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粪便钙卫蛋白作为小儿囊性纤维化患者肠道炎症的标志物。

Fecal calprotectin as a marker of intestinal inflammation in pediatric cystic fibrosis patients.

作者信息

Akbulut Damla Gökçeer, Özden Tülin Ayşe, Cantez Mustafa Serdar, Ertekin Vildan, Tamay Zeynep, Durmaz Özlem

机构信息

University of Health Sciences, Kanuni Sultan Süleyman Training and Research Hospital, Department of Pediatric Cardiology, Istanbul, Türkiye.

Department of Pediatric Biochemistry, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey.

出版信息

Eur J Pediatr. 2025 Sep 8;184(10):605. doi: 10.1007/s00431-025-06442-9.

Abstract

UNLABELLED

The study aimed to evaluate fecal calprotectin levels as indicators of intestinal inflammation in children with cystic fibrosis while examining their relationship with clinical signs, genetic mutations, and therapeutic approaches. Due to the limited number of patients with certain mutation types and the heterogeneity of mutations, patients were grouped accordingly for the analysis of fecal calprotectin levels, in relation to genetic mutation categories. This single-centre study at Istanbul Medical Faculty included 45 cystic fibrosis patients (19 girls, 26 boys) aged 1-18 years and 45 age- and sex-matched healthy controls. We assessed fecal calprotectin levels, clinical features, genetic mutations, and laboratory parameters. Genetic mutations were categorized, and patients were grouped based on mutation types for analysis. The patient group showed significantly lower height Z-scores compared to controls (p < 0.007). Patients with cystic fibrosis had significantly higher fecal calprotectin levels (177.8 ± 3.0 µg/g) than controls (53.7 ± 2.2 µg/g) (p < 0.001). The levels of fecal calprotectin reached 537.7 ± 2.8 µg/g during pulmonary exacerbations but remained at 149.4 ± 2.8 µg/g during non-exacerbation periods (p = 0.012). The fecal calprotectin levels of patients taking pancreatic enzyme replacement therapy reached 245.3 ± 2.7 µg/g, whereas patients without this therapy had levels of 92.6 ± 2.9 µg/g (p = 0.005). The antibiotic users demonstrated higher fecal calprotectin levels (305.4 ± 3.1 µg/g vs 145.4 ± 2.9 µg/g), although the difference was not statistically significant (p = 0.074). The ΔF508 mutation carriers showed a trend of elevated fecal calprotectin levels (261.2 ± 3.4 µg/g) compared to homozygous polymorphism carriers (99.5 ± 3.6 µg/g), but the difference did not reach statistical significance (p = 0.082). The results of the sweat test showed a positive correlation with fecal calprotectin levels (r = 0.377, p = 0.020).

CONCLUSION

Our results show that bowel inflammation is problematic among children with cystic fibrosis, and fecal calprotectin is a useful marker. The peripheral zone effects become evident through elevated levels, which occur during pulmonary exacerbations and when patients receive pancreatic enzyme therapy. The degree of intestinal inflammation and fecal calprotectin levels vary depending on the type of genetic mutation in patients with cystic fibrosis.

WHAT IS KNOWN

• Cystic fibrosis causes inflammation in both respiratory and gastrointestinal systems. • Fecal calprotectin is a biomarker for detecting intestinal inflammation in pediatric diseases.

WHAT IS NEW

• Fecal calprotectin levels increase during pulmonary exacerbations and in children receiving pancreatic enzyme replacement therapy, indicating persistent intestinal inflammation. • Levels may also vary with CF genetic mutation types, but only a few studies have addressed this association. • Sweat test results correlate with intestinal inflammation, suggesting a broader role beyond diagnosis.

摘要

未标注

本研究旨在评估粪便钙卫蛋白水平作为囊性纤维化患儿肠道炎症指标的情况,同时研究其与临床体征、基因突变及治疗方法的关系。由于某些突变类型的患者数量有限且突变具有异质性,故将患者按基因突变类别分组,以分析粪便钙卫蛋白水平。这项在伊斯坦布尔医学院进行的单中心研究纳入了45例年龄在1至18岁的囊性纤维化患者(19名女孩,26名男孩)以及45名年龄和性别匹配的健康对照者。我们评估了粪便钙卫蛋白水平、临床特征、基因突变及实验室参数。对基因突变进行分类,并根据突变类型对患者分组进行分析。患者组的身高Z评分显著低于对照组(p < 0.007)。囊性纤维化患者的粪便钙卫蛋白水平(177.8 ± 3.0 μg/g)显著高于对照组(53.7 ± 2.2 μg/g)(p < 0.001)。肺部加重期粪便钙卫蛋白水平达到537.7 ± 2.8 μg/g,但在非加重期维持在149.4 ± 2.8 μg/g(p = 0.012)。接受胰酶替代治疗的患者粪便钙卫蛋白水平达到245.3 ± 2.7 μg/g,而未接受该治疗的患者水平为92.6 ± 2.9 μg/g(p = 0.005)。使用抗生素的患者粪便钙卫蛋白水平较高(305.4 ± 3.1 μg/g对145.4 ± 2.9 μg/g),尽管差异无统计学意义(p = 0.074)。与纯合子多态性携带者(99.5 ± 3.6 μg/g)相比,ΔF508突变携带者的粪便钙卫蛋白水平有升高趋势(261.2 ± 3.4 μg/g),但差异未达到统计学意义(p = 0.082)。汗液试验结果与粪便钙卫蛋白水平呈正相关(r = 0.377,p = 0.020)。

结论

我们的结果表明,肠道炎症在囊性纤维化患儿中是个问题,粪便钙卫蛋白是一个有用的标志物。外周区域效应通过在肺部加重期及患者接受胰酶治疗时升高的水平得以体现。肠道炎症程度和粪便钙卫蛋白水平因囊性纤维化患者的基因突变类型而异。

已知信息

• 囊性纤维化会导致呼吸和胃肠道系统炎症。• 粪便钙卫蛋白是检测儿科疾病肠道炎症的生物标志物。

新发现

• 肺部加重期及接受胰酶替代治疗儿童的粪便钙卫蛋白水平升高,表明存在持续性肠道炎症。• 水平也可能因囊性纤维化基因突变类型而异,但仅有少数研究涉及这种关联。• 汗液试验结果与肠道炎症相关,表明其作用超出诊断范畴。

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