Evaluation of diagnostic criteria for mild-to-advanced stages of Fontan-associated liver disease: A nationwide epidemiological survey in Japan.

IF 3.9 3区医学 Q1 GASTROENTEROLOGY & HEPATOLOGY

Hepatology Research Pub Date : 2024-11-27 DOI:10.1111/hepr.14141

Tomomi Kogiso, Daisuke Tokuhara, Satoko Ohfuji, Atsushi Tanaka, Tatsuya Kanto

{"title":"Evaluation of diagnostic criteria for mild-to-advanced stages of Fontan-associated liver disease: A nationwide epidemiological survey in Japan.","authors":"Tomomi Kogiso, Daisuke Tokuhara, Satoko Ohfuji, Atsushi Tanaka, Tatsuya Kanto","doi":"10.1111/hepr.14141","DOIUrl":null,"url":null,"abstract":"Aim: Fontan-associated liver disease (FALD) is a complication after Fontan surgery, and a common cause of liver tumors and cirrhosis. However, no diagnostic criteria for FALD have been established, leading to an underestimation of its prevalence.Methods: We conducted a national survey to elucidate the characteristics of FALD by collecting data from high-volume centers managing patients who had undergone the Fontan surgery in Japan. In total, 1168 patients were enrolled in the study. First, we examined typical liver findings on ultrasonography after the Fontan surgery. Next, we proposed diagnostic criteria for FALD and advanced FALD based on blood tests, imaging, liver tumors, and pathological examinations. We investigated the sensitivity of histologically diagnosed FALD and advanced FALD based on criteria for blood or imaging tests.Results: Hepatomegaly, hepatic venous dilatation, caudate lobe enlargement, splenomegaly, liver atrophy, ascites, hepatocellular carcinoma, and hepatic tumors other than hepatocellular carcinoma were observed in 37.7%, 29.9%, 18.4%, 33.2%, 3.2%, 6.0%, 0.85%, and 10.0% of patients, respectively. Typical ultrasound findings of FALD included hepatomegaly, hepatic vein dilatation, and splenomegaly, reflecting liver congestion. With the progression of fibrosis, caudate lobe enlargement and splenomegaly became more prominent. Based on these findings, we proposed diagnostic criteria for FALD. Using these criteria, FALD was diagnosed in 1014 (86.8%) of the patients, and all patients with a pathological diagnosis of FALD were successfully identified. Eight patients were found to have pathological cirrhosis, and all were diagnosed with advanced FALD using our criteria based on blood tests or imaging.Conclusion: Our diagnostic criteria facilitate detection of FALD or advanced FALD after the Fontan surgery. The accuracy of these criteria should be further evaluated.","PeriodicalId":12987,"journal":{"name":"Hepatology Research","volume":" ","pages":""},"PeriodicalIF":3.9000,"publicationDate":"2024-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hepatology Research","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/hepr.14141","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Aim: Fontan-associated liver disease (FALD) is a complication after Fontan surgery, and a common cause of liver tumors and cirrhosis. However, no diagnostic criteria for FALD have been established, leading to an underestimation of its prevalence.

Methods: We conducted a national survey to elucidate the characteristics of FALD by collecting data from high-volume centers managing patients who had undergone the Fontan surgery in Japan. In total, 1168 patients were enrolled in the study. First, we examined typical liver findings on ultrasonography after the Fontan surgery. Next, we proposed diagnostic criteria for FALD and advanced FALD based on blood tests, imaging, liver tumors, and pathological examinations. We investigated the sensitivity of histologically diagnosed FALD and advanced FALD based on criteria for blood or imaging tests.

Results: Hepatomegaly, hepatic venous dilatation, caudate lobe enlargement, splenomegaly, liver atrophy, ascites, hepatocellular carcinoma, and hepatic tumors other than hepatocellular carcinoma were observed in 37.7%, 29.9%, 18.4%, 33.2%, 3.2%, 6.0%, 0.85%, and 10.0% of patients, respectively. Typical ultrasound findings of FALD included hepatomegaly, hepatic vein dilatation, and splenomegaly, reflecting liver congestion. With the progression of fibrosis, caudate lobe enlargement and splenomegaly became more prominent. Based on these findings, we proposed diagnostic criteria for FALD. Using these criteria, FALD was diagnosed in 1014 (86.8%) of the patients, and all patients with a pathological diagnosis of FALD were successfully identified. Eight patients were found to have pathological cirrhosis, and all were diagnosed with advanced FALD using our criteria based on blood tests or imaging.

Conclusion: Our diagnostic criteria facilitate detection of FALD or advanced FALD after the Fontan surgery. The accuracy of these criteria should be further evaluated.

查看原文本刊更多论文

评估丰坦相关肝病轻度至晚期的诊断标准：日本全国流行病学调查。

目的：丰坦相关性肝病（FALD）是丰坦手术后的一种并发症，也是肝肿瘤和肝硬化的常见原因。然而，目前尚未建立 FALD 的诊断标准，导致其发病率被低估：我们进行了一项全国性调查，通过收集管理日本接受过丰坦手术患者的高容量中心的数据来阐明 FALD 的特征。共有1168名患者参与了这项研究。首先，我们研究了丰坦手术后典型的肝脏超声检查结果。然后，我们根据血液检查、影像学检查、肝脏肿瘤和病理学检查提出了FALD和晚期FALD的诊断标准。我们研究了基于血液或影像学检查标准的组织学诊断FALD和晚期FALD的敏感性：结果：分别有 37.7%、29.9%、18.4%、33.2%、3.2%、6.0% 和 10.0%的患者出现肝脏肿大、肝静脉扩张、尾状叶增大、脾脏肿大、肝萎缩、腹水、肝细胞癌和肝细胞癌以外的肝脏肿瘤。FALD 的典型超声波检查结果包括肝脏肿大、肝静脉扩张和脾脏肿大，反映了肝脏充血。随着肝纤维化的进展，尾状叶增大和脾肿大变得更加突出。根据这些发现，我们提出了 FALD 的诊断标准。根据这些标准，1014 例（86.8%）患者被诊断为 FALD，所有病理诊断为 FALD 的患者均被成功鉴定。八名患者被发现患有病理肝硬化，根据我们基于血液检查或影像学检查的标准，他们都被诊断为晚期FALD：结论：我们的诊断标准有助于在丰坦手术后发现 FALD 或晚期 FALD。结论：我们的诊断标准有助于在丰坦手术后发现 FALD 或晚期 FALD，其准确性有待进一步评估。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Hepatology Research 医学-胃肠肝病学

CiteScore

8.30

自引率

14.30%

发文量

124

审稿时长

1 months

期刊介绍： Hepatology Research (formerly International Hepatology Communications) is the official journal of the Japan Society of Hepatology, and publishes original articles, reviews and short comunications dealing with hepatology. Reviews or mini-reviews are especially welcomed from those areas within hepatology undergoing rapid changes. Short communications should contain concise definitive information.