每日一题_2023.09.06

A 10-year-old boy with an X-linked immunodeficiency disease suffers from chronic recurrent gastrointestinal inflammation, which only moderately improves with cyclosporine therapy. The child has had previous laboratory evaluation that showed a negative reaction to the nitroblue tetrazolium test. The patient’s father wants to know how his son can have both an immunodeficiency disease and an autoimmune disease. Deficiencies in which of the following provide a logical link between this child’s immunodeficiency and his autoimmune gut inflammation?

一名患x连锁免疫缺陷病的10岁男孩患慢性复发性胃肠道炎症，使用环孢素治疗后仅略有改善。该儿童之前曾接受过实验室评估，结果显示亚硝基蓝四唑试验呈阴性反应。病人的父亲想知道他的儿子怎么会同时患有免疫缺陷病和自身免疫性疾病。下列哪项缺陷在该儿童的免疫缺陷和自身免疫性肠道炎症之间提供了逻辑联系?

(A) Antibodies 抗体

(B) IgA

(C) IgM

(D) Lysosomes 溶酶体

(E) Neutrophils 中性粒细胞

答案解析：

慢性肉芽肿病(Chronic granulomatous disease, CGD)是中性粒细胞无法完全清除某些吞噬细菌和真菌的疾病。因此，对这些滞留的病原体的慢性免疫反应导致了自我组织损伤的发生。这个答案在CGD(中性粒细胞产生烟酰胺腺嘌呤二核苷酸磷酸酶[NADPH]氧化酶的障碍，导致中性粒细胞不能完全清除某些被吞噬的细菌和真菌)和自身免疫病之间提供了一个逻辑联系。这一答案指向了质疑为什么50%的CGD患者患有与克罗恩病相似的慢性肠道炎症的理论。

正确答案：E