[Systemic mastocytosis--definition of an internal disease].

Medizinische Klinik Pub Date : 2010-08-01 Epub Date: 2010-09-08 DOI:10.1007/s00063-010-1093-8
Jürgen Homann, Ulrich W Kolck, Andreas Ehnes, Thomas Frieling, Martin Raithel, Gerhard J Molderings
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引用次数: 11

Abstract

Systemic mastocytosis comprises disorders characterized by an accumulation of genetically altered mast cells in all organs and tissues due to an increased proliferation rate and reduced apoptosis of those pathologic mast cells. Release of their mediators can effectively influence organ function and can lead to systemic effects without inducing traces in routinely used laboratory parameters or imaging methods. In most cases, little invasive investigations allow diagnosing the disease and, hence, an appropriate therapy consisting of a basic medication with antihistamine and mast cell membrane-stabilizing compounds that should be supplemented, if required, by a medication adapted to individual symptoms, can be initiated. Because of the probably high prevalence of the disorder, systemic mastocytosis should be considered as a differential diagnosis in particular in the case of chronic gastrointestinal complaints such as abdominal pain/discomfort possibly associated with diarrhea, at an early stage.

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【系统性肥大细胞增多症——一种内部疾病的定义】。
全身性肥大细胞增多症包括以遗传改变的肥大细胞在所有器官和组织中积累为特征的疾病,这是由于这些病理性肥大细胞增殖率增加和凋亡减少所致。它们的介质的释放可以有效地影响器官功能,并可导致全身效应,而不会在常规使用的实验室参数或成像方法中引起痕迹。在大多数情况下,少量的侵入性检查可以诊断疾病,因此,可以开始适当的治疗,包括抗组胺药和肥大细胞膜稳定化合物的基本药物,如果需要,还可以补充适应个体症状的药物。由于这种疾病的患病率可能很高,系统性肥大细胞增多症应被视为一种鉴别诊断,特别是在早期出现慢性胃肠道疾病(如可能与腹泻相关的腹痛/不适)的情况下。
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Medizinische Klinik
Medizinische Klinik 医学-医学:内科
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