[Gastrointestinal manifestations of systemic mast cell activation disease - A practice-oriented guide to clinical picture, diagnostics and therapy].

IF 1.6 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Zeitschrift fur Gastroenterologie Pub Date : 2025-02-01 Epub Date: 2025-01-03 DOI:10.1055/a-2468-5553
Martin Raithel, Jürgen Homann, Ralf J Rieker, Gerhard J Molderings
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Abstract

Systemic mast cell activation disease (MCAD) is an epigenetic and genetic disease entity with a very pronounced clinical symptomatology in a variety of clinical manifestations in potentially every organ and tissue due to inappropriate release of mast cell mediators accompanied with the accumulation of both morphologically normal and mutated mast cells. Due to the prevalence of the disease of 17% in Germany, gastroenterologists and endoscopists are often unknowingly faced with MCAD in everyday clinical practice. In addition, gastroenterological examinations are an essential part of the diagnosis of MCAD. It is therefore essential for every physician working in gastroenterology to possess basic knowledge of this disease and, in particular, to be informed about its problems in the field of gastroenterology. This overview summarizes the current state of knowledge on the causes, diagnosis and treatment of the highly complex MCAD, focusing on the gastroenterological aspects.

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[全身性肥大细胞活化病的胃肠道表现-临床图像、诊断和治疗的实践导向指南]。
全身性肥大细胞活化病(MCAD)是一种表观遗传和遗传性疾病,由于肥大细胞介质的不适当释放伴随着形态正常和突变肥大细胞的积累,可能在每个器官和组织中表现出多种临床表现,具有非常明显的临床症状。由于该疾病在德国的患病率为17%,因此在日常临床实践中,胃肠病学家和内窥镜医师经常在不知情的情况下面临MCAD。此外,胃肠病学检查是诊断MCAD的重要组成部分。因此,对于每一位从事胃肠病学工作的医生来说,掌握本病的基本知识,特别是了解其在胃肠病学领域的问题是至关重要的。本文综述了目前关于高度复杂的MCAD的病因、诊断和治疗的知识状况,重点是胃肠病学方面。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Zeitschrift fur Gastroenterologie
Zeitschrift fur Gastroenterologie 医学-胃肠肝病学
CiteScore
1.40
自引率
15.40%
发文量
562
审稿时长
6-12 weeks
期刊介绍: Die Zeitschrift für Gastroenterologie ist seit über 50 Jahren die führende deutsche Fachzeitschrift auf dem Gebiet der Gastroenterologie. Sie richtet sich an Gastroenterologen und alle anderen gastroenterologisch interessierten Ärzte. Als offizielles Organ der Deutschen Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten sowie der Österreichischen Gesellschaft für Gastroenterologie und Hepatologie informiert sie zuverlässig und aktuell über die wichtigen Neuerungen und Entwicklungen in der Gastroenterologie.
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