中华肝脏病杂志最新文献

{"title":"[Controversy and consensus on antiviral treatment strategies for low-level hepatitis B surface antigens].","authors":"Y H Hou, L Y Zhang","doi":"10.3760/cma.j.cn501113-20250427-00161","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20250427-00161","url":null,"abstract":"<p><p>Hepatitis B virus infection is a global public health issue, and there exist many controversies about the antiviral treatment strategies for patients with low-level hepatitis B surface antigen. This article reviews the definition, clinical significance, and current controversial focus of treatment strategies for low-level hepatitis B surface antigen, including treatment timing, drug selection, treatment course determination, and other aspects, while also suggesting future research directions.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 6","pages":"521-525"},"PeriodicalIF":0.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144638247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Effects of PEG-IFN-α treatment on the expression of CD⁺161 and PD-1 in CD8⁺ T cells of patients with chronic hepatitis B].","authors":"Y P Li, C R Liu, X Zhang, N Huang, W Zhang, L Yang, S S Dang","doi":"10.3760/cma.j.cn501113-20240921-00501","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20240921-00501","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To investigate and explore the expressional condition and therapeutic role of PD-1 and CD161 in the peripheral blood of patients treated with PEG-IFN-α for chronic hepatitis B (CHB), and their correlation with the degree of decrease in hepatitis B surface antigen (HBsAg). &lt;b&gt;Methods:&lt;/b&gt; A retrospective cohort study was conducted. CHB patients who visited the Second Affiliated Hospital of Xi'an Jiaotong University from July 2022 to December 2023 and healthy controls during the same period were included. Peripheral blood samples were collected from the IFN treatment group (31 cases), the non-IFN treatment group (30 cases), and the healthy control group (30 cases). Flow cytometry was used to detect the CD8,&lt;sup&gt;+&lt;/sup&gt; PD-1,&lt;sup&gt;+&lt;/sup&gt; CD161&lt;sup&gt;+&lt;/sup&gt; T lymphocytes and their subpopulations among the three groups. The proportions of cellular subpopulations were compared to analyze intergroup differences using Kruskal-Wallis and Mann-Whitney &lt;i&gt;U&lt;/i&gt; tests. The patients in the IFN treatment group were divided into two subgroups, high-and low-level, according to the median levels of PD-1&lt;sup&gt;+&lt;/sup&gt; lymphocytes, CD8&lt;sup&gt;+&lt;/sup&gt;PD-1&lt;sup&gt;+&lt;/sup&gt;T cells, and CD161&lt;sup&gt;+&lt;/sup&gt; lymphocytes. The magnitude of HBsAg decline was compared between the two groups. &lt;b&gt;Results:&lt;/b&gt; The proportions of PD-1&lt;sup&gt;+&lt;/sup&gt; lymphocytes and CD8&lt;sup&gt;+&lt;/sup&gt;PD-1&lt;sup&gt;+&lt;/sup&gt;T cells in the IFN treatment group were significantly higher than those in the healthy control group and the non-IFN treatment group (&lt;i&gt;P&lt;/i&gt;&lt;0.001). Moreover, the proportions of PD-1&lt;sup&gt;+&lt;/sup&gt; lymphocytes [IFN treatment group 48 weeks: 24.3 (23.7, 28.0)%, non-IFN treatment group: 12.7 (10.0, 18.5)%, &lt;i&gt;P&lt;/i&gt;&lt;0.01] and CD8&lt;sup&gt;+&lt;/sup&gt;PD-1&lt;sup&gt;+&lt;/sup&gt;T cells [IFN treatment group 48 weeks: 29.29 (26.73, 32.98)%, non-IFN treatment group: 17.69 (9.62, 20.68)%, &lt;i&gt;P&lt;/i&gt;&lt;0.05] were higher in the IFN treatment group than those in the non-IFN treatment group at 48 weeks. The proportion of CD8&lt;sup&gt;+&lt;/sup&gt;CD161&lt;sup&gt;+&lt;/sup&gt;T cells was significantly lower in patients treated with IFN than in the non-IFN treatment group (&lt;i&gt;P&lt;/i&gt;&lt;0.05) at 24 and 48 weeks, with no statistically significant difference with the healthy control group (&lt;i&gt;P&lt;/i&gt;&gt;0.05). In the IFN treatment group, patients with high levels of PD-1&lt;sup&gt;+&lt;/sup&gt; and CD8&lt;sup&gt;+&lt;/sup&gt; PD1 lymphocytes had a significantly lower HBsAg decline compared to low-level patients, whereas no significant correlation was found between CD161 levels and HBsAg decline [PD-1&lt;sup&gt;+&lt;/sup&gt; lymphocytes: 0.15 (0.02, 0.18) log&lt;sub&gt;10&lt;/sub&gt; IU/mL &lt;i&gt;vs&lt;/i&gt;. 0.32 (0.13, 0.42) log&lt;sub&gt;10&lt;/sub&gt; IU/mL, &lt;i&gt;P&lt;/i&gt;&lt;0.01; CD8&lt;sup&gt;+&lt;/sup&gt;PD-1&lt;sup&gt;+&lt;/sup&gt;T cells: 0.16 (0.03, 0.17) log&lt;sub&gt;10&lt;/sub&gt; IU/mL &lt;i&gt;vs&lt;/i&gt;. 0.34 (0.13, 0.44) log&lt;sub&gt;10&lt;/sub&gt; IU/mL, &lt;i&gt;P&lt;/i&gt;&lt;0.05]. &lt;b&gt;Conclusion:&lt;/b&gt; The proportions of CD8&lt;sup&gt;+&lt;/sup&gt;PD-1&lt;sup&gt;+&lt;/sup&gt;T cells and CD8&lt;sup&gt;+&lt;/sup&gt;CD161&lt;sup&gt;+&lt;/sup&gt;T cells were significantly regulated by PEG-IFN-α therapy in the peripheral blood of pat","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 6","pages":"570-576"},"PeriodicalIF":0.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144638251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Current status of interventional therapy for hepatic encephalopathy associated with spontaneous portosystemic shunts].","authors":"Z H Cai, Q Ke, Y B Jiao, X T Weng, J He, X H Huang, L Li, W H Guo","doi":"10.3760/cma.j.cn501113-20241116-00583","DOIUrl":"10.3760/cma.j.cn501113-20241116-00583","url":null,"abstract":"<p><p>Hepatic encephalopathy (HE) is a kind of neuropsychiatric syndrome caused by acute or chronic liver failure or portosystemic venous shunt (hereinafter referred to as portosystemic shunt), which can lead to the occurrence of functional impairment, personality and behavioral abnormalities, coma, and even death. Most patients with cirrhosis combined with HE have spontaneous portosystemic shunt (SPSS), especially those with recurrent or persistent HE. Internal medicine's current standard of treatment for HE associated with SPSS in cirrhotic patients is unsatisfactory, and even after treatment, recurrent HE episodes may still occur. Although interventional therapy has shown significant results and has been applied in clinical practice for many years for SPSS-associated HE, the number of treatment-related cases is relatively small, and there is a lack of large samples and well-designed research. Currently, interventional therapy for SPSS-associated HE in patients with cirrhosis is still under continuous exploration.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 6","pages":"595-600"},"PeriodicalIF":0.0,"publicationDate":"2025-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144638249","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Evaluation of the short-term prognosis of patients with HBV-related acute-on-chronic liver failure by combining ferritin with COSSH-ACLF II score].","authors":"Y X Liu, Z Y Li, L Y Ma, Y F Gao, Y Wang, C Y Zhao","doi":"10.3760/cma.j.cn501113-20240603-00282","DOIUrl":"10.3760/cma.j.cn501113-20240603-00282","url":null,"abstract":"<p><p><b>Objective:</b> To explore the predictive value of ferritin combined with the COSSH-ACLF Ⅱ score for the prognosis of patients with hepatitis B virus (HBV)-related acute-on-chronic liver failure (HBV-ACLF). <b>Methods:</b> The clinical data of 419 cases with HBV-ACLF hospitalized at the Third Hospital of Hebei Medical University were retrospectively analyzed between January 1, 2013 and September 30, 2022, and were divided into the death (<i>n</i>=127) and survival group (<i>n</i>=292) according to the survival status of 28 days of follow-up. The Mann-Whitney U test was used to compare confirmation of non-normally distributed continuous data between two groups. The chi-square test was used for the comparison of numerical data between the two groups. Binary logistic regression analysis was used to analyze the independent risk factors affecting the prognosis of HBV-ACLF patients. The predictive value of ferritin combined with the COSSH-ACLF Ⅱ score on the prognosis of HBV-ACLF was evaluated by the receiver operating characteristic curve (ROC curve) and area under the curve (AUC), net reclassification index (NRI), and comprehensive discriminant improvement index (IDI). <b>Results:</b> There were statistically significant differences in age, neutrophil count (NEUT), alanine aminotransferase (ALT), aspartate aminotransferase (AST), total bilirubin (TBil), serum creatinine (Scr), serum urea, prothrombin time (PT), prothrombin activity (PTA), international normalized ratio (INR), serum ferritin (SF), hepatic encephalopathy, and COSSH-ACLF Ⅱ scores between the two groups (<i>P</i><0.05). Ferritin (<i>OR</i>=1.001, 95%<i>CI</i>:1.001-1.002, <i>P</i><0.001) and COSSH-ACLF Ⅱ score (<i>OR</i>=2.898, 95%<i>CI</i>:1.560-5.384, <i>P</i><0.001) were independent factors for predicting short-term prognosis for patients with HBV-ACLF. Ferritin combined with COSSH-ACLF II score had a higher prognostic predictive value than ferritin (AUC=0.697, 95%<i>CI</i>: 0.651-0.741) and COSSH-ACLF II score (AUC=0.819, 95% <i>CI</i>: 0.779-0.855) for patients with HBV-ACLF (AUC=0.857, 95%<i>CI</i>: 0.819-0.889), with a statistically significant difference (<i>Z</i>=6.287 and 2.666, respectively, <i>P</i> <0.05). The predictive effect was significantly improved following the addition of ferritin to the COSSH-ACLF Ⅱ score (<i>P</i><0.001), and the NRI and IDI were both >0 (NRI=0.144, 95%<i>CI</i>: 0.064-0.225; IDI=0.080, 95%<i>CI</i>: 0.052-0.108). <b>Conclusion:</b> Ferritin and COSSH-ACLF Ⅱ scores are independent factors that can predict short-term prognosis for patients with HBV-ACLF, and combing both has a higher predictive value.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 5","pages":"456-462"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144318135","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Paradoxical roles of spontaneous portosystemic shunts and their management strategy in splenectomy with devascularization].","authors":"Y Bu, M G Tian","doi":"10.3760/cma.j.cn501113-20250505-00171","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20250505-00171","url":null,"abstract":"<p><p>Spontaneous portosystemic shunt (SPSS) is an important pathological feature in the progression of cirrhotic portal hypertension and acts as the body's compensatory mechanism for reducing portal hypertension. Recent research results show that SPSS is an important link in the development of cirrhosis into the decompensated stage and is also the main cause of hepatic encephalopathy after splenectomy combined with devascularization. Therefore, during splenectomy combined with devascularization, it is necessary to decide on treatment measures such as disconnection, flow limitation, or retention of the distal side as a selective shunt channel according to its location and shunt volume.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 5","pages":"416-421"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144318138","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Comparison of clinical characteristics between patients with Polygonum multiflorum-induced liver injury and those with other drug-induced liver injuries].","authors":"K A Tan, W N Yang, Y W Qiu, X Z Liu, X W Sun, L L Pang, F Q Hou","doi":"10.3760/cma.j.cn501113-20250216-00058","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20250216-00058","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To compare the clinical characteristics of patients with drug-induced liver injury (DILI) caused by Polygonum multiflorum and other drug-induced liver injuries (DILI). &lt;b&gt;Methods:&lt;/b&gt; A retrospective cohort study was conducted. Clinical data of seventy-three cases confirmedly diagnosed with DILI caused by Polygonum multiflorum, 168 cases diagnosed with DILI caused by other traditional Chinese medicines, and 225 cases diagnosed with DILI caused by modern medicines admitted to Peking University First Hospital, the Fipth People's Hospital of Wuxi, Yantai Qishan Hospital, and Qinhuangdao Third Hospital from January 1995 to August 2019 were selected and collected as the research subjects. The Mann-Whitney &lt;i&gt;U&lt;/i&gt; test was used for comparison of skewed distribution of continuous data between two groups. The Kruskal-Wallis rank-sum test was used for comparison between three groups. The &lt;i&gt;χ&lt;/i&gt;&lt;sup&gt;2&lt;/sup&gt; test was used for comparing count data between groups. &lt;b&gt;Results:&lt;/b&gt; Among the 73 cases with DILI caused by Polygonum multiflorum, 11 (15.1%) took a single herb of Polygonum multiflorum (including its powder and boiled water), 37 (50.7%) took traditional Chinese patent medicines containing Polygonum multiflorum, and 25 (34.2%) took a traditional Chinese medicine formula containing Polygonum multiflorum. The age of the DILI group caused by Polygonum multiflorum was 48 years old, which was lower than the other two groups (the DILI group caused by other traditional Chinese medicines: 55 years old, the DILI group caused by modern medicines: 52 years old; &lt;i&gt;P&lt;/i&gt;&lt;0.01). The levels of alanine aminotransferase (ALT), aspartate aminotransferase, and alkaline phosphatase were all higher than the other two groups (&lt;i&gt;P&lt;/i&gt;&lt;0.05). The proportion of patients with antinuclear antibody positivity rate and severity of liver damage grade 3 was higher in the DILI group induced by Polygonum multiflorum than those in the modern drug-induced DILI group (&lt;i&gt;P&lt;/i&gt;&lt;0.05). The liver cell injury type accounted for 96.6% (57/59) in the DILI group caused by Polygonum multiflorum, which was higher than that in the modern drug-induced DILI group (69.3%, 156/225) (&lt;i&gt;P&lt;/i&gt;&lt;0.001). There was no statistically significant difference (&lt;i&gt;P&lt;/i&gt;&gt;0.05) in gender, age, medication duration, and various biochemical indicators between patients with DILI caused by Polygonum multiflorum monotherapy and compound preparations in terms of compatibility. The ALT level in the DILI group caused by raw Polygonum multiflorum was higher than that in the DILI group caused by processed Polygonum multiflorum [the DILI group caused by raw Polygonum multiflorum: 1 289.0(921.8, 1 851.8)U/L, the DILI group caused by processed Polygonum multiflorum: 890.0(304.0,1 320.0)U/L;&lt;i&gt;P&lt;/i&gt;&lt;0.05] according to the comparison of processing methods. &lt;b&gt;Conclusion:&lt;/b&gt; The degree of DILI caused by Polygonum multiflorum is more obvious than that caused by other drugs. There was no difference in t","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 5","pages":"463-469"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144318134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[The evaluation value of mNUTRIC and NRS-2002 scores in assessing nutritional status and clinical outcomes in patients with end-stage liver disease].","authors":"J Y Yang, X R Mao, Z H Yang, X J Zhou, X Gou, J F Li","doi":"10.3760/cma.j.cn501113-20240723-00344","DOIUrl":"10.3760/cma.j.cn501113-20240723-00344","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; Comparative analysis of the mNUTRIC and NRS-2002 scores for evaluating nutritional risk and predicting clinical outcomes in end stage liver disease patients. &lt;b&gt;Method:&lt;/b&gt; A retrospective cohort study method was used to screen 114 cases with end-stage liver disease admitted to the intensive care unit (ICU) of the First Hospital of Lanzhou University from December 1, 2016 to March 31, 2021 according to the inclusion and exclusion criteria. The patient's demographic data, blood routine, blood biochemical indexes, coagulation function indexes, arterial blood gas analysis and imaging examination data were collected. The mNUTRIC score, NRS-2002 score, sequential organ failure (SOFA) score, model for end-stage liver disease (MELD) score, acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) score, Child-Pugh grade, and clinical outcomes at 28 and 90 days at 24 h post-ICU admission were collected. The differences in clinical indicators between the mNUTRIC high group (≥5 points) and the low group, and the NRS-2002 high group (≥3 points) and the low group were compared. Spearman correlation analysis was used to explore the correlation between the mNUTRIC score and NRS-2002 score, clinical indicators, and 28 and 90-day mortality rates. Multivariate logistic regression analysis was used to determine the risk factors associated with 28-day and 90-day mortality in patients. The value of mNUTRIC score and NRS-2002 score in assessing the clinical outcomes of patients with end-stage liver disease was explored by receiver operating characteristic (ROC) curve. &lt;b&gt;Results:&lt;/b&gt; The clinical indicators related to nutritional status of patients were worse in the high-mNUTRIC group than those in the low-mNUTRIC group, and the 28-day and 90-day mortality rates were significantly higher than those in the low-mNUTRIC group [89.0%(65/73) vs. 29.2%(12/41), 97.2%(71/73) vs. 39.0%(16/41), &lt;i&gt;P&lt;/i&gt;&lt;0.001]. There was no statistically significant difference in the incidence rate of hepatic encephalopathy, esophageal variceal bleeding, and ascites between the high and low mNUTRIC group. The clinical indicators related to nutritional status were worse in the high-NRS-2002 group than those in the low-NRS-2002 group of patients, and the 28-day and 90-day mortality rates were significantly higher than those in the low-group [73.0%(73/100) vs. 4/14, 81.0%(81/100) vs. 6/14, &lt;i&gt;P&lt;/i&gt;=0.008, 0.004]. The NRS-2002 high-score group did not differ significantly from the low-score group in terms of hepatic encephalopathy, esophagogastric variceal bleeding, or ascites prevalence. Patient's age, white blood cell count (WBC), urea nitrogen (BUN), creatinine (UREA), uric acid (UA), total cholesterol (TG), Child-Pugh, MELD, SOFA, APACHE Ⅱscores were significantly positively correlated with the mNUTRIC score. Conversely, albumin (Alb) and Glasgow Coma Scale (GCS) were significantly negatively correlated. Patient's age, WBC, CREA, BUN, UREA, UA, Child-Pugh, MELD, SOFA,","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 ","pages":"470-480"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144002829","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical and molecular genetic analysis of nine patients with neonatal Dubin-Johnson syndrome].","authors":"T Xu, D Li, L Guo, M Deng, W X Lin, Y Z Song","doi":"10.3760/cma.j.cn501113-20241026-00558","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20241026-00558","url":null,"abstract":"<p><p><b>Objective:</b> Dubin-Johnson syndrome (DJS) is a hereditary liver disease caused by biallelic pathogenic variants in the <i>ABCC2</i> gene. As a rare disease, the <i>ABCC2</i> genotype and clinical phenotype characteristics of DJS patients still need to be summarized in depth. <b>Methods:</b> Nine cases diagnosed with DJS and treated in the Department of Pediatrics of the First Affiliated Hospital of Jinan University were collected as the study subjects. Clinical and laboratory data, general information, symptoms, signs, pathological changes, treatment, and prognostic conditions were systematically analyzed. Targeted high-throughput sequencing was used to detect hereditary diseases. The positive results for the family lineage were verified by Sanger sequencing. The pathogenicity of the novel <i>ABCC2</i> variants was evaluated according to the American College of Medical Genetics and Genomics guidelines and standards. One-way analysis of variance or Kruskal-Wallis test was used to compare the statistical differences between multiple groups of data. <b>Results:</b> Among the nine DJS cases, seven and two were males, and females. All of them had the initial symptom of jaundice (100%), with a median age of onset of 5 (2,15) days. During the course of the disease, seven (7/9) and two (2/9) cases had hepatomegaly and splenomegaly. All of the patients exhibited direct hyperbilirubinemia, concurrently with elevated total bile acids (TBA) and γ-glutamyl transferase (GGT). Serum transaminases (4/9) and alkaline phosphatase levels (3/9) were elevated in some patients. A total of twelve types of <i>ABCC2</i> variants were detected in nine cases, of which c.2362_2363del (p.Leu788ValfsTer13), c.364C>T (p.Gln122Ter), c.338T>C (p.Leu113Pro) and c.419T>A (p.Ile140Lys) were novel pathogenic/likely pathogenic variants. Jaundice disappeared and alleviated in five cases (5/9) and four cases (4/9), while hepatomegaly improved in five cases (5/9) at the last follow-up at 7.79 (7.0,15.25) months following treatment with drugs such as liver protectives, choleretics, and jaundice-reducing agents. Among them, three cases (3/9) had a normal restored liver size. All patients had varying degrees of improvement in bilirubin, TBA, GGT, and ALP levels. <b>Conclusions:</b> The onset of high GGT cholestatic jaundice is the main clinical manifestation in patients with neonatal DJS. The genetic analysis results showed four novel types of variants, which expanded the <i>ABCC2</i> gene variation spectrum, providing novel molecular markers for confirming a diagnosis of DJS. The patient's clinical manifestations and laboratory abnormalities improved or disappeared after internal medicine treatment, suggesting that DJS may be a type of genetic disease with a favorable long-term prognosis.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 5","pages":"481-488"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144318133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Guideline for diagnosis and management of metabolic dysfunction-associated fatty liver disease in primary care (2025)].","authors":"","doi":"10.3760/cma.j.cn501113-20250305-00073","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20250305-00073","url":null,"abstract":"","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 5","pages":"422-433"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144318137","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[The impact of spontaneous portosystemic shunt on clinical outcomes in patients with liver cirrhosis and hepatic encephalopathy].","authors":"Q Ke, T Lin, X J Lei, X T Weng, J He, X H Huang, L Li, W H Guo","doi":"10.3760/cma.j.cn501113-20240917-00495","DOIUrl":"https://doi.org/10.3760/cma.j.cn501113-20240917-00495","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the incidence, clinical characteristics, and impact of spontaneous portosystemic shunt (SPSS) in patients with liver cirrhosis combined with hepatic encephalopathy (HE). <b>Methods:</b> The basic clinical and follow-up data were retrospectively analyzed for patients diagnosed with cirrhosis combined with HE at Mengchao Hepatobiliary Hospital of Fujian Medical University from January 2017 to December 2022. The patients were divided into large and small SPSS groups and a control group based on the results of abdominal enhanced CT or MRI.The clinical characteristics and outcome differences were compared among the three groups. Kaplan-Meier survival curves were used to compare HE-free survival time and overall survival time among the three groups. The log-rank test was used to compare the differences between groups. Cox regression analysis was used to identify the relevant risk factors affecting HE-free survival time and overall survival time. <b>Results:</b> A total of 223 cases with liver cirrhosis combined with HE were enrolled, including 150 in the SPSS and 73 in the control groups. The incidence rate of SPSS was 67.3% (150/223). The group was divided into small SPSS (79/150, 52.7%) and large SPSS group (71/150, 47.3%) according to the cross-sectional area of the diversion channel. The HE-free survival was shorter in the small and large SPSS groups compared with the control group (35.5 months in the small SPSS group and 21.3 months in the large SPSS group; <i>P</i><0.001). The HE-free survival time was shorter in the large SPSS than with small SPSS group (<i>P</i>=0.003). The overall survival time in the small SPSS group and the large SPSS group was shorter compared with the control group (small SPSS group: 39.4 months, large SPSS group: 52.9 months; <i>P</i><0.001). There was no statistically significant difference in overall survival time between the small SPSS and large SPSS groups (<i>P</i>=0.700). Cox regression analysis showed that SPSS was an independent risk factor affecting patients' HE-free survival time and overall survival time (<i>P</i><0.05). <b>Conclusion:</b> SPSS is more common in patients with liver cirrhosis combined with HE. Patients who combined with SPSS showed significant reductions in both HE-free survival time and overall survival time, especially evident in those with combined large SPSS.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 5","pages":"440-447"},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144318143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}