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Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis.

机译:血清类胰蛋白酶和SCORMA(SCORing巨噬细胞增多)指数是儿童和成人皮肤肥大细胞增多症的疾病严重程度参数。

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BACKGROUND: Skin lesions are the predominant clinical feature of the commonest form of mastocytosis. Mastocytosis is classified according to World Health Organization criteria. Determination of the levels of mast-cell mediators or their metabolites reflects the mast-cell burden. The extent of cutaneous mastocytosis can be assessed clinically using a scoring system (SCORing MAstocytosis; SCORMA Index) that we have developed. OBJECTIVE: Serum tryptase levels were compared with the SCORMA Index in a large group of paediatric and adult patients to investigate whether there was any correlation between the two. METHODS: The SCORMA Index in 64 patients (31 children and 33 adults) was compared with serum tryptase levels. The results of the first visit at which SCORMA and tryptase were evaluated were analysed. RESULTS: There was a positive correlation between the SCORMA Index and serum tryptase levels, indicating the value of the SCORMA Index in the assessment of mastocytosis with skin involvement. CONCLUSION: The results of this study showed that the SCORMA Index is a useful tool for evaluating the severity of cutaneous mastocytosis. The correlation between the SCORMA Index and serum tryptase levels underlines the benefit of the SCORMA Index as a clinical tool. Repeated SCORMA Index measurements can provide a rapid impression of changes in the clinical state of mastocytosis. This is particularly relevant in children, because taking blood samples from this group is much more difficult. The well-established methods for evaluation of disease severity may be expanded by the rapid SCORMA Index method.
机译:背景:皮肤病变是肥大细胞增多症最常见形式的主要临床特征。肥大细胞增多症是根据世界卫生组织的标准分类的。确定肥大细胞介体或其代谢产物的水平反映了肥大细胞的负担。皮肤肥大细胞增多症的程度可以使用我们开发的评分系统(SCORing MAstocytosis; SCORMA Index)进行临床评估。目的:将一大批儿科和成年患者的血清类胰蛋白酶水平与SCORMA指数进行比较,以调查两者之间是否存在相关性。方法:将64名患者(31名儿童和33名成人)的SCORMA指数与血清类胰蛋白酶水平进行了比较。分析首次访问时评估SCORMA和类胰蛋白酶的结果。结果:SCORMA指数与血清类胰蛋白酶水平呈正相关,表明SCORMA指数在皮肤浸润性肥大性评估中的价值。结论:这项研究的结果表明,SCORMA指数是评估皮肤肥大性严重程度的有用工具。 SCORMA指数与血清类胰蛋白酶水平之间的相关性强调了SCORMA指数作为临床工具的益处。重复的SCORMA指数测量可以快速反映肥大细胞增多症临床状态的变化。这在儿童中尤其重要,因为从该组中采集血液样本要困难得多。快速的SCORMA指数方法可以扩展公认的疾病严重性评估方法。

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