Ana Piñar-Gutiérrez, Esther Quintana-Gallego, Pablo J Remón-Ruiz, Ángeles Pizarro, Irene González-Navarro, Andrés Jiménez-Sánchez, Silvia García-Rey, María Del Carmen Roque-Cuéllar, Sheila Gato, Inmaculada Domínguez, Francisco Javier Castell, Manuel Romero-Gómez, Pedro Pablo García-Luna
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The secondary objective was to determine the diagnostic yield of widely available non-invasive liver markers for steatosis and fibrosis, and vibration controlled transitional elastography (VCTE) releasing Control Attenuation Parameter (CAP) (dB/m) and stiffness (kPa), with the aim of proposing a diagnostic algorithm.</p><p><strong>Methods: </strong>We conducted a cross-sectional study including 101 adult patients with cystic fibrosis seen in a multidisciplinary unit. The study encompassed a clinical evaluation, morpho-functional assessment, VCTE, non-invasive liver markers and MR-PDFF and MRE. Diagnostic accuracy was assessed using ROC curves and 2 × 2 tables.</p><p><strong>Results: </strong>MR-PDFF detected hepatic steatosis in 18 of 101 (17.8 %) patients, while MRE detected significant liver fibrosis in 15 of 101 (14.9 %). The VCTE cut-off with the best diagnostic yield, determined by the Youden index, was 222 dB/m for the presence of steatosis (AUC 0.864 (95 % CI 0.768-0.961; p < 0.001) and the VCTE cut-off was 6.65 kPa for liver fibrosis (AUC 0.951(95 % CI 0.81-1; p = 0.053). A screening algorithm for hepatic steatosis was developed using the fatty liver index (FLI) and CAP, with a negative predictive value of 83.3 %. For liver fibrosis, it was outperformed by the Hepamet Fibrosis Score (HFS) and VCTE, with a negative predictive value of 100 %.</p><p><strong>Conclusions: </strong>The prevalence of hepatic steatosis and liver fibrosis was 17.8 % and 14.9 %, respectively. VCTE alone or in combination with FLI for steatosis or HFS for fibrosis demonstrated high diagnostic accuracy. 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引用次数: 0
摘要
背景与目的：囊性纤维化累及肝胆是一种异质性和全身性的实体。主要目的是通过磁共振质子密度脂肪分数（MR-PDFF）和磁共振弹性成像（MRE）确定囊性纤维化成人队列中脂肪变性和肝纤维化的患病率。次要目标是确定广泛使用的脂肪变性和纤维化的非侵入性肝脏标志物的诊断率，以及释放控制衰减参数（CAP） （dB/m）和刚度（kPa）的振动控制过渡弹性成像（VCTE），目的是提出一种诊断算法。方法：我们进行了一项横断面研究，包括101名在多学科单位就诊的囊性纤维化成年患者。该研究包括临床评估、形态功能评估、VCTE、非侵入性肝脏标志物、MR-PDFF和MRE。采用ROC曲线和2 × 2表评估诊断准确性。结果：101例患者中MR-PDFF检出肝脂肪变性18例（17.8%），MRE检出显著肝纤维化15例（14.9%）。由约登指数确定的最佳诊断率的VCTE截止值为222 dB/m，表明存在脂肪变性(AUC 0.864 (95% CI 0.768-0.961；p < 0.001)，肝纤维化的VCTE截止值为6.65 kPa (AUC 0.951(95% CI 0.81-1；P = 0.053)。采用脂肪肝指数（FLI）和CAP建立肝脂肪变性筛查算法，阴性预测值为83.3%。对于肝纤维化，其表现优于肝纤维化评分（HFS）和VCTE，阴性预测值为100%。结论：肝脂肪变性和肝纤维化的发生率分别为17.8%和14.9%。VCTE单独或联合FLI诊断脂肪变性或HFS诊断纤维化显示出较高的诊断准确性。这种方法有效地排除了脂肪变性和纤维化，从而减少了MR-PDFF和MRE研究的需要。
Non-invasive evaluation of steatosis and fibrosis in the liver in adults patients living with cystic fibrosis.
Background & aims: Cystic fibrosis hepatobiliary involvement is a heterogeneous and systemic entity. The primary objective was to determine the prevalence of steatosis, by magnetic resonance-proton density fat fraction (MR-PDFF), and liver fibrosis, by magnetic resonance elastography (MRE), in a cohort of adults with cystic fibrosis. The secondary objective was to determine the diagnostic yield of widely available non-invasive liver markers for steatosis and fibrosis, and vibration controlled transitional elastography (VCTE) releasing Control Attenuation Parameter (CAP) (dB/m) and stiffness (kPa), with the aim of proposing a diagnostic algorithm.
Methods: We conducted a cross-sectional study including 101 adult patients with cystic fibrosis seen in a multidisciplinary unit. The study encompassed a clinical evaluation, morpho-functional assessment, VCTE, non-invasive liver markers and MR-PDFF and MRE. Diagnostic accuracy was assessed using ROC curves and 2 × 2 tables.
Results: MR-PDFF detected hepatic steatosis in 18 of 101 (17.8 %) patients, while MRE detected significant liver fibrosis in 15 of 101 (14.9 %). The VCTE cut-off with the best diagnostic yield, determined by the Youden index, was 222 dB/m for the presence of steatosis (AUC 0.864 (95 % CI 0.768-0.961; p < 0.001) and the VCTE cut-off was 6.65 kPa for liver fibrosis (AUC 0.951(95 % CI 0.81-1; p = 0.053). A screening algorithm for hepatic steatosis was developed using the fatty liver index (FLI) and CAP, with a negative predictive value of 83.3 %. For liver fibrosis, it was outperformed by the Hepamet Fibrosis Score (HFS) and VCTE, with a negative predictive value of 100 %.
Conclusions: The prevalence of hepatic steatosis and liver fibrosis was 17.8 % and 14.9 %, respectively. VCTE alone or in combination with FLI for steatosis or HFS for fibrosis demonstrated high diagnostic accuracy. This approach effectively allows for the exclusion of steatosis and fibrosis, thereby reducing the need for MR-PDFF and MRE studies.
期刊介绍：
The Journal of Cystic Fibrosis is the official journal of the European Cystic Fibrosis Society. The journal is devoted to promoting the research and treatment of cystic fibrosis. To this end the journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to cystic fibrosis. The journal also publishes news and articles concerning the activities and policies of the ECFS as well as those of other societies related the ECFS.
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