Alexopoulou Alexandra, Pouriki Sophia, Vasilieva Larisa, Alexopoulos Theodoros, Filaditaki Vasiliki, Gioka Maria, Diamantea Filia, Dourakis Spyros P
a 2nd Department of Internal Medicine and Research Laboratory , Medical School, National and Kapodistrian University of Athens, Hippokration Hospital , Athens , Greece.
b Third Respiratory Medicine Department , Sismanogleio General Hospital , Athens , Greece.
Scand J Gastroenterol. 2018 Dec;53(12):1547-1552. doi: 10.1080/00365521.2018.1534986. Epub 2019 Jan 2.
In cystic fibrosis (CF), liver disease (LD) is the third leading cause of mortality. As liver biopsy was considered inconsistent in CFLD diagnosis, a combination of modalities were utilized in the conventional Debray criteria (DC). More recently, noninvasive liver fibrosis biomarkers were applied by Koh et al (New criteria-NC). In the current study, we aimed to evaluate noninvasive biomarkers for the CFLD diagnosis.
Longitudinal data were collected from a cohort of genetically confirmed CF patients. CFLD was diagnosed by both DC and NC. Apart from transient elastography (TE) > 6.8 kPa, biomarkers incorporated in the NC included AST/ALT-ratio (AAR) ≥ 1, FIB-4 index ≥3.25 and APRI >0.50.
62 patients with CF, [56.5% male, age at enrollment 25 (22-31) years], were prospectively followed-up for 33 (28-36) months. Sixteen (25.8%) and 27 (43.5%) patients met DC and NC, respectively. Twenty-four fulfilling NC had at least one positive biomarker (6 TE, 7 AAR, 6 both TE and AAR, 2 both APRI and AAR and 3 both APRI and TE). Thirteen (48.1%) had diffuse LD/cirrhosis by the NC and all had at least one additional parameter classifying them as CFLD. From the 14 (51.8%) with no-diffuse-LD, 64.3%, 14.3% and 21.4% had 2, 3 and 4 of the necessary modalities incorporated in NC, respectively, confirming their classification as CFLD. TE was 100% specific to rule in CFLD but had a moderate sensitivity.
NC were able to identify 17.7% more CFLD patients compared to DC. The multiple biomarkers incorporated in NC may enhance the ability to detect CFLD.
在囊性纤维化（CF）中，肝脏疾病（LD）是第三大死亡原因。由于肝活检在CFLD诊断中被认为不一致，传统的德布雷标准（DC）采用了多种检查方法的组合。最近，Koh等人应用了非侵入性肝纤维化生物标志物（新标准-NC）。在本研究中，我们旨在评估用于CFLD诊断的非侵入性生物标志物。
从一组基因确诊的CF患者中收集纵向数据。通过DC和NC诊断CFLD。除了瞬时弹性成像（TE）>6.8kPa外，NC纳入的生物标志物还包括AST/ALT比值（AAR）≥1、FIB-4指数≥3.25和APRI>0.50。
62例CF患者（男性占56.5%，入组年龄25[22-31]岁）接受了33[28-36]个月的前瞻性随访。分别有16例（25.8%）和27例（43.5%）患者符合DC和NC标准。24例符合NC标准的患者至少有一项生物标志物呈阳性（6例TE、7例AAR、6例TE和AAR均阳性、2例APRI和AAR均阳性以及3例APRI和TE均阳性）。13例（48.1%）通过NC诊断为弥漫性LD/肝硬化，且所有患者至少有一项额外参数将其归类为CFLD。在14例（51.8%）无弥漫性LD的患者中，分别有64.3%、14.3%和21.4%的患者具备NC中纳入的2、3和4种必要检查方法，证实了他们被归类为CFLD。TE对CFLD的诊断具有100%的特异性，但敏感性中等。
与DC相比，NC能够多识别出17.7%的CFLD患者。NC中纳入的多种生物标志物可能会增强检测CFLD的能力。
