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消化性淀粉样变性的趋势:临床、病理及影像学特征

Digestive Amyloidosis Trends: Clinical, Pathological, and Imaging Characteristics.

作者信息

Bucurica Sandica, Nancoff Andreea-Simona, Moraru Miruna Valeria, Bucurica Ana, Socol Calin, Balaban Daniel-Vasile, Mititelu Mihaela Raluca, Maniu Ionela, Ionita-Radu Florentina, Jinga Mariana

机构信息

Department of Internal Medicine and Gastroenterology, Carol Davila University of Medicine and Pharmacology, 020021 Bucharest, Romania.

Department of Gastroenterology, University Emergency Central Military Hospital "Dr. Carol Davila", 024185 Bucharest, Romania.

出版信息

Biomedicines. 2024 Nov 17;12(11):2630. doi: 10.3390/biomedicines12112630.

DOI:10.3390/biomedicines12112630
PMID:39595194
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11591665/
Abstract

Amyloidosis is a group of diseases characterized by the extracellular deposition of abnormally folded, insoluble proteins that lead to organ dysfunction. While it commonly affects the cardiovascular system, gastrointestinal (GI) tract involvement is undetermined. Recent research has focused on understanding the pathophysiology, diagnostic challenges, and therapeutic approaches to GI amyloidosis, particularly in systemic amyloid light-chain (AL) and amyloid A (AA) forms. GI manifestations can include motility disorders, bleeding, and, in severe cases, bowel obstruction. This review highlights the importance of the early recognition of digestive symptoms and associated imagistic findings in GI amyloidosis by analyzing the research that included clinical, pathological, and endoscopic approaches to amyloidosis. A systematic search of the PubMed and Scopus databases identified 19 relevant studies. Our findings showed that amyloid deposits commonly affect the entire GI tract, with AL amyloidosis being the most predominant form. Endoscopic evaluations and biopsy remain key diagnostic tools, with Congo Red staining and mass spectrometry being used to confirm amyloid type. Although progress has been made in diagnosis, the absence of targeted therapies and the indistinct nature of GI symptoms continue to be challenging.

摘要

淀粉样变性是一组以异常折叠的不溶性蛋白质细胞外沉积导致器官功能障碍为特征的疾病。虽然它通常影响心血管系统,但胃肠道受累情况尚不确定。最近的研究集中在了解胃肠道淀粉样变性的病理生理学、诊断挑战和治疗方法,特别是在系统性淀粉样轻链(AL)和淀粉样A(AA)形式方面。胃肠道表现可包括运动障碍、出血,严重时可出现肠梗阻。本综述通过分析包括淀粉样变性的临床、病理和内镜方法在内的研究,强调了早期识别胃肠道淀粉样变性的消化症状及相关影像学表现的重要性。对PubMed和Scopus数据库进行系统检索后,确定了19项相关研究。我们的研究结果表明,淀粉样沉积物通常累及整个胃肠道,其中AL淀粉样变性是最主要的形式。内镜评估和活检仍然是关键的诊断工具,刚果红染色和质谱分析用于确认淀粉样蛋白类型。尽管在诊断方面取得了进展,但缺乏靶向治疗以及胃肠道症状的不明确性质仍然具有挑战性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/50e199690309/biomedicines-12-02630-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/f1c81d884744/biomedicines-12-02630-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/de6a6158b3d2/biomedicines-12-02630-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/a5e246d1b198/biomedicines-12-02630-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/e2df5a9b49e8/biomedicines-12-02630-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/50e199690309/biomedicines-12-02630-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/f1c81d884744/biomedicines-12-02630-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/de6a6158b3d2/biomedicines-12-02630-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/a5e246d1b198/biomedicines-12-02630-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/e2df5a9b49e8/biomedicines-12-02630-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7126/11591665/50e199690309/biomedicines-12-02630-g005.jpg

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引用本文的文献

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BMC Gastroenterol. 2025 Feb 15;25(1):81. doi: 10.1186/s12876-025-03670-z.