中华肝脏病杂志最新文献

Hepatolenticular degeneration, also known as Wilson disease (WD), is a type of copper metabolism disorder caused by an ATP7B gene variant, which is manifested by the abnormal accumulation of copper in the liver and other organs, resulting in multisystem damage. This article summarizes the latest research progress, with an emphasis on clinical characteristics, analysis of the optimization of diagnostic technology, and the clinical application of novel copper chelator therapy, as well as the development status and future prospects of gene therapy for WD. Future research should focus on the in-depth analysis of the mechanism, the application of multidimensional precision diagnosis technology, the development of individualized treatment plans, and the development of multicenter clinical trials in order to improve the comprehensive treatment effects and quality of life for patients with WD.

Autoimmune hepatitis (AIH) is a kind of immune-mediated chronic liver disease, and its specific pathogenesis has not yet been fully elucidated. In recent years, the International Autoimmune Hepatitis Expert Group has revised histology and autoantibody evaluation criteria for diagnosing AIH and has clarified the definition of treatment response. The current standard treatment regimen is still glucocorticoids and azathioprine, but novel biological agents offer new therapeutic options for patients with refractory AIH. This article reviews the new progress in the diagnosis and treatment of AIH and explores the current challenges and future research directions.

Autoimmune liver diseases (AILDs) is a group of chronic inflammatory liver diseases mediated by autoimmune disorders, while viral hepatitis is a group of infectious diseases mainly induced by hepatotropic viruses, resulting in liver inflammation and necrotic lesions. A viral infection is a risk factor for AILDs, and the two conditions may coexist. This article provides a review of the diagnosis and treatment of AILDs combined with viral hepatitis in recent years.

Objective: To retrospectively analyze the current status of consultation, clinical characteristics, and treatment status of patients with IgG4-related disease (IgG4-RD) in order to provide assistance and a basis for early and standardized diagnosis and treatment. Methods: IgG4-RD cases admitted to our hospital from June 2015 to October 2023 were collected. The details of patients' basic information, initial symptoms, department visits, laboratory and imaging findings, histopathological examination results, and treatment plans were recorded. A statistical descriptive analysis was performed on the data. Results: A total of 105 patients with IgG4-RD were included, with a median age of 59.0 (18.0, 78.0) years. The main departments visited were clinical immunology and gastroenterology (83.8%, 88/105). The median diagnostic duration was eight months, with a maximum of 300 months, and 33.3% (35/105) of patients needed over one year for diagnosis. 92 cases underwent histopathological examinations and IgG4 staining, with a total positivity rate of 87.0% (80/92). Among these, sixteen cases underwent pathological examination after surgery, with a positivity rate of 100%; the remaining 76 cases out of 92 underwent liver biopsy, with a positivity rate of 76.1%. Out of these, there were 22 cases from the pancreas, 21 from the submaxillary gland, nine from the labial gland, and seven each from the duodenal papilla and liver, with positivity rates of 81.8%, 81.0%, 55.6%, 85.7%, and 85.7%, respectively. Eleven cases (10.5%) with normal serum IgG4 were diagnosed based on multi-organ involvement and pathological results. 94 cases (89.5%) had elevated IgG4, with a predominance of＞2.70 g/L. The median follow-up period for the 87 cases was 14 months. Two cases had poor response, twelve patients relapsed, five cases relapsed without combined drug treatment after surgery, five cases relapsed due to drug withdrawal, and two cases relapsed while tapering off steroids. Conclusions: As a multisystem disease, IgG4-RD still faces the difficulties of time-consuming diagnosis and inappropriate treatment. Therefore, it is necessary to rely on a multidisciplinary collaboration model to improve the awareness level and promote the early and standardized diagnosis and treatment of patients with IgG4-RD.

Objective: To explore the effect of psoas muscle index (PMI) and construct a machine learning model to validate the 180-day prognosis in patients with decompensated liver cirrhosis. Methods: Retrospective data were collected from patients with decompensated liver cirrhosis at Henan Provincial People's Hospital from January 2022 to November 2022. The area of the psoas muscle index (PMI) at the level of the third lumbar vertebra was measured and calculated based on the abdominal X-ray computed tomography images stored in the Eastern China Hospital Information System (HIS). Patients were divided into low PMI and normal PMI groups according to the receiver operating characteristic curve. Patients clinical data and complication status were collected.The general conditions of both groups were compared using a t-test, chi-square test, and Mann-Whitney U test. The Kaplan-Meier method was applied for survival analysis. The outcome variable was 180-day mortality, and variables were selected using Cox and LASSO regression. The dataset was divided into training and testing sets in a 7∶3 ratio. Machine learning algorithms were used to build models in the training set, and model performance was validated by the test set. The model for MELD-Na score was compared with the model for End-Stage Liver Disease score. Results: A total of 298 patients with decompensated liver cirrhosis were included.The MELD scores, Child-Pugh classification, and NRS2002 scores, along with the incidence rate of complications such as ascites, hepatic encephalopathy, infections, and gastrointestinal bleeding, were significantly higher in the low PMI than the normal PMI group, with statistically significant differences (P<0.05). The area under a receiver operating characteristic curve for the extreme gradient boosting model was higher than traditional clinical scores (MELD score 0.658, MELD_Na score 0.719) in the machine learning model. Furthermore, the application of SHAP results model indicated that PMI, hemoglobin, NRS2002 score, direct bilirubin, and blood ammonia were important factors in predicting the prognosis of patients with decompensated liver cirrhosis. Conclusion: A low PMI is closely related to poorer survival rates and the development of complication rates in patients with decompensated liver cirrhosis. The machine learning prediction model based on this construction, especially extreme gradient boosting, has favorable predictive performance, which is superior to the traditional clinical scoring system and can provide patients with the most accurate risk assessment and individualized treatment plan.

Chronic hepatitis B (CHB) can gradually progress to life-threatening diseases such as cirrhosis and hepatocellular carcinoma (HCC). In recent years, with the change in people's lifestyles, the incidence rate of metabolic associated fatty liver disease has been steadily increasing and the patients combined with CHB and MAFLD has significantly surged. However, the impact of MAFLD on patients with CHB in aspects of antiviral response, clinical outcomes, and others is still controversial. This article reviews research progress on the impact of MAFLD with regard to natural course and antiviral treatment response in CHB and the survival rate in combination with CHB and MAFLD so as to provide a certain theoretical reference for prevention, diagnosis, and treatment of this disease.

Drug-induced liver injury (DILI) is an important adverse drug reaction with diverse clinical manifestations. Drug-induced autoimmune-like hepatitis (DI-ALH) is a special type of DILI possessing clinical, serological, and histological features similar to autoimmune hepatitis (AIH). However, there are significant differences between DI-ALH and AIH in terms of treatment plan, course of disease, and prognosis; therefore, differential diagnosis between DI-ALH and AIH is crucial. This article summarizes the epidemiology, pathogenesis, clinical characteristics, diagnosis and differential diagnosis, treatment, and prognosis of DI-ALH and analyzes the existing problems in order to provide guidance for the diagnosis, treatment, and future research direction.

Primary biliary cholangitis (PBC) is a type of autoimmune liver disease characterized by chronic intrahepatic cholestasis. Although portal hypertension is a common complication in patients with cirrhotic PBC, portal hypertension and its related complications can occur in the early stage of the disease, that is, before the cirrhosis onset. Therefore, early identification and long-term management are of great significance to reduce the occurrence of portal hypertension and decompensation events and improve long-term prognosis in patients with PBC. This paper focuses on the epidemiology, pathophysiological mechanism, clinical characteristics, non-invasive diagnosis, and treatment strategies for portal hypertension in early-stage PBC patients.

Copper, as a kind of trace element, is crucial for the physiological functions of various key enzymes in the body, and the liver plays a central role in maintaining copper metabolism. Theoretically, dysfunction in the body's metabolic processes, such as copper absorption, transportation, and excretion, can lead to copper deposition or deficiency in various organs. Wilson's disease's characteristic pathological manifestation is deposition of copper in liver. However, during liver pathological examinations, it has been found in clinical practice that certain patients with non-Wilson's disease and inherited metabolic liver disease may also have copper deposition. This review summarizes the inherited metabolic liver diseases that can cause liver copper deposition, their related pathogenesis, and the differential diagnosis approach from the perspectives of clinical and pathological characteristics.

Metabolic-associated fatty liver disease has become a common chronic liver disease with changes in lifestyle and the increasing prevalence rate of overweight and obesity in adults and even children. The liver synthesizes bile acids via cholesterol metabolism, which are important signaling molecules that modulate and regulate host glucose, lipid metabolism, and immunity. Abnormal bile acid metabolism closely correlates with the occurrence and progression of metabolic-associated fatty liver disease. This article systematically organizes the research of bile acid metabolism in children and adults with metabolic-associated fatty liver disease from the perspective of analyzing bile acid profiles by mass spectrometry detection, and compares the characteristics of bile acid metabolic disorders across different age groups and different developmental stages of disease so as to provide a reference for subsequent research.