IPEX syndrome concurrent with gut-origin sepsis: A child patient report and literature review

doi:10.3969/j.issn.1004-583X.2021.05.013

Abstract

Abstract:

In the study, a child patient with immune dysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome concurrent with gut-origin sepsis who was admitted to our hospital was selected, we analyzed in detail the clinical manifestations, auxiliary examinations, diagnosis and treatment of his and reviewed related literature. This result shows that it is necessary to increase the awareness and attention of IPEX syndrome concurrent with gut-origin sepsis, and for children, especially infants, with special disease manifestations, such as early refractory diarrhea, multiple endocrine diseases, autoimmune phenomena, systemic inflammatory response syndrome as well as growth retardation. We may conside genetic testing for systemic immune system diseases while symptomatic treatment; effective regular treatment options such as gene therapy or allogeneic hematopoietic stem cell transplantation are helpful in achieving long-term survival.

Key words: IPEX syndrome, gut-origin sepsis, klebsiella pneumoniae, genetic testing

CLC Number:

R722.1

Eamran Hossain, Tian Ya, Chen Yuan, Zhang Shaodan, Zhang Huifeng. IPEX syndrome concurrent with gut-origin sepsis: A child patient report and literature review[J]. Clinical Focus, 2021, 36(5): 453-457.

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URL: https://huicui.hebmu.edu.cn/EN/10.3969/j.issn.1004-583X.2021.05.013

https://huicui.hebmu.edu.cn/EN/Y2021/V36/I5/453

Figures/Tables 2

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