Hepatology Forum最新文献

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a rare autoimmune disease characterized by fibroinflammatory lesions and bile duct strictures, often associated with type 1 autoimmune pancreatitis (AIP). Isolated IgG4-SC, occurring without AIP, is particularly uncommon and can clinically and radiologically mimic hilar cholangiocarcinoma, presenting with jaundice and bile duct strictures. Accurate differentiation between these conditions is essential, as surgical resection is the standard treatment for cholangiocarcinoma, whereas steroid therapy is the first-line treatment for IgG4-SC. This case report discusses a 55-year-old female patient who underwent left hepatectomy due to a hilar bile duct stricture initially suspected to be cholangiocarcinoma but was ultimately diagnosed as isolated IgG4-SC based on postoperative histopathological and immunohistochemical findings. The report highlights the diagnostic challenges of isolated IgG4-SC and emphasizes the importance of integrating histology, imaging, and serology to prevent unnecessary surgical interventions.

Background and aim: The present study compared the long-term efficacy of weak and potent antiviral treatments in patients with hepatitis B virus (HBV)-related cirrhosis.

Materials and methods: A total of 120 patients with HBV-related cirrhosis were enrolled. The primary outcome measure was viral suppression. A secondary outcome measure was to determine the development of decompensation or hepatocellular carcinoma (HCC).

Results: The virological response (VR) was significantly better in patients treated with potent antiviral agents than in those treated with weaker antiviral agents over time (p<0.001). With intention-to-treat, the VR after 1 year, 2 years, 3 years, and 4 years of potent antiviral treatment was 69.7%, 77.0%, 82.2%, and 81.2%, respectively, while the VR with weak antiviral therapies was 50.0%, 41.6%, 37.5%, and 37.5%. HBeAg (Hepatitis B e-Antigen) loss was achieved in 30.4% of HBeAg-positive patients. None of the patients had experienced HBsAg loss while on antiviral treatment. New HCC developed in 10 patients. The cumulative probability of the development of HCC was 2.6% at 1 year, 6.8% at 2 years, and 8.7% at 3 and 5 years of antiviral therapy. MELD scores among patients treated with potent antiviral treatment significantly improved from baseline to week 60 (p=0.006). Antiviral therapies were well tolerated.

Conclusion: Potent antiviral treatment effectively maintained VR in the long-term follow-up of patients with HBV-related cirrhosis. HCC may still develop, albeit at a lower rate in these patients.

Background and aim: The transcription factor MafF is a novel regulator of adipogenesis, but its role in hepatic steatosis remains unclear. This study aimed to explore the impact of MafF on hepatocyte steatosis and its underlying mechanisms.

Materials and methods: A stable MafF-overexpressing cell line was established using lentiviral infection. RT-qPCR and Western blot analysis confirmed MafF expression. Free fatty acid (FFA) or ethanol (ETOH) induction was used to simulate hepatocyte steatosis in non-alcoholic or alcoholic fatty liver disease (NAFLD or AFLD). Cell activity and lipid accumulation were assessed through the CCK-8 assay, Calcein-AM/PI staining, and Oil Red O staining. The changes in lipid metabolism-related gene expression before and after FFA or ETOH treatment were detected using RT-qPCR.

Results: FFA or ETOH induced lipid accumulation in hepatocytes, and overexpression of MafF significantly ameliorated ETOH-induced hepatocyte steatosis but had little effect on FFA-induced hepatocyte steatosis. MafF overexpression significantly reduced the expression of peroxisome proliferator-activated receptor gamma (PPARG), acetyl-CoA carboxylase (ACC), and lipoprotein lipase (LPL) in hepatocytes. Upon FFA induction, control (NC) cells exhibited downregulation of these genes, whereas MafF-overexpressing cells upregulated LPL expression. In contrast, under ETOH treatment, NC cells upregulated these genes, while MafF-overexpressing cells showed downregulation.

Conclusion: This study highlighted the regulation of lipid-related genes by MafF, including PPARG, ACC, and LPL, and its effect on FFA- and ETOH-induced hepatocellular lipid accumulation in distinct ways. MafF showed a more pronounced improvement in ETOH-induced hepatocyte steatosis, providing crucial insights into MafF's role in hepatic lipid metabolism and potential therapeutic strategies for NAFLD and AFLD.

Alcoholic liver disease(ALD) is considered as a growing public health issue with universally increasing disease burden. Various genetic and environmental factors play role in its etiology. ALD recently has become the major indication for Liver Transplantation (LT). Most LT programs select their candidates by adhering to six months of alcohol abstinence policy. Nevertheless, early liver transplantation (ELT) has become a subject of research, both in Europe and the United States, as an effective and lifesaving option among highly selected severe alcohol-associated hepatitis (SAH) patients. ELT is a promising way in the management of ALD, perhaps changing clinical practice for carefully selected patient groups.

The association of dermatomyositis (DM) and autoimmune hepatitis (AIH) is rare and presents a diagnostic and therapeutic challenge. We describe the case of a 36-year-old man with DM diagnosed in 2012 and treated with corticosteroid and methotrexate. The patient achieved total remission 18 months later. In 2022, an AIH was diagnosed (cytolysis, cholestasis, anti-LC1, and anti-SLA antibodies) while DM was in remission. Liver function normalized after two months of treatment with mycophenolate mofetil and corticosteroids. Liver damage in systemic autoimmune diseases can result from viral, iatrogenic, or autoimmune processes. The association between DM and AIH is exceptional and has only been documented in one previous observation. Autoantibodies are essential for diagnosing and managing patients with inflammatory myopathy and AIH. In conclusion, this exceptional association of AIH and DM raises many questions regarding the presence of etiopathogenic links, such as genetic predisposition, autoimmunity disorders, viral infection triggers, or simply a happenstance.

Hepatocellular cancer (HCC) is the most common primary malignant tumor of the liver. The organs that HCC most commonly metastasizes to are the lungs, intra-abdominal lymph nodes, bones, and adrenal glands. Brain metastases have been reported rarely. Herein, we report a 54-year-old female patient who was diagnosed with cryptogenic cirrhosis by liver biopsy in 2010. Solid lesions were detected on radiological examination during follow-up in 2019. The patient's complaints of severe headache, nausea, and vomiting continued during the follow-up, and imaging was performed. A contrast-enhancing lesion, 2 cm in size, was reported in the left half of the clivus on T1 examination after intravenous contrast administration. A biopsy was performed on the mass extending from the clivus to the nasopharynx. The biopsy concluded that it was an HCC metastasis. Intracranial metastases of HCC have been reported very rarely compared to other extrahepatic sites. HCC cases with intracranial metastases have a poor prognosis. Intracranial metastases should be considered in the differential diagnosis in patients presenting with central nervous system findings.

Background and aim: The impact of fluid status changes on liver stiffness measurements (LSM) using transient elastography (TE) in dialysis patients remains unclear. This study aimed to evaluate LSM variations during hemodialysis (HD) and analyze contributing factors.

Materials and methods: A cross-sectional study was conducted on dialysis patients at a tertiary care hospital. TE and bioelectrical impedance analysis were performed at four time points: before dialysis, immediately after, the first day after, and the second day after dialysis. LSM values were compared across these time points.

Results: Seventy patients were enrolled, with two cases showing consistently extremely elevated LSM values exceeding 20 kPa, considered outliers. The mean LSM values were 7.6±7.0 kPa before dialysis, 6.12±2.94 kPa immediately after, 6.64±5.27 kPa on the first day, and 6.94±5.12 kPa on the second day after dialysis. The mean pre-HD LSM was significantly higher than immediately after and on the first day after dialysis, with mean differences of 1.54 kPa (95% CI 0.22-2.86, p=0.02) and 1.02 kPa (95% CI 0.15-1.9, p=0.02), respectively. The ultrafiltration volume positively correlated with the LSM difference pre- and post-HD (r=0.315, p=0.008). Patients with residual fluid overload had significantly higher post-HD LSM compared to euvolemic patients (p=0.003).

Conclusion: LSM values significantly decreased after dialysis and remained lower for up to 24 hours. Transient elastography should preferably be performed within 24 hours post-dialysis when the patient is in a euvolemic state.

Acute immunoallergic hepatitis presents as acute liver injury, often accompanied by nonspecific findings of fever, rash, and abdominal pain, and is often induced by drug ingestion. Allopurinol has been implicated in multiple cases of acute immunoallergic hepatitis. We present the case of a young East Asian male with gout who experienced acute immunoallergic hepatitis, complicated by DRESS syndrome with a severe cutaneous reaction, as a result of allopurinol intake. The patient was positive for the HLA-B58*01 gene, a significant risk factor for developing allopurinol-induced liver injury. The patient's liver injury and skin reaction improved with the administration of IV methylprednisolone, followed by a course of oral prednisone. Our case prompts clinicians to prescribe allopurinol with caution in certain high-risk populations and emphasizes the importance of administering corticosteroids early in such a presentation to avoid long-term liver damage.

Glycogen Storage Disease Type 1b (GSD Type 1b) is predominantly diagnosed in childhood. Rare cases emerging in adulthood present a unique set of clinical challenges, particularly concerning liver lesions. We report a 22-year-old male diagnosed unusually late with GSD Type 1b, underlining the hepatic complexities involved. He initially presented with hepatomegaly and solid nodular lesions in the liver. An abdominal magnetic resonance imaging (MRI) revealed a sizable hepatocellular adenoma (HCA), subsequently removed through surgical segmentectomy. Histopathology confirmed the lesion as a hepatocyte nuclear factor-1 alpha (HNF-1alpha) mutation-positive HCA. Follow-up MRI revealed the persistence of multiple smaller liver nodules, necessitating continued clinical surveillance. Hepatic adenomas are a common complication in GSD Type 1 patients, posing management challenges due to their size, multiplicity, and risk of malignancy. While liver transplantation is a last resort option, it can worsen metabolic control. Sodium-glucose cotransporter-2 (SGLT-2) inhibitors offer a potential alternative for improving glycemic regulation and possibly affecting the adenoma size.