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Ⅰ型干扰素病25例临床特点分析 被引量:3
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作者 王伟 王薇 +6 位作者 邹丽萍 何庭艳 马明圣 李文道 于仲勋 杨军 宋红梅 《中华儿科杂志》 CAS CSCD 北大核心 2021年第12期1043-1047,共5页
目的总结分析Ⅰ型干扰素病患儿的临床特点,为其早识别、早诊断提供线索。方法回顾性分析2016年1月至2021年9月北京协和医院儿科就诊的20例及深圳市儿童医院风湿免疫科就诊的5例基因确诊的Ⅰ型干扰素病患儿的临床资料,对其基因数据、临... 目的总结分析Ⅰ型干扰素病患儿的临床特点,为其早识别、早诊断提供线索。方法回顾性分析2016年1月至2021年9月北京协和医院儿科就诊的20例及深圳市儿童医院风湿免疫科就诊的5例基因确诊的Ⅰ型干扰素病患儿的临床资料,对其基因数据、临床表现及辅助检查结果进行分析。结果25例患儿中男12例、女13例。发病年龄1日龄~11岁。其中21例(84%)在3岁前起病。Aicardi-Goutières综合征(AGS)14例(3例AGS1型、1例AGS2型、4例AGS3型、1例AGS6型、5例AGS7型)、腺苷脱氨酶2缺陷(DADA2)6例、干扰素基因刺激蛋白相关婴儿期起病的血管炎(SAVI)3例、脊椎软骨发育异常伴免疫调节异常(SPENCD)2例。共18例(72%)患儿神经系统受累,其中16例(64%)表现为颅内钙化、11例(44%)为肌张力异常、10例(40%)为脑白质病变、6例(24%)为癫痫、5例(20%)脑萎缩及5例(20%)脑卒中。15例(60%)患儿皮肤受累,其中8例(32%)为冻疮样皮疹,4例(16%)为网状青斑、3例(12%)为红斑,结节性红斑及雷诺现象各有2例(8%)。12例(48%)自身免疫抗体阳性,10例(40%)生长发育落后,8例(32%)肺间质病变,7例(28%)甲状腺功能异常。1例(4%)患儿11岁死亡。结论Ⅰ型干扰素病可累及全身多个脏器,具有全身炎症及自身免疫性疾病的特点。3岁前起病、神经系统症状(脑血管事件、颅内钙化、脑白质病变、脑萎缩)、皮疹(冻疮样皮疹、网状青斑、红斑)、自身免疫抗体阳性、发育落后、肺间质病变、甲状腺功能异常等特点对Ⅰ型干扰素病有提示意义。 展开更多
关键词 遗传性自身炎症性疾病 干扰素Ⅰ型 儿童
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Recent development and progress of structural energy devices 被引量:1
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作者 Yong Liu zhongxun yu +5 位作者 Jia Chen Chenxi Li Zhengjie Zhang Xiaoyu Yan Xinhua Liu Shichun Yang 《Chinese Chemical Letters》 SCIE CAS CSCD 2022年第4期1817-1830,共14页
In order to fully replace the traditional fossil energy supply system, the efficiency of electrochemical energy conversion and storage of new energy technology needs to be continuously improved to enhance its market c... In order to fully replace the traditional fossil energy supply system, the efficiency of electrochemical energy conversion and storage of new energy technology needs to be continuously improved to enhance its market competitiveness. The structural design of energy devices can achieve satisfactory energy conversion and storage performance. To achieve lightweight design, improve mechanical support, enhance electrochemical performance, and adapt to the special shape of the device, the structural energy devices develop very quickly. To help researchers analyze the development and get clear on developing trend,this review is prepared. This review summarizes the latest developments in structural energy devices, including special attention to fuel cells, lithium-ion batteries, lithium metal batteries, and supercapacitors.Finally, the existing problems of structural energy devices are discussed, and the current challenges and future opportunities are summarized and prospected. Structural energy devices can undoubtedly overcome the performance bottlenecks of traditional energy devices, break the limitations of existing materials and structures, and provide a guidance for the development of equipment with high performance,light weight and low cost in the future. 展开更多
关键词 Structural energy devices Fuel cells Lithium-ion batteries Lithium metal batteries SUPERCAPACITORS
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