内瑟顿综合征综合病因治疗的临床效果

Q4 Medicine
Nikolay N. Murashkin, Leonid A. Opryatin, Anna S. Bridan-Rostovskaya, Ekaterina S. Pavlova, Roman A. Ivanov, Karine O. Avetisyan, Kirill A. Kulikov
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引用次数: 0

摘要

背景。内瑟顿综合征是一种严重的常染色体隐性遗传病,不仅基于遗传决定的角化疾病,而且基于免疫系统失调。临床病例描述。男童K., 9岁,诊断为严重特应性皮炎,检查时发现内瑟顿综合征。由于存在严重的多致敏和过敏过程的临床体征,给予杜匹单抗。在此治疗期间观察到疾病的波浪形病程。患者在治疗第7个月出现急性加重-广泛性脓疱性皮疹。根据国外关于白细胞介素(IL) 17抑制剂治疗内瑟顿综合征的发病文献,启动了secukinumab治疗。它已经显示出积极的动力。目前患者采用IL-17和IL-4/13抑制剂联合治疗,疗效显著。结论。针对Th17和Th2的基因工程生物药物在内瑟顿综合征的治疗中已显示出其疗效。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Results of Combined Pathogenetic Therapy in Netherton Syndrome: Clinical Case
Background . Netherton syndrome is a severe autosomal recessive disease based not only on genetically determined keratinization disorders, but also on immune system dysregulation. Clinical case description . Boy K., 9 years old, diagnosed with severe atopic dermatitis and revealed Netherton syndrome during examinations. The dupilumab was administered due to the presence of severe polysensitization and clinical signs of allergic process. Wave-like course of the disease was observed during this therapy. Patient had acute exacerbation — generalized pustular rash — at the 7th month of treatment. The therapy with secukinumab was initiated according to the foreign literature on pathogenetic treatment of Netherton syndrome with interleukin (IL) 17 inhibitor. It has shown positive dynamics. Currently patient is administered with the combined therapy of IL-17 and IL-4/13 inhibitors with significant positive effect. Conclusion. Genetically engineered biologic drugs targeting both the Th17 and Th2 have shown their efficacy in Netherton syndrome management.
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来源期刊
Voprosy Sovremennoi Pediatrii - Current Pediatrics
Voprosy Sovremennoi Pediatrii - Current Pediatrics Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.70
自引率
0.00%
发文量
48
审稿时长
8 weeks
期刊介绍: The main purpose of the academic journal "Current Pediatrics" is to cover the issues related to health, emotional state, and social adaptation of children. In the journal we publish the most pressing issues of research and applied problems (such as social paediatrics; clinical findings, anamnesis, and diagnosis of paediatric diseases; prevention and rehabilitation; supply of healthy and sick child), reviews on the current state of medical science and management of health care in Russia. Along with the concern for providing a high level of basic research publications, the journal, being a mass media tool, tends to meet the interests and requirements of practitioners from different regions and bring up vital and urgent questions. To accomplish this purpose the journal includes the materials of practical interest presented in the following sections: "Exchange of experience", "Doctor''s aid", "Continuous professional education", "Expert Opinion". The journal presents actual official information from the Union of Paediatricians of Russia and publishes materials on the history of medical science, congresses, seminars, conferences, both in our country and abroad.
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