Impact of ursodeoxycholic acid treatment on Fontan-associated liver disease.

Background: Fontan-associated liver disease (FALD) is a type of progressive liver fibrosis that occurs following Fontan surgery and can be complicated by hepatocellular carcinoma (HCC). Established treatments for FALD are lacking. Therefore, we investigated the efficacy of ursodeoxycholic acid (UDCA) in patients with FALD.

Importance of gamma-glutamyl transferase elevation in patients with Fontan-associated liver disease.

Aim: In patients with Fontan-associated liver disease (FALD), gamma-glutamyl transferase (GGT) levels are often elevated, however, its clinical importance is unclear. We investigated the relationship between the clinical course of FALD and GGT levels.

Methods: We enrolled 145 patients with FALD who underwent right-heart catheterization (RHC) and visited our department.

Serum level of full-length connective tissue growth factor reflects liver fibrosis stage in patients with Fontan-associated liver disease.

Background: Chronic liver disease leads to liver fibrosis, and an accurate diagnosis of the fibrosis stage is crucial for medical management. Connective tissue growth factor (CTGF) is produced by endothelial cells and platelets and plays a central role in inducing fibrosis in various organs. In the present study, we tested the validity of measuring the serum levels of two types of CTGF to estimate the biopsy-confirmed liver fibrosis stage.

Analysis of genetic factors associated with fatty liver disease-related hepatocellular carcinoma.

Aim: Single-nucleotide polymorphisms (SNPs) in PNPLA3 and hydroxysteroid 17-beta dehydrogenase 13 (HSD17B13) genes are associated with fatty liver disease (FLD) progression and carcinogenesis. In the present study, we evaluated the characteristics of Japanese FLD patients according to HSD17B13 polymorphisms.

Methods: We enrolled 402 patients who were clinically and pathologically diagnosed with FLD (alcoholic: 63 cases, nonalcoholic: 339 cases) at our hospital in 1990-2018 (228 males; median age: 54.

Outcomes of fatty liver disease with and without metabolic comorbidities and risk factors for mortality.

Background And Aim: As the clinical course of metabolic-associated fatty liver disease (MAFLD) is unclear, we compared the clinical courses of MAFLD and non-alcoholic FLD (NAFLD).

Methods: Asian FLD patients ( = 987) from 1991 to 2021 (biopsy-proven in 939) were enrolled. The patients were divided into NAFLD (N-alone,  = 92), both MAFLD and N (M&N,  = 785), and M-alone ( = 90) groups.

Risk and protective factors of acute kidney injury in decompensated cirrhotic patients with ascites on tolvaptan.

Background And Aim: Acute kidney injury (AKI) is a life-threatening complication of liver cirrhosis. Here, we evaluated the risk factors and characteristics of AKI in cirrhosis.

Patients/methods: This was a single-center retrospective study.

Long-term outcome of Wilson's disease complicated by liver disease.

Background And Aim: Wilson's disease (WD) is a rare inherited disease that causes systemic copper accumulation. This study examined the long-term course of WD patients with liver disease.

Methods: The 12 patients (9 female patients) enrolled in the study had a median age of 28 years (range: 19-57 years) at their first visit to our hospital.

The utility of liver transplantation to treat acute liver failure caused by adult-onset Still's disease: case reports.

Purpose: Adult-onset Still's disease (AOSD) is an inflammatory condition commonly complicated by mild liver dysfunction. However, severe liver failure is rarely reported. We report three cases of severe acute hepatic failure (ALF) associated with AOSD.

Long-term Follow-up of a Patient with Portal Hypertension and Hepatic Failure Due to Hepatic Hydatid Disease.

We observed liver failure with a presumed etiology of echinococcosis in an 89-year-old woman. Our patient had been born and then resided on Rebun Island until she was 12 years old. At 46 years old, she had been referred to our hospital due to right abdominal pain.

A case of Fontan-related hepatocellular carcinoma successfully treated with proton beam therapy.

A 27-year-old woman was admitted to our hospital due to a liver tumor. She had been born late at 41 weeks of gestation and had heterotaxy syndrome, polysplenia, and complete transposition of the great arteries. She underwent percutaneous balloon angioplasty at 5 years of age and the Fontan procedure at 6 years of age.