Dana Jérémy, Girard Muriel, Franchi-Abella Stéphanie, Berteloot Laureline, Benoit-Cherifi Martina, Imbert-Bismut Françoise, Sermet-Gaudelus Isabelle, Debray Dominique
Department of Pediatric Radiology, Hôpital Necker-Enfants Malades, AP-HP, Paris, France; IHU-Strasbourg (Institut Hospitalo-Universitaire), Strasbourg, France; Institut National de la Santé et de la Recherche Médicale (Inserm), U1110, Institut de Recherche sur les Maladies Virales et Hépatiques, Strasbourg, France.
Pediatric Hepatology unit, Centre de Référence Maladies Rares (CRMR) de l'atrésie des voies biliaires et cholestases génétiques (AVB-CG), National network for rare liver diseases (Filfoie), ERN rare liver, Hôpital Necker-Enfants Malades, AP-HP, Université de Paris, Paris, France; Inserm U1151, Institut Necker-Enfants Malades, Paris, France.
Clin Res Hepatol Gastroenterol. 2022 Mar;46(3):101855. doi: 10.1016/j.clinre.2021.101855. Epub 2021 Dec 20.
Reliable markers are needed for early diagnosis and follow-up of liver disease in Cystic Fibrosis (CF). The objective was to evaluate the diagnostic performance of Transient Elastography (TE), Real-Time ShearWave Ultrasound Elastography (SWE), Magnetic Resonance Elastography (MRE) and the FibroTest as markers of Cystic Fibrosis Liver Disease (CFLD).
A monocentric prospective cross-modality comparison study was proposed to all children (6 to 18 years of age) attending the CF center. Based on liver ultrasound findings, participants were classified into 3 groups: multinodular liver or portal hypertension (Nodular US/PH, advanced CFLD), heterogeneous increased echogenicity (Heterogeneous US, CFLD) or neither (Normal/Homogeneous US, no CFLD). The 4 tests were performed on the same day. The primary outcome was the FibroTest value and liver stiffness measurements (LSM).
55 participants (mean age 12.6 ± 3.3 years; 25 girls) were included between 2015 and 2018: 23 in group Nodular US/PH, 8 in group Heterogeneous US and 24 in group Normal/Homogeneous US (including 4 with steatosis). LSM on TE, SWE and MRE were higher in participants with CFLD (groups Nodular US/PH and Heterogeneous US) compared to others (group Normal/Homogeneous US) (p<0.01), while FibroTest values did not differ (p = 0.09). The optimal cut-off values for predicting CFLD on TE, SWE and MRE were 8.7 (AUC=0.83, Se=0.71, Sp=0.96), 7.8 (AUC=0.85, Se=0.73, Sp=0.96) and 4.15 kPa (AUC=0.68, Se=0.73, Sp=0.64), respectively. LSM predicted the occurrence of major liver-related events at 3 years. TE and SWE were highly correlated (Spearman's ρ=0.9) and concordant in identifying advanced CFLD (Cohen's κ=0.84) while MRE was moderately correlated and concordant with TE (ρ=0.41; κ=36) and SWE (ρ=0.5; κ=0.50).
This study demonstrated excellent diagnostic performance of TE, SWE and MRE for the diagnosis of CFLD.
囊性纤维化（CF）患者的肝脏疾病需要可靠的标志物用于早期诊断和随访。本研究旨在评估瞬时弹性成像（TE）、实时剪切波超声弹性成像（SWE）、磁共振弹性成像（MRE）以及FibroTest作为囊性纤维化肝病（CFLD）标志物的诊断性能。
对所有在CF中心就诊的6至18岁儿童进行一项单中心前瞻性跨模态比较研究。根据肝脏超声检查结果，将参与者分为3组：多结节性肝脏或门静脉高压（结节性超声/门静脉高压，晚期CFLD）、不均匀回声增强（不均匀超声，CFLD）或两者皆无（正常/均匀超声，无CFLD）。4项检查在同一天进行。主要观察指标为FibroTest值和肝脏硬度测量值（LSM）。
2015年至2018年共纳入55名参与者（平均年龄12.6±3.3岁；25名女孩）：结节性超声/门静脉高压组23例，不均匀超声组8例，正常/均匀超声组24例（包括4例脂肪变性患者）。与其他组（正常/均匀超声组）相比，CFLD患者（结节性超声/门静脉高压组和不均匀超声组）的TE、SWE和MRE的LSM更高（p<0.01），而FibroTest值无差异（p=0.09）。TE、SWE和MRE预测CFLD的最佳截断值分别为8.7（AUC=0.83，Se=0.71，Sp=0.96）、7.8（AUC=0.85，Se=0.73，Sp=0.96）和4.15kPa（AUC=0.68，Se=0.73，Sp=0.64）。LSM可预测3年内主要肝脏相关事件的发生。TE和SWE高度相关（Spearman's ρ=0.9），在识别晚期CFLD方面具有一致性（Cohen's κ=0.84），而MRE与TE（ρ=0.41；κ=0.36）和SWE（ρ=0.5；κ=0.50）中度相关且具有一致性。
本研究表明TE、SWE和MRE在诊断CFLD方面具有出色的诊断性能。
