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对囊性纤维化中 ABPA 和曲霉菌病的诊断生物标志物的研究。

An investigation into biomarkers for the diagnosis of ABPA and aspergillus disease in cystic fibrosis.

机构信息

Division of Respiratory Medicine, BC Children's Hospital, Vancouver, British Columbia, Canada.

出版信息

Pediatr Pulmonol. 2019 Nov;54(11):1787-1793. doi: 10.1002/ppul.24465. Epub 2019 Jul 29.

Abstract

BACKGROUND

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to Aspergillus fumigatus (Af) which occurs in 10% of patients with cystic fibrosis (CF). ABPA is associated with increased morbidity and accelerated lung function decline; however, existing diagnostic criteria are nonspecific and diagnosis remains challenging. As ABPA is driven by Th2 inflammation, the aim of this study was to evaluate exhaled nitric oxide (FE ), eosinophilic cationic protein (ECP), peripheral eosinophil count, and bronchodilator response (BDR) in patients with CF.

METHODS

A prospective observational cohort study of pediatric CF patients in a tertiary center. Patients had a clinical and serologic ABPA assessment, FE , serum ECP, peripheral eosinophil count, and assessment of BDR. Patients were stratified into three groups; ABPA, Af sensitized (AFS), and non-ABPA non-Af-sensitized (non-AFS).

RESULTS

A total of 62 patients were included in the study: 13% ABPA, 19% AFS, and 68% non-AFS. Mean FE was higher in the ABPA group at 37.8 ppb compared to AFS 15.1 ppb (P = .05) and non-AFS 13.7 ppb (P = .04). Mean peripheral eosinophil count in ABPA group was also higher at 1000 cells/uL, compared to AFS 221 cells/uL (P = .03) and non-AFS 220 cells/uL (P = .03). Mean BDR in ABPA group was 13% compared to 5.5% in non-AFS (P = .01). Serum ECP was higher in patients with ABPA positive compared to the other groups, although this was not statistically significant.

CONCLUSION

In children with cystic fibrosis, FE and peripheral eosinophil counts are elevated in ABPA compared to those that are just sensitized to Aspergillus and may serve as useful diagnostic tests.

摘要

背景

变应性支气管肺曲霉病(ABPA)是一种由烟曲霉(Af)引起的过敏反应性肺部疾病,发生在 10%的囊性纤维化(CF)患者中。ABPA 与发病率增加和肺功能加速下降有关;然而,现有的诊断标准不具有特异性,诊断仍然具有挑战性。由于 ABPA 是由 Th2 炎症驱动的,本研究旨在评估囊性纤维化患者的呼出气一氧化氮(FE )、嗜酸性阳离子蛋白(ECP)、外周血嗜酸性粒细胞计数和支气管扩张剂反应(BDR)。

方法

对三级中心的儿科 CF 患者进行前瞻性观察队列研究。患者进行临床和血清学 ABPA 评估、FE 、血清 ECP、外周血嗜酸性粒细胞计数和 BDR 评估。患者分为三组;ABPA、Af 致敏(AFS)和非 ABPA 非-Af 致敏(非 AFS)。

结果

本研究共纳入 62 例患者:ABPA 占 13%,AFS 占 19%,非 AFS 占 68%。ABPA 组的平均 FE 为 37.8 ppb,高于 AFS 组的 15.1 ppb(P=0.05)和非 AFS 组的 13.7 ppb(P=0.04)。ABPA 组的平均外周血嗜酸性粒细胞计数也较高,为 1000 个/μL,高于 AFS 组的 221 个/μL(P=0.03)和非 AFS 组的 220 个/μL(P=0.03)。ABPA 组的平均 BDR 为 13%,而非 AFS 组为 5.5%(P=0.01)。ABPA 阳性患者的血清 ECP 高于其他组,但差异无统计学意义。

结论

在囊性纤维化儿童中,与仅对曲霉致敏的患者相比,ABPA 患者的 FE 和外周血嗜酸性粒细胞计数升高,这可能是有用的诊断测试。

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