Imoto Koji, Goya Takeshi, Azuma Yuki, Hioki Tomonobu, Aoyagi Tomomi, Nagata Hazumu, Nishizaki Akiko, Kakino Takamori, Ishikita Ayako, Yamamura Kenichiro, Sakamoto Ichiro, Tanaka Masatake, Abe Kohtaro, Ogawa Yoshihiro
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences , Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582, Japan.
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582, Japan.
BMC Gastroenterol. 2025 May 8;25(1):341. doi: 10.1186/s12876-025-03965-1.
The Fontan procedure has improved the prognosis of patients with a functional single ventricle; however, late complications-including Fontan-associated liver disease (FALD)-have surfaced as clinical concerns. FALD with signs of portal hypertension has been defined as advanced FALD (aFALD) due to its poor prognosis. Recently, noninvasive tests (NITs) have been found to predict liver fibrosis in FALD. Liver stiffness measurement excluding strain elastography (SE) was affected by hepatic congestion; however, to our knowledge, not many studies have evaluated the SE-derived Liver Fibrosis Index (LFI). This study aimed to determine the efficacy of NITs, especially LFI, for discriminating aFALD.
In this retrospective study, 46 Japanese patients with FALD were included and classified into the aFALD (33 patients; 22 males and 11 females; median age: 28.0 years) and non-aFALD (13 patients; seven males and six females; median age: 22.0 years) groups based on the presence/absence of signs of portal hypertension.
The platelet count, FIB-4 index, Forns index, and LFI differed significantly between the two groups and demonstrated moderate accuracy for discriminating aFALD. The shear wave velocity (Vs) measured by Shear Wave Elastography (SWE) did not differ significantly between the two groups. The cut-off value of platelet counts below 185 × 10/μL had 78.8% sensitivity and 92.3% specificity. While 25/26 (96.2%) of the patients with FALD who had platelet counts below 185 × 10/μL were aFALD, 8/20 (40.0%) of the patients with FALD who had platelet counts above below 185 × 10/μL were also aFALD, indicating the need for additional markers. In the patients with FALD who had platelet counts above 185 × 10/μL, only SE indicated moderate diagnostic accuracy, and the LFI cut-off value of 2.21 had 100% sensitivity and 75.0% specificity.
Using a two-step approach, discriminating aFALD with platelet counts below 185 × 10/μL by platelets alone, and for those with higher platelet counts, requiring LFI > 2.21 could discriminate aFALD with high accuracy. Early detection of aFALD and early intervention, including testing for aFALD, may lead to an improved prognosis of aFALD.
Fontan手术改善了功能性单心室患者的预后；然而，包括Fontan相关肝病（FALD）在内的晚期并发症已成为临床关注的问题。伴有门静脉高压体征的FALD因其预后不良被定义为晚期FALD（aFALD）。最近，已发现非侵入性检查（NITs）可预测FALD中的肝纤维化。排除应变弹性成像（SE）的肝脏硬度测量受肝充血影响；然而，据我们所知，评估基于SE的肝脏纤维化指数（LFI）的研究不多。本研究旨在确定NITs，尤其是LFI，对鉴别aFALD的有效性。
在这项回顾性研究中，纳入了46例日本FALD患者，并根据是否存在门静脉高压体征分为aFALD组（33例患者；22例男性和11例女性；中位年龄：28.0岁）和非aFALD组（13例患者；7例男性和6例女性；中位年龄：22.0岁）。
两组间血小板计数、FIB-4指数、Forns指数和LFI有显著差异，并显示出鉴别aFALD的中等准确性。剪切波弹性成像（SWE）测量的剪切波速度（Vs）在两组间无显著差异。血小板计数低于185×10/μL的临界值具有78.8%的敏感性和92.3%的特异性。虽然血小板计数低于185×10/μL的FALD患者中有25/26（96.2%）为aFALD，但血小板计数高于185×10/μL的FALD患者中有8/20（40.0%）也是aFALD，这表明需要其他标志物。在血小板计数高于185×10/μL的FALD患者中，只有SE显示出中等诊断准确性，LFI临界值2.21具有100%的敏感性和75.0%的特异性。
采用两步法，仅通过血小板鉴别血小板计数低于185×10/μL的aFALD，对于血小板计数较高的患者，要求LFI>2.21可高精度鉴别aFALD。早期检测aFALD并进行早期干预，包括检测aFALD，可能会改善aFALD的预后。
