyte 8850, neutral 53%, lymph 47% .IgG363mg%, IgA36mg%, IgM55mg%, in vitro lymphocyte transformation rate of 50%, blood culture sterile growth.