噬血细胞性淋巴组织细胞增多症致发热性感染相关性癫痫综合征1例。

Q3 Medicine
Xiao-Lu Deng, Li-Fen Yang, Xia Wang, Hui Zhang, Jian He, Jing Peng
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引用次数: 0

摘要

患者为女童,10岁,因发热5天,外周血全血细胞减少2天入院。骨髓检查显示存在吞噬活性,外周血检查显示全血细胞减少,铁蛋白增加,纤维蛋白原减少,甘油三酯和sCD25增加,自然杀伤细胞活性降低,从而诊断为噬血细胞性淋巴组织细胞增多症(HLH)。患儿入院当天出现惊厥,迅速发展为难治性癫痫持续状态,符合热性感染相关癫痫综合征的表现。积极免疫治疗后HLH得到控制,伴顽固性癫痫后遗症,认知功能基本在正常范围内。本文在国内首次报道了HLH所致发热性感染相关癫痫综合征的发病情况,旨在提高临床医生对该病的认识。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Febrile infection-related epilepsy syndrome caused by hemophagocytic lymphohistiocytosis: a case report].

The patient was a girl, aged 10 years, who was admitted due to fever for 5 days and pancytopenia in peripheral blood for 2 days. Bone marrow examination showed the presence of phagocytic activity, and peripheral blood tests showed pancytopenia, an increase in ferritin, a reduction in fibrinogen, increases in triglyceride and sCD25, and a reduction in natural killer cell activity, which led to the diagnosis of hemophagocytic lymphohistiocytosis (HLH). On the day of admission, the child developed convulsions and rapidly progressed to refractory status epilepticus, which was consistent with the manifestations of febrile infection-related epilepsy syndrome. HLH was controlled after active immunotherapy, with the sequela of refractory epilepsy, and her cognitive function was essentially within normal limits. This article reports the condition of febrile infection-related epilepsy syndrome caused by HLH for the first time in China, in order to improve the awareness of this disease among clinicians.

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来源期刊
中国当代儿科杂志
中国当代儿科杂志 Medicine-Pediatrics, Perinatology and Child Health
CiteScore
1.50
自引率
0.00%
发文量
5006
期刊介绍: The Chinese Journal of Contemporary Pediatrics (CJCP) is a peer-reviewed open access periodical in the field of pediatrics that is sponsored by the Central South University/Xiangya Hospital of Central South University and under the auspices of the Ministry of Education of China. It is cited as a source in the scientific and technological papers of Chinese journals, the Chinese Science Citation Database (CSCD), and is one of the core Chinese periodicals in the Peking University Library. CJCP has been indexed by MEDLINE/PubMed/PMC of the American National Library, American Chemical Abstracts (CA), Holland Medical Abstracts (EM), Western Pacific Region Index Medicus (WPRIM), Scopus and EBSCO. It is a monthly periodical published on the 15th of every month, and is distributed both at home and overseas. The Chinese series publication number is CN 43-1301/R;ISSN 1008-8830. The tenet of CJCP is to “reflect the latest advances and be open to the world”. The periodical reports the most recent advances in the contemporary pediatric field. The majority of the readership is pediatric doctors and researchers.
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