Department of Pediatrics, Medical University of Silesia, Katowice, Poland.
Upper Silesian Child Health Centre, Katowice, Poland.
Mediators Inflamm. 2020 Mar 25;2020:5696185. doi: 10.1155/2020/5696185. eCollection 2020.
Changes in the liver and bile ducts observed in patients diagnosed with cystic fibrosis result from inflammatory processes as well as fibrosis, remodeling, apoptosis, and cholestasis. As a consequence, portal hypertension, cirrhosis, and hepatic failure may develop. So far, the complexity of these processes has not been elucidated. . The aim of the study was to evaluate the selected parameters of hepatitis and fibrosis (Fibrotest, Actitest, and APRI) in patients diagnosed with cystic fibrosis. . The study included 79 patients with cystic fibrosis, aged 1 to 20 years (mean age 9.8 years), 49 girls (62%) and 30 boys (38%). The analysis involved the following: age, sex, clinical manifestations, laboratory tests evaluating pancreas function, parameters of liver damage, and cholestasis. Fibrotest, Actitest, and APRI were performed in all subjects. . Elevated parameters of hepatic cell damage (hypertransaminasemia) were found in 31/79 (39.2%) patients, while abnormal cholestasis parameters in 21/79 (26.6%). The abnormal results of Fibrotest were reported in 15% of patients (12/79), while of Actitest in 10% (8/79). In contrast, elevated APRI values were found in only 7.6% (6/79) of subjects. There was a statistically significant correlation between APRI and age (higher values were observed in younger children) and between Fibrotest and Actitest and pancreatic insufficiency (higher values were found in subjects without this abnormality). Moreover, Fibrotest values were significantly higher in girls. There was no correlation between Fibrotest, Actitest, and APRI values and the type of mutation. . It appears that Fibrotest may be used as an early marker of liver fibrosis in patients with cystic fibrosis. Increased APRI values were only found in subjects with advanced hepatic lesions, most often in the form of portal hypertension.
在诊断为囊性纤维化的患者中观察到的肝脏和胆管变化是由炎症过程以及纤维化、重塑、细胞凋亡和胆汁淤积引起的。因此，可能会发展为门静脉高压、肝硬化和肝衰竭。到目前为止，这些过程的复杂性尚未阐明。。本研究的目的是评估诊断为囊性纤维化的患者的肝炎和纤维化的选定参数（Fibrotest、Actitest 和 APRI）。。该研究纳入了 79 名年龄在 1 至 20 岁（平均年龄 9.8 岁）的囊性纤维化患者，其中 49 名女孩（62%）和 30 名男孩（38%）。分析包括以下内容：年龄、性别、临床表现、评估胰腺功能的实验室检查、肝损伤和胆汁淤积的参数。所有患者均进行了 Fibrotest、Actitest 和 APRI 检查。31/79（39.2%）例患者出现肝损伤（高转氨酶血症）的参数升高，21/79（26.6%）例患者出现异常胆汁淤积参数。15%（12/79）的患者报告 Fibrotest 结果异常，10%（8/79）的患者报告 Actitest 结果异常。相反，仅在 7.6%（6/79）的受试者中发现 APRI 值升高。APRI 值与年龄呈统计学显著相关（年龄越小，值越高），Fibrotest 和 Actitest 与胰腺功能不全呈统计学显著相关（无此异常的患者值较高）。此外，Fibrotest 值在女孩中显著较高。Fibrotest、Actitest 和 APRI 值与突变类型之间无相关性。。似乎 Fibrotest 可作为囊性纤维化患者肝纤维化的早期标志物。仅在具有晚期肝损伤的受试者中发现升高的 APRI 值，最常见的形式是门静脉高压。
