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一名11岁女性高免疫球蛋白E综合征的综合多学科管理:一例儿科病例报告

Comprehensive Multidisciplinary Management of Hyper-IgE Syndrome in an 11-Year-Old Female: A Pediatric Case Report.

作者信息

Elashmawy Ahmed, Chokr Mohammadali, Sharif Saima, Ferrantino Lauren

机构信息

Pediatrics, Wayne State University School of Medicine, Detroit, USA.

Anesthesia, Corewell Health East, Dearborn, USA.

出版信息

Cureus. 2024 Jul 25;16(7):e65377. doi: 10.7759/cureus.65377. eCollection 2024 Jul.

DOI:10.7759/cureus.65377
PMID:39188427
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11346673/
Abstract

Hyper-IgE syndrome (HIES) or Job syndrome is a rare immunodeficiency characterized by elevated levels of IgE and recurrent infections, eczema, and connective tissue abnormalities. Patients with HIES are prone to recurrent pyogenic and opportunistic infections due to impaired immune responses. Here, we present the case of an 11-year-old female diagnosed with HIES, who was admitted to the hospital with bacterial pneumonia and leg pain associated with a history of osteopenia. The patient's clinical course included fever, cough, throat pain, and leg pain. Management involved a rigorous course of antibiotics, antifungals, and cultures of pertinent pathogens, along with imaging of the lower extremity. This case underscores the importance of appropriate management strategies for patients with HIES and their comorbidities to mitigate the risk of infections and improve patient outcomes.

摘要

高免疫球蛋白E综合征(HIES)或乔布综合征是一种罕见的免疫缺陷病,其特征为免疫球蛋白E水平升高、反复感染、湿疹和结缔组织异常。由于免疫反应受损,HIES患者易发生反复的化脓性和机会性感染。在此,我们报告一例11岁女性诊断为HIES的病例,该患者因细菌性肺炎和与骨质减少病史相关的腿痛入院。患者的临床病程包括发热、咳嗽、咽痛和腿痛。治疗包括严格使用抗生素、抗真菌药物以及对相关病原体进行培养,同时对下肢进行影像学检查。该病例强调了针对HIES患者及其合并症采取适当管理策略以降低感染风险和改善患者预后的重要性。

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