在自身免疫性多胞胎症候群病 (Candidiasis Ectodermal dystrophy) 中的脏疾病 (APECED):一个系统性审查
Mohammadreza Shafiei1, Solale Hosseini2, Soodeh Ghadimi3
1Student Research Committee, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran. Mohammadrezashafiei.md@gmail.com.
BMC pediatrics
|February 25, 2025
概括
自身免疫性多性腺瘤症候群性外皮缩症 (APECED) 患者经常出现脏问题,脏瘤是最常见的. 早期监测脏并发症对于APECED患者的更好的结果至关重要.
科学领域:
- 免疫学 免疫学 免疫学
- 腎臟病學 (nephrology) 是一種醫學專業.
- 遗传学 遗传学 是一个
背景情况:
- 自体免疫多分泌症候群症外皮变 (APECED) 或自体免疫多分泌症综合征I型 (APS-I) 是一种免疫的先天错误.
- 它表现为免疫失调,内分泌问题和非内分泌表现,如慢性粘膜皮肤候群病.
- 脏疾病是APECED患者显著的,研究不足的并发症.
研究的目的:
- 系统地审查APECED患者并发症的文献.
- 为了确定与APECED相关的脏疾病的患病率和类型.
- 强调脏监测在APECED管理中的重要性.
主要方法:
- 在PubMed,Web of Science和Scopus进行了全面的文献搜索.
- 对APECED的临床和免疫学特征进行了系统审查.
- 选择了包括APECED患者在内的研究,这些患者至少有一种部并发症.
主要成果:
- 分析了30项研究中的93名APECED患者.
- 在超过38%的患者中存在瘤瘤.
- 管间炎 (23%),高血压 (13%),尿路感染 (20.5%) 也是常见的并发症.
结论:
- 脏并发症是APECED患者的一个重大问题.
- 定期监测功能对于管理APECED至关重要.
- 解决脏问题可以改善患者的治疗结果,并保持功能.
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