Rheumatic diseases are a group of chronic disorders characterized by abnormalities in the immune system， while portal hypertension occurs due to increased blood flow or heightened resistance in the portal venous system or obstruction of hepatic venous outflow. Both rheumatic diseases and their medications can lead to noncirrhotic portal hypertension. The hypercoagulable state associated with rheumatic diseases can result in thrombosis within the portal and hepatic venous systems， and damage to the intrahepatic portal system and hepatic sinusoidal endothelial system can lead to porto-sinusoidal vascular disease and hepatic sinusoidal obstruction syndrome. Moreover， drugs used for the treatment of rheumatic diseases may cause liver parenchymal injury， which further leads to liver fibrosis and cirrhosis， or they may damage the hepatic vascular endothelium and thus cause noncirrhotic portal hypertension. This article elaborates on the mechanisms and characteristics by which common rheumatic diseases and their therapeutic agents lead to portal hypertension， in order to provide insights and assistance for clinical diagnosis， treatment， and follow-up monitoring.

Objective:To analyze the clinical, genetic mutation characteristics, and treatment prognosis of type 2A hereditary hemochromatosis (HH) in China.Methods:Peripheral blood samples and clinical data of patients with primary iron overload were collected through the China Registry of Genetic/Metabolic Liver Disease from June 2015 to November 2023. HH-related genes were detected by Sanger sequencing. Clinical characteristics and gene mutation characteristics of HH patients carrying HJV gene mutations were analyzed.Results:Among the 37 cases with primary iron overload, ten cases (27.0%, 10/37) had detectable HJV gene mutations, which included four homozygous mutations, five compound heterozygous mutations, and one monoheterozygous mutation. p.Q6H and p.C321X (80.0%, 8/10) were the most common mutated sites. The average age of onset was 30.7±14.7 years. The age of diagnosis was 35.7±16.2 years, with male-to-female ratio of 7:3. Ferritin and transferrin saturation were (5 267±905) ng/ml, and 94.3%±1.2%, respectively. Magnetic resonance imaging showed iron overload in the liver, pancreas, and myocardium. Liver biopsy showed diffuse iron deposition within hepatocytes. All ten cases had elevated transaminases; one case (1/10, 10.0%) had liver cirrhosis; four cases (4/10, 40.0%) had heart failure and arrhythmia; five cases (5/10, 50.0%) had diabetes; six cases (6/10, 60.0%) had hypogonadism; six cases (6/10, 60.0%) had skin pigmentation; and six cases (6/10, 60.0%) had fatigue symptoms. All six cases underwent bloodletting therapy, and ferritin levels dropped to about 100 ng/ml. Two cases of oral administration of the iron chelator deferasirox did not meet the ferritin level standard, and one case died from acute heart failure following a confirmed diagnosis during hospitalization.Conclusion:The HJV gene may be one of the main pathogenic genes of HH in China. The p.Q6H and p.C321X mutations were one of the hotspot mutations. The onset age of HJV gene-related HH was between 20 and 30 years old, and their condition was severe. Therefore, early bloodletting treatment can have a favorable outcome.

Hepatolenticular degeneration (HLD) is an autosomal recessive inherited disorder of copper metabolism.The mutations in the ATP7B gene on chromosome 13 leads to the functional defect of ATP7B,which produces pathological deposits of copper in liver,brain,comea and kidney,with diverse clinical manifestations in various forms of liver disease,nervous system disease and corneal disease (Kayser-Fleischer rings).Early diagnosis and proper treatment can improve the prognosis of hepatolenticular degeneration.Conversely,it may progress to end-stage liver disease or severe motor dysfunction,which seriously affects patient quality of life.

OBJECTIVE: The aims of the present study were to explore the occurrence of childhood trauma and importantly to determine the impacts of childhood trauma on psychosocial features in a Chinese sample of young adults. METHODS: A survey was carried out in a group of 555 university students by using Childhood Trauma Questionnaire (CTQ), Self-rating Depression Scale (SDS), Self-rating Anxiety Scale (SAS), Dysfunctional Attitudes Questionnaire (DAS), Eysenck Personality Questionnaire (EPQ), and Social Support Rating Scale (SSRS). The moderate-severe cut-off scores for CTQ were used to calculate the prevalence of childhood trauma, and then psychosocial features were compared between individuals with and without childhood trauma. RESULTS: A proportion of 18.6% of university students had self-reported childhood trauma exposures. Subjects with childhood trauma reported higher scores of SDS, SAS, DAS, and psychoticism and neuroticism dimensions of EPQ (t=4.311–5.551, p < 0.001); while lower scores of SSRS and extraversion dimension of EPQ (t=-4.061– -3.039, p < 0.01). Regression analyses further revealed that scores of SAS and DAS were positively (Adjusted B=0.211–0.230, p < 0.05), while scores of SSRS were negatively (Adjusted B=-0.273– -0.240, p < 0.05) associated with specific CTQ scores. CONCLUSION: Childhood trauma is still a common social and psychological problem. Individuals with childhood trauma show much more depression, anxiety, distorted cognition, personality deficits, and lower levels of social support, which may represent the social and psychological vulnerability for developing psychiatric disorders after childhood trauma experiences.
Anxiety ; Asian Continental Ancestry Group* ; Cognition ; Depression ; Extraversion (Psychology) ; Humans ; Prevalence ; Young Adult*

Objective: To explore the diagnostic values of FibroTouch and FibroScan for liver fibrosis in patients with chronic hepatitis B(CHB). Methods: This study enrolled patients with CHB who was accepted liver biopsy at Beijing Friendship Hospital, Capital Medical University between March 2014 to December 2017. FibroTouch and FibroScan were performed among these patients at same time. Liver stiffness measurement(LSM), optimal cut-off value, receiver operating characteristic(ROC) were compared. Results: In our 103 patients, there were no significantly different between FibroTouch and FibroScan in LSM. The threshold of the optimal cut-off value for FibroTouch and FibroScan were 5.45 versus 5.55 kPa (≥S1), 7.10 versus 6.65 kPa (≥S2), 11.05 versus 9.20 kPa (≥S3), 15.50 versus 15.45 kPa (S4), respectively. The area under the ROC curve for the prediction of the stage1, stage2, stage2, stage 4 of liver fibrosis in these patients were 0.858 versus 0.765 (P=0.54), 0.812 versus 0.801 (P=0.68), 0.863 versus 0.878 (P=0.45), and 1.0 versus 0.99 (P=0.38) respectively. Conclusions FibroTouch and FibmScan have a good consistency in the evaluation of the degree of liver fibrosis in patients with CHB.

Primary biliary cholangitis (PBC) is a chronic cholestatic disease with unknown pathogenesis. Positive anti-mitochondrial antibody has high sensitivity and specificity in the diagnosis of this disease. Ursodeoxycholic acid is mainly used for the treatment of PBC, but 40% of patients have an unsatisfactory biochemical response to this drug. 6-Ethylchenodeoxycholic acid is a new drug approved for the treatment of PBC, and liver transplantation remains the only effective method for the treatment of patients with end-stage PBC.

Primary biliary cholangitis is a chronic intrahepatic cholestatic liver disease and has increasing incidence and prevalence rates, as is reported in the literature. Examination of specific antibodies including serum anti-mitochondrial antibody subtype M2 and liver histopathological examination help to make a definite diagnosis of this disease. Ursodeoxycholic acid (UDCA) is often used for the treatment of this disease, but patients with an unsatisfactory biochemical response to UDCA tend to have rapid disease progression. At present, there are no effective treatment methods for such patients. The addition of budesonide, fenofibrate, bezafibrate, or obeticholic acid may be effective in these patients, but this needs to be verified by further clinical studies.

Objective To investigate the protective effect of augmenter of liver regeneration (ALR) against acute liver injury and related mechanisms.Methods HL-7702 cells were divided into normal control group,carbon tetrachloride (CCl4)-induced acute liver injury group,ALR+CCl4 intervention group,3-methyladerine (3-MA)+CCl4 intervention group,and ALR+3-MA+CCl4 intervention group.The ALR+CCl4 and ALR+3-MA+CCl4 intervention groups were transfected with ALR plasmids at 8 hours before CCl4 treatment.All groups except the normal control group were treated with CC14,and 30 minutes later,the 3-MA+CC14 and ALR+3-MA+CCl4 intervention groups were treated with 3-MA.The cells were collected at 24 hours after CCl4 treatment.The HL-7702 cells and supematant were collected to measure the levels of alanine aminotransferase (ALT) and aspartate aminotransferase (AST) (IU/L).Westem blot was used to measure the levels ofALR,cyclin D,cyclin E,proliferating cell nuclear antigen (PCNA),autophagy-related gene 7 (Atg7),and autophagy genes LC3,p62,and Beclin-1.Quantitative real-time PCR was used to measure the mRNA expression ofALR.A oneway analysis of variance was used for comparison of means between any two groups.Results The ALR+CCl4 intervention group had significant increases in the protein and mRNA expression of ALR compared with the acute liver injury group (both P ＜ 0.05).The CC14-induced acute liver injury group had significant increases in the protein and mRNA expression of ALR compared with the normal control group (both P ＜ 0.05).Compared with the CCl4-induced acute liver injury group,the ALR+CCl4 intervention group had significant reductions in ALT (0.73±0.17 IU/L vs 1.43±0.38 IU/L,P ＜ 0.05) and AST (19.85±1.83 IU/L vs 56.73±6.25 IU/L,P ＜ 0.05) in supematant,significantly increased expression of cyclin D,cyclin E,PCNA,LC3,Atg7,and Beclin-1 in hepatocytes,and significantly reduced expression of p62,which suggested that ALR protected the liver against acute liver injury,promoted the regeneration of hepatocytes,and enhanced the autophagy of hepatocytes.The ALR+3-MA+CCl4 intervention group had a significant reduction in the expression of regeneration-associated proteins compared with the ALR+CCl4 intervention group,while there was no significant difference between the ALR+3-MA+CCL4 intervention group and 3-MA+CCL4 intervention group,which suggested that after the inhibition of autophagy,there were significant reductions in the regeneration of hepatocytes and liver regeneration promoted by ALR.Conclusion ALR can promote the regeneration of hepatocytes in liver parenchyma,which is achieved by the regulation of autophagy.

OBJECTIVETo perform a comparative assessment of the performance of FibroTouch and FibroScan in patients with hepatitis B.

METHODSA total of 211 patients with hepatitis B, including cases of chronic hepatitis B (CHB) and of compensated cirrhosis, were enrolled for study between June and November of 2013. The patients underwent FibroScan testing (group 1) and FibroTouch testing (group 3), after which the operator examined a time motion ultrasound image from the FibroScan test and located a specific liver portion for focused FibroTouch testing (group 2). The consistency between the two tests' results was investigated by Pearson's correlation analysis, and the difference of liver stiffness between CHB patients and compensated cirrhosis patients was investigated by the two independent samples t-test or Mann-Whitney U test.

RESULTSThe values of liver stiffness were 5.30 (4.30,8.65) in group 1,6.10 (4.70,8.90) in group 2, and 5.70 (4.50, 8.00) in group 3 (all P < 0.05); the Pearson correlation coefficients were all more than 0.8 (P < 0.05) and there was no statistically significant difference found between the results from FibroScan and FibroTouch.The values of liver stiffness were significantly different between the CHB patients and the compensated cirrhosis patients (P < 0.05). The rates of successful detection were 100% for FibroTouch and 97% for FibroScan.

CONCLUSIONFibroTouch and FibroScan have good consistency in the evaluation of the degree of liver fibrosis. FibroTouch has a higher rate of successful detection than FibroScan.

Elasticity Imaging Techniques ; Hepatitis B, Chronic ; pathology ; Humans ; Liver Cirrhosis ; diagnosis

OBJECTIVETo investigate noise hazard and its influence on hearing loss in workers in the automotive component manufacturing industry.

METHODSNoise level in the workplace of automotive component manufacturing enterprises was measured and hearing examination was performed for workers to analyze the features and exposure levels of noise in each process, as well as the influence on hearing loss in workers.

RESULTSIn the manufacturing processes for different products in this industry, the manufacturing processes of automobile hub and suspension and steering systems had the highest degrees of noise hazard, with over-standard rates of 79.8% and 57.1%, respectively. In the different technical processes for automotive component manufacturing, punching and casting had the highest degrees of noise hazard, with over-standard rates of 65.0% and 50%, respectively. The workers engaged in the automotive air conditioning system had the highest rate of abnormal hearing ability (up to 3.1%).

CONCLUSIONIn the automotive component manufacturing industry, noise hazard exceeds the standard seriously. Although the rate of abnormal hearing is lower than the average value of the automobile manufacturing industry in China, this rate tends to increase gradually. Enough emphasis should be placed on the noise hazard in this industry.

Automobiles ; China ; Hearing Loss, Noise-Induced ; epidemiology ; Hearing Tests ; Humans ; Manufacturing Industry ; Noise, Occupational ; adverse effects ; Occupational Exposure ; Occupations ; Workplace