GI Manifestations With a Focus on the Esophagus: Recent Progress in Understanding Pathogenesis.
GI Manifestations With a Focus on the Esophagus: Recent Progress in Understanding Pathogenesis.
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DOI：
10.1007/s11926-019-0841-x
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发表时间：
2019-07-03
影响因子：
5
通讯作者：
Kahrilas P
中科院分区：
文献类型：
--
作者：
Tétreault MP;Kahrilas P
食道功能障碍在系统性硬化症（SSc）患者中很常见。硬皮病食道疾病的治疗选择有限。在这里，我们讨论了最近在硬皮病食管疾病患者的诊断、治疗和特征方面的最新进展。在过去的几年里，新的诊断工具为SSc患者的食管运动障碍提供了深入的了解。正在试验的新药可能会改善伴有食道功能障碍的SSc患者的症状和生活质量。分子分层方法有助于识别SSc患者食道中的分子特征。Friend白血病整合1 （Fli1）条件敲除小鼠是第一个报道具有SSc特征的食管表型的动物模型。伴有食管功能障碍的SSc患者的临床表现具有异质性，使诊断和治疗复杂化。食管症状和功能障碍诊断工具的改进，以及SSc小鼠模型和患者活检中分子方法的使用，为改善SSc食管疾病的特征提供了机会，这将有助于改善管理和治疗决策。
Esophageal dysfunction is common in systemic sclerosis (SSc) patients. Limited treatment options are available for scleroderma esophageal disease. Here, we discuss recent updates on the diagnosis, treatment, and characterization that have been made in patients with scleroderma esophageal disease. In the past few years, novel diagnostic tools have provided insight into esophageal dysmotility in SSc patients. New drugs are being tested and might improve symptoms and quality of life in SSc patients with esophageal dysfunction. Molecular stratification methods have facilitated the identification of molecular signatures in the esophagus of SSc patients. The Friend leukemia integration 1 (Fli1) conditional knockout mouse is the first animal model to report an esophageal phenotype with SSc features. The clinical presentation in SSc patients with esophageal dysfunction is heterogeneous, complicating diagnosis and management. The improvement of diagnostic tools for esophageal symptoms and dysfunction and the use of molecular approaches in SSc mouse models and patient biopsies offer an opportunity to improve the characterization of SSc esophageal disease, which should help improve management and treatment decisions.
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影响因子：
3.5
作者：
Kimmel JN;Carlson DA;Hinchcliff M;Carns MA;Aren KA;Lee J;Pandolfino JE
通讯作者：
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5.5
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影响因子：
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作者：
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