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沙特阿拉伯人群中遗传性大疱性表皮松解症的遗传学影响及管理

Genetic Implications and Management of Epidermolysis Bullosa in the Saudi Arabian Population.

作者信息

Shehata Nancy A, Shaik Noor A, Irfan Thalib Husna

机构信息

Department of Dermatology, King Abdullah Medical Complex, Jeddah, SAU.

Department of Genetic Medicine, Faculty of Medicine at King Abdul Aziz University, Jeddah, SAU.

出版信息

Cureus. 2024 Aug 12;16(8):e66678. doi: 10.7759/cureus.66678. eCollection 2024 Aug.

DOI:10.7759/cureus.66678
PMID:39262533
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11389075/
Abstract

Epidermolysis bullosa (EB) is a genetic skin disorder characterized by skin fragility and blister formation. This review explores the genetic basis and management of EB in the Saudi population, emphasizing the need for genetic insights to enable precise diagnosis, targeted treatments, and effective counseling. Diagnosis in Saudi Arabia relies on clinical assessments and genetic testing. Prenatal diagnosis may be suggested in families with children affected by EB, but it is not widely used in the Middle East. Current management focuses on symptom relief, while emerging experimental approaches such as gene and stem cell therapies are under extensive research. Challenges in EB research include developing effective targeted therapies and understanding the variability in how genotypes manifest phenotypically. Continuous research is crucial to enhance diagnostic methods, therapeutic approaches, and overall patient care.

摘要

大疱性表皮松解症(EB)是一种遗传性皮肤病,其特征为皮肤脆弱和水疱形成。本综述探讨了沙特人群中EB的遗传基础和管理,强调了获得遗传见解以实现精确诊断、靶向治疗和有效咨询的必要性。沙特阿拉伯的诊断依赖于临床评估和基因检测。对于有EB患儿的家庭可能会建议进行产前诊断,但在中东地区并未广泛应用。目前的管理重点是缓解症状,而基因和干细胞疗法等新兴实验方法正在进行广泛研究。EB研究中的挑战包括开发有效的靶向疗法以及理解基因型如何在表型上表现出变异性。持续研究对于改进诊断方法、治疗方法和整体患者护理至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/0efb2a049b46/cureus-0016-00000066678-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/d64dde67a4f5/cureus-0016-00000066678-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/cf4613377098/cureus-0016-00000066678-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/0efb2a049b46/cureus-0016-00000066678-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/d64dde67a4f5/cureus-0016-00000066678-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/cf4613377098/cureus-0016-00000066678-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea16/11389075/0efb2a049b46/cureus-0016-00000066678-i03.jpg

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本文引用的文献

1
Emerging Gene Therapeutics for Epidermolysis Bullosa under Development.正在开发中的新型基因疗法治疗大疱性表皮松解症。
Int J Mol Sci. 2024 Feb 13;25(4):2243. doi: 10.3390/ijms25042243.
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Neonatal epidermolysis bullosa: a clinical practice guideline.新生儿大疱性表皮松解症:临床实践指南。
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Revolutionary breakthrough: FDA approves Vyjuvek, the first topical gene therapy for dystrophic epidermolysis bullosa.革命性突破:美国食品药品监督管理局(FDA)批准了Vyjuvek,这是首个用于营养不良型大疱性表皮松解症的局部基因疗法。
Ann Med Surg (Lond). 2023 Oct 17;85(12):6298-6301. doi: 10.1097/MS9.0000000000001422. eCollection 2023 Dec.
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Epidermolysis Bullosa Simplex With Dystonin Gene Mutation: First Reported Case in Saudi Arabia.伴有桥粒斑蛋白基因突变的单纯性大疱性表皮松解症:沙特阿拉伯首例报告病例
Cureus. 2023 Aug 9;15(8):e43206. doi: 10.7759/cureus.43206. eCollection 2023 Aug.
5
Birch Bark Extract: A Review in Epidermolysis Bullosa.桦树皮提取物:对大疱性表皮松解症的综述。
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Gene therapy advances shine the spotlight on epidermolysis bullosa, bringing hope to patients.基因治疗的进展使大疱性表皮松解症备受关注,给患者带来了希望。
Mol Ther. 2023 Jul 5;31(7):1860-1861. doi: 10.1016/j.ymthe.2023.06.002. Epub 2023 Jun 26.
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Ther Clin Risk Manag. 2023 Jun 14;19:455-473. doi: 10.2147/TCRM.S386923. eCollection 2023.
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9
Targeted NGS in Diagnostics of Genodermatosis Characterized by the Epidermolysis Bullosa Symptom Complex in 268 Russian Children.针对以大疱性表皮松解症综合征为特征的遗传性皮肤病的靶向 NGS 检测:268 例俄罗斯儿童病例分析。
Int J Mol Sci. 2022 Nov 18;23(22):14343. doi: 10.3390/ijms232214343.
10
Epidermolysis Bullosa-A Different Genetic Approach in Correlation with Genetic Heterogeneity.大疱性表皮松解症——与遗传异质性相关的不同遗传研究方法
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