University Hospital Leipzig, Department of Internal Medicine, Dermatology and Neurology, Medical Clinic for Gastroenterology and Rheumatology, Leipzig, Germany.
PLoS One. 2012;7(7):e42139. doi: 10.1371/journal.pone.0042139. Epub 2012 Jul 25.
Cystic fibrosis-related liver disease (CFLD) is present in up to 30% of cystic fibrosis patients and can result in progressive liver failure. Diagnosis of CFLD is challenging. Non-invasive methods for staging of liver fibrosis display an interesting diagnostic approach for CFLD detection.
We evaluated transient elastography (TE), acoustic radiation force impulse imaging (ARFI), and fibrosis indices for CFLD detection.
TE and ARFI were performed in 55 adult CF patients. In addition, AST/Platelets-Ratio-Index (APRI), and Forns' score were calculated. Healthy probands and patients with alcoholic liver cirrhosis served as controls.
Fourteen CF patients met CFLD criteria, six had liver cirrhosis. Elastography acquisition was successful in >89% of cases. Non-cirrhotic CFLD individuals showed elastography values similar to CF patients without liver involvement. Cases with liver cirrhosis differed significantly from other CFLD patients (ARFI: 1.49 vs. 1.13 m/s; p = 0.031; TE: 7.95 vs. 4.16 kPa; p = 0.020) and had significantly lower results than individuals with alcoholic liver cirrhosis (ARFI: 1.49 vs. 2.99 m/s; p = 0.002). APRI showed the best diagnostic performance for CFLD detection (AUROC 0.815; sensitivity 85.7%, specificity 70.7%).
ARFI, TE, and laboratory based fibrosis indices correlate with each other and reliably detect CFLD related liver cirrhosis in adult CF patients. CF specific cut-off values for cirrhosis in adults are lower than in alcoholic cirrhosis.
囊性纤维化相关肝病（CFLD）在多达 30%的囊性纤维化患者中存在，并可导致进行性肝衰竭。CFLD 的诊断具有挑战性。用于分期肝纤维化的非侵入性方法为 CFLD 的检测提供了一种有趣的诊断方法。
我们评估了瞬时弹性成像（TE）、声辐射力脉冲成像（ARFI）和纤维化指数在 CFLD 检测中的应用。
对 55 例成年 CF 患者进行了 TE 和 ARFI 检查。此外，还计算了 AST/血小板比值指数（APRI）和福纳斯评分。健康对照者和酒精性肝硬化患者作为对照组。
14 例 CF 患者符合 CFLD 标准，6 例患者患有肝硬化。>89%的病例成功获得了弹性成像数据。无肝硬化的 CFLD 患者的弹性成像值与无肝受累的 CF 患者相似。肝硬化病例与其他 CFLD 患者差异显著（ARFI：1.49 与 1.13 m/s；p = 0.031；TE：7.95 与 4.16 kPa；p = 0.020），且明显低于酒精性肝硬化患者（ARFI：1.49 与 2.99 m/s；p = 0.002）。APRI 对 CFLD 检测具有最佳的诊断性能（AUROC 0.815；敏感性 85.7%，特异性 70.7%）。
ARFI、TE 和基于实验室的纤维化指数相互关联，可可靠地检测成年 CF 患者的 CFLD 相关肝硬化。成人 CF 相关肝硬化的 CF 特定截断值低于酒精性肝硬化。
