[Epidemiological characteristics of inpatients with liver failure at the Beijing You'an Hospital from 2012 to 2021].

Objective: To elucidate the epidemiological characteristics and changing trends of liver failure in order to provide evidence-based strategies for prevention and treatment. Methods: The epidemiological information of inpatients with liver failure admitted and treated at Beijing You'an Hospital from 2012 to 2021 was retrospectively collected. The trend test was used to analyze age, gender, as well as the year-by-year changes in the underlying acute and chronic etiology of acute liver failure (ALF), sub-acute liver failure (SALF), acute-on-chronic liver failure (ACLF), and chronic liver failure (CLF). Results: During the study period, information on a total of 8512 inpatients, aged 51.3±13.5 years and mainly male (71.9%) with liver failure, was collected. The highest to lowest proportions of liver failure types were ACLF 4 023 (47.3%), CLF 3 571(42.0%), SALF 670 (7.9%), and ALF 248 (2.9%). The top five causes of liver failure in the overall population, accounting for 87.6% of the total, were hepatitis B 3 199 (37.58%), alcoholic liver disease 2 237 (26.28%), cryptogenic liver disease 906(10.61%), hepatitis B + alcoholic liver disease 603 (7.08%), drugs 488 (5.73%), The top three etiologies of patients with different types of liver failure were acute etiologies for acute liver failure (ALF), followed by drugs 107 (43.1%), hepatitis B 47(19.0%), and unknown etiology 36 (14.5%); sub-acute liver failure (SALF), followed by drugs 381(56.9%), unknown etiology 106 (15.8%), and sepsis 56 (8.4%); and acute-on-chronic liver failure (ACLF), followed by drugs 2 092(52.0%), alcoholic liver disease 813(20.2%), and cryptogenic liver disease 398(9.9%); and chronic etiologies for chronic liver failure (CLF), followed by alcoholic liver disease 1 410(39.5%), hepatitis B 1 028(28.8%), and cryptogenic liver disease 364(10.2%). Longitudinal analysis showed that the average age of patients with liver failure increased year by year, but the sex ratio trend did not change significantly, with male patients predominating throughout. The proportion of drug-induced liver failure in patients with ALF and SALF increased year by year, and the difference in the trend test was statistically significant (P < 0.05). The proportion of patients with chronic etiologies of ACLF and CLF decreased year by year among hepatitis B, while the proportion of alcoholic liver disease, autoimmune liver disease, and cryptogenic liver disease increased year by year (the difference was statistically significant, P < 0.05). Conclusion: The etiological spectrum of liver failure is changing in our country. Although hepatitis B is still the main cause of liver failure, its proportion shows a decreasing trend year by year, with the exception of ACLF, which is no longer the primary etiology of other types of liver failure, while drug-induced liver disease, alcoholic liver disease, autoimmune liver disease, and cryptogenic liver disease are increasing year by year and will become the focus of liver disease prevention and treatment in the future.

[Study on evaluation method of circular small shadow profusion in chest CT reconstruction images of pneumoconiosis].

Objective: To select chest CT image patterns for the diagnosis of pneumoconiosis and establish a method for determining the profusion of circular small shadows in chest CT. Methods: In April 2021, 66 cases of occupational pneumoconiosis patients with digital radiography (DR) chest radiographs and chest CT imaging data with circular small shadow as the main manifestations were selected as the study objects. 1.5 mm and 5 mm chest CT axial images, 1 mm and 5 mm chest CT coronal multi-plane recombination (MPR) images, and 5 mm chest CT coronal maximum intensity projection (MIP) images were used to observe the different characteristics of pneumoconiosis patients, and were compared and analyzed with DR chest radiographs to establish the experimental chest CT standards. The consistency of the profusion results between the experimental chest CT standards and GBZ 70-2015 Diagnosis of Occupational Pneumoconiosis was verified. Results: All the 66 objects were male, including 33 cases of stage Ⅰ pneumoconiosis, 17 cases of stage Ⅱ pneumoconiosis and 16 cases of stage Ⅲ pneumoconiosis. By observing five chest CT images of 66 objects, we found that chest CT images of different modes could clearly display and identify abnormal images such as small circular shadow, large shadow, small shadow aggregation, honeycomb glass shadow, flake glass shadow, uniform low-profusion glass shadow, mesh glass shadow, cable shadow, linear shadow, subpleural spinous shadow, subpleural nodules, various kinds of emphysema and lung texture distortion and fracture. Small shadow aggregation was usually accompanied by the appearance of large shadow. The vascular shadows in 5 mm CT images had good ductility, and small nodules were easy to distinguish. The coronal MIP image of 5 mm chest CT used edge enhancement technology, which was prone to small shadow fusion and fibrotic shadow fusion. The coronal MPR image of 5 mm chest CT was highly consistent with the DR chest radiographs in terms of the integrity of film reading. GBZ 70-2015 standard was used to compare the profusion of DR chest radiographs and 5 mm chest CT coronal MPR images of 66 objects, and the consistency test Kappa=0.64. GBZ 70-2015 standard and experimental chest CT standard were used to compare the profusion results of DR chest radiographs and 5 mm chest CT coronal MPR images of 66 objects, respectively, and the consistency test Kappa=0.80, with high consistency. Conclusion: 5 mm coronal MPR image is suitable for chest CT imaging in the diagnosis of pneumoconiosis. Following the selection path and method of GBZ 70-2015 profusion criterion, the established experimental chest CT standard in determining the profusion of small circular shadows in 5 mm coronal MPR images of chest CT with pneumoconiosis has a high consistency with GBZ 70-2015 standard.

[Efficacy of stent placement in treatment of malignant tracheoesophageal fistula and relevant factors of fistula closure].

Objective: To investigate the efficacy of stent placement in the treatment of malignant tracheoesophageal fistula (MTEF) and the factors affecting the closure of the fistula. Methods: Clinical, pathological, laboratory, and imaging data of 288 patients with MTEF admitted to Zhongda Hospital, Southeast University from 2015 to 2021were retrospectively analyzed. Among them, there were 208 males; the age was (63.6±10.5) years. A total of 94 patients received conservative treatment (conservative group), and 194 in the stent group (170 cases with esophageal stents and 24 cases with tracheal stents). Patients were followed-up at 2 weeks, 1 month, 3 months, and 6 months to evaluate the effect of stent implantation. Multivariable logistic regression was used to analyze factors affecting fistula closure. Results: Age, fistula size, leukocyte count before treatment, and fistula location were significantly different between the conservative group and the stent group (P<0.05). The Karnofsky functional status (KPS) score before treatment in the conservative group was lower than the stent group, (45.1±1.0) vs (51.8±0.7) scores, respectively (P<0.001). After 2 weeks and 1 month of treatment, improvement in KPS scores was significantly better in the stent group than in the conservative group (P<0.05). At 1 month, the pulmonary infection rate in the stent group was 33.5% (58/173), significantly lower than that in the conservative group [77.0% (47/61); P<0.001]. Among the 288 patients, the fistula was closed in 196 patients and unclosed in 92 patients. Fistula size (OR=3.429, 95%CI: 1.623-7.829, P=0.001), leukocyte count before treatment (OR=1.160, 95%CI: 1.027-1.317, P=0.018), KPS score before treatment (OR=0.898, 95%CI: 0.848-0.945, P<0.001) and the treatment method (conservative treatment as reference, esophageal stent OR=0.010, 95%CI: 0.004-0.030, P<0.001; tracheal stent OR=0.003, 95%CI: 0.000-0.042, P<0.001) were factors affecting fistula closure. In the 170 patients in the esophageal stent group, early complications (≤24 h) occurred in 71 patients, and late (>24 h) complications occurred in 11 patients. While in the 24 patients in the tracheal stent group, 9 had early complications and 2 had late complications. Conclusions: Stent placement is an effective treatment for MTEF compared to conservative treatment. Stent treatment, small fistula size, low pre-treatment leukocyte count, and high pre-treatment KPS score are beneficial to fistula closure.

[The application value of metagenomic next-generation sequencing technology in diagnosis and treatment of pulmonary infection in immunocompromised patients].

Objective: To evaluate the application value of metagenomic next-generation sequencing (mNGS) in the diagnosis and treatment of pulmonary infection in immunocompromised patients. Methods: A total of 78 patients with immunocompromised pulmonary infection [55 males and 23 females, aged (50.3±16.9) years] and 61 patients with non-immunocompromised pulmonary infection [42 males and 19 females, aged (63.6±15.9) years] in the Intensive Care Unit of the First Medical Center of College of the Pulmonary & Critical Care Medicine, Chinese PLA General Hospital from November 2018 to May 2022 were retrospectively selected. Patients in both groups received bronchoalveolar lavage fluid (BALF) mNGS and conventional microbiological tests (CMTs) while clinically diagnosed with pulmonary infection. The diagnostic positive rate, pathogen detection rate and clinical coincidence rate of the two methods were compared. At the same time, the difference of adjustment rate of anti-infective treatment strategy based on the results of mNGS detection was compared between the two groups. Results: The positive rates of mNGS in patients with pulmonary infection were 94.9% (74/78) and 82.0% (50/61) in the immunocompromised group and the non-immunocompromised group, respectively. The positive rates of CMTs in patients with pulmonary infection were 64.1% (50/78) and 75.4% (46/61) in the immunocompromised group and the non-immunocompromised group, respectively. The positive rates of mNGS and CMTs in patients with pulmonary infection in immunocompromised group showed a statistically significant difference (P<0.001). The detection rates of mNGS in the immunocompromised group for pneumocystis jirovecii and cytomegalovirus were 41.0% (32/78) and 37.2% (29/78), respectively, and the detection rates of Klebsiella pneumoniae, chlamydia psittaci and Legionella pneumophila were 16.4% (10/61), 9.8% (6/61) and 8.2% (5/61) in the non-immunocompromised patients, respectively, which were higher than those of CMTs [1.3% (1/78), 7.7% (6/78), 4.9% (3/61), 0 and 0] (all P<0.05). In the immunocompromised group, the clinical coincidence rates of mNGS and CMTs and were 89.7% (70/78) and 43.6% (34/78), respectively, with a statistically significant difference (P<0.001). In the non-immunocompromised group, the clinical coincidence rates of mNGS and CMTs were 83.6% (51/61) and 62.3% (38/61), with a statistically significant difference (P=0.008). In the immunocompromised group, according to the results of the etiology of mNGS, the adjustment rate of anti-infection treatment strategy was 87.2% (68/78), while in the non-immunocompromised group, the adjustment rate of anti-infective treatment strategy was 60.7% (37/61), with a statistically significant difference (P<0.001). Conclusion: In patients with immunocompromised pulmonary infection, mNGS has more advantages than CMTs in diagnostic positive rate, diagnosis rate of mixed infection, pathogen detection rate and guidance of anti-infection treatment strategy adjustment, which is worthy of clinical promotion and application.

[Plurihormonal PIT1-lineage pituitary neuroendocrine tumors: a clinicopathological study].

Objective: To investigate the clinicopathological characteristics of plurihormonal PIT1-lineage pituitary neuroendocrine tumors. Methods: Forty-eight plurihormonal PIT1-lineage tumors were collected between January 2018 and April 2022 from the pathological database of Sanbo Brain Hospital, Capital Medical University. The related clinical and imaging data were retrieved. H&E, immunohistochemical and special stains were performed. Results: Out of the 48 plurihormonal PIT1-lineage tumors included, 13 cases were mature PIT1-lineage tumors and 35 cases were immature PIT1-lineage tumors. There were some obvious clinicopathological differences between the two groups. Clinically, the mature plurihormonal PIT1-lineage tumor mostly had endocrine symptoms due to increased hormone production, while a small number of immature PIT1-lineage tumors had endocrine symptoms accompanied by low-level increased serum pituitary hormone; patients with the immature PIT1-lineage tumors were younger than the mature PIT1-lineage tumors; the immature PIT1-lineage tumors were larger in size and more likely invasive in imaging. Histopathologically, the mature PIT1-lineage tumors were composed of large eosinophilic cells with high proportion of growth hormone expression, while the immature PIT1-lineage tumors consisted of chromophobe cells with a relatively higher expression of prolactin; the mature PIT1-lineage tumors had consistently diffuse cytoplasmic positive staining for keratin, while the immature PIT1-lineage tumors had various expression for keratin; the immature PIT1-lineage tumors showed more mitotic figures and higher Ki-67 proliferation index; in addition, 25.0% (12/48) of PIT1-positive plurihormonal tumors showed abnormal positive staining for gonadotropin hormones. There was no significant difference in the progression-free survival between the two groups (P=0.648) by Kaplan-Meier analysis. Conclusions: Plurihormonal PIT1-lineage tumor belongs to a rare type of PIT1-lineage pituitary neuroendocrine tumors, most of which are of immature lineage. Clinically increased symptoms owing to pituitary hormone secretion, histopathologically increased number of eosinophilic tumor cells with high proportion of growth hormone expression, diffusely cytoplasmic keratin staining and low proliferative activity can help differentiate the mature plurihormonal PIT1-lineage tumors from the immature PIT1-lineage tumors. The immature PIT1-lineage tumors have more complicated clinicopathological characteristics.

[Nonalcoholic fatty liver disease and bilirubin: correlation, mechanism, and therapeutic perspectives].

Non-alcoholic fatty liver disease (NAFLD) is a metabolic-related disorder induced by multiple factors and mainly characterized by excessive fat buildup in hepatocytes. With the consumption of a Western-style diet and obesity prevalence in recent years, the incidence of NAFLD has gradually increased, becoming an increasingly serious public health problem. Bilirubin is a heme metabolite and a potent antioxidant. Studies have demonstrated that bilirubin levels have an inverse correlation with the incidence rate of NAFLD; however, which form of bilirubin plays the main protective role is still controversial. It is considered that the main protective mechanisms for NAFLD are bilirubin antioxidant properties, insulin resistance reduction, and mitochondrial function. This article summarizes the correlation, protective mechanism, and possible clinical application of NAFLD and bilirubin.

[Epidemiological characteristics of familiar adult inherited metabolic liver disease].

Inherited metabolic liver diseases can occur in multi-age groups such as children, adolescents, adults, and others. With the improvement of diagnosis and treatment levels, more and more patients with childhood-onset diseases are surviving into adulthood. Some diseases originally faced by pediatric hepatologists also appear in adult hepatology clinics. This raises new challenges for adult hepatologists, requiring them to master more professional knowledge. However, specific data on the incidence rate of most inherited metabolic liver diseases is still lacking in our country. This article reviews the research progress of hereditary metabolic liver diseases and summarizes the epidemiological characteristics of familiar hereditary metabolic liver diseases in China.

[Progress in the treatment of sarcopenia in patients with cirrhosis].

Sarcopenia, a common complication of cirrhosis, has an incidence rate as high as 40% ~ 70%. Furthermore, adverse events such as shortened survival, lower quality of life, prolonged hospital stay, increased complications, a higher mortality rate, and sometimes altering the liver transplantation prognostic outcome in patients with liver cirrhosis are closely related. In recent years, some progress has been made in the research on the treatment of sarcopenia, but there is no clear treatment plan at present; thus, research and development by researchers and clinicians still need to be strengthened. This article briefly describes the relevant treatment methods for sarcopenia in patients with cirrhosis.

[Ten-year changes in clinical characteristics and antiviral treatment patterns of chronic hepatitis B in China: a CR-HepB-based real-world study].

Objective: To understand ten-year changes in clinical characteristics and antiviral treatment patterns of chronic hepatitis B in China. Methods: Patients with chronic HBV infection:demographic, virologic, hematologic, blood biochemistry, and antiviral treatment data were extracted from the China Registry of Hepatitis B (CR-HepB) database between 2012 and 2022 for descriptive statistics and change trend analysis. Multiple group comparisons were conducted using the Kruskal Wallis H test, while counting data was compared between groups using χ (2) test. Results: A total of 180 012 patients with chronic HBV infection were included, with a median age of 40 years old, and a male proportion accounting for 60.2%. The HBeAg positive rate was 43.3%. Over time, the median age of new patients each year increased from 39 to 47 years, while the HBeAg positive rate decreased from 51.3% to 32.8%. The initial diagnosis of patients was mainly CHB (71.4%), followed by hepatitis B cirrhosis (11.8%), inactive HBsAg carrier status (10.6%), and chronic HBV carrier status (6.2%). Among the newly registered patients every year from 2012 to 2022, the proportion of hepatitis B cirrhosis remained stable, but after 2019, the proportion of CHB increased and the proportion of other diagnoses decreased. The proportion of patients with cirrhosis increased with age in different age groups, with 3.5%, 19.3%, and 30.4% in the < 40, 40-69, and≥70 age groups, respectively. The proportion of women in patients with cirrhosis also increased with age, from 16.1% in those < 30 years old to 44.3% in those≥80 years old. From 2012 to 2022, the proportion of patients receiving first-line nucleos(t)ide analog antiviral treatment increased year by year, from 51.0% in 2012-2013 to 99.8% in 2022. Conclusion: The CR-HepB registration data reflect the changes in clinical characteristics and antiviral treatment patterns in patients with chronic HBV infection in China over the past ten years and can thus provide a reference to promote hepatitis B diagnosis and treatment practice, as well as scientific research.

[Application of different prognostic scores in liver transplantation decision-making for acute-on-chronic liver failure].

Objective: To compare the impact of different prognostic scores in patients with acute-on-chronic liver failure (ACLF) in order to provide treatment guidance for liver transplantation. Methods: The information on inpatients with ACLF admitted at Beijing You'an Hospital Affiliated to Capital Medical University and the First Affiliated Hospital of Zhejiang University School of Medicine from January 2015 to October 2022 was collected retrospectively. ACLF patients were divided into liver transplantation and non-liver transplantation groups, and the two groups prognostic conditions were followed-up. Propensity score matching was carried out between the two groups on the basis of liver disease (non-cirrhosis, compensated cirrhosis, and decompensated cirrhosis), the model for end-stage liver disease incorporating serum sodium (MELD-Na), and ACLF classification as matching factors. The prognostic condition of the two groups after matching was compared. The difference in 1-year survival rate between the two groups was analyzed under different ACLF grades and MELD-Na scores. The independent sample t-test or rank sum test was used for inter-group comparison, and the χ (2) test was used for the comparison of count data between groups. Results: In total, 865 ACLF inpatients were collected over the study period. Of these, 291 had liver transplantation and 574 did not. The overall survival rates at 28, 90, and 360 days were 78%, 66%, and 62%, respectively. There were 270 cases of matched ACLF post-liver transplantation and 270 cases without ACLF, in accordance with a ratio of 1:1. At 28, 90, and 360 days, patients with non-liver transplantation had significantly lower survival rates (68%, 53%, and 49%) than patients with liver transplantation (87%, 87%, and 78%, respectively; P < 0.001). Patients were classified into four groups according to the ACLF classification criteria. Kaplan-Meier survival analysis showed that the survival rates of liver transplantation and non-liver transplantation patients in ACLF grade 0 were 77.2% and 69.4%, respectively, with no statistically significant difference (P = 0.168). The survival rate with an ACLF 1-3 grade was significantly higher in liver transplantation patients than that of non-liver transplantation patients (P < 0.05). Patients with ACLF grades 1, 2, and 3 had higher 1-year survival rates compared to non-liver transplant patients by 50.6%, 43.6%, and 61.7%, respectively. Patients were divided into four groups according to the MELD-Na score. Among the patients with a MELD-Na score of < 25, the 1-year survival rates for liver transplantation and non-liver transplantation were 78.2% and 74.0%, respectively, and the difference was not statistically significant (P = 0.149). However, among patients with MELD-Na scores of 25-30, 30-35, and≥35, the survival rate was significantly higher in liver transplantation than that of non-liver transplantation, and the 1-year survival rate increased by 36.4%, 54.9%, and 62.5%, respectively (P < 0.001). Further analysis of the prognosis of patients with different ACLF grades and MELD-Na scores showed that ACLF grades 0 or 1 and MELD-Na score of < 30 had no statistically significant difference in the 1-year survival rate between liver transplantation and non-liver transplantation (P > 0.05), but in patients with MELD-Na score≥30, the 1-year survival rate of liver transplantation was higher than that of non-liver transplantation patients (P < 0.05). In the ACLF grade 0 and MELD-Na score of≥30 group, the 1-year survival rates of liver transplantation and non-liver transplantation patients were 77.8% and 25.0% respectively (P < 0.05); while in the ACLF grade 1 and MELD-Na score of≥30 group, the 1-year survival rates of liver transplantation and non-liver transplantation patients were 100% and 20.0%, respectively (P < 0.01). Among patients with ACLF grade 2, the 1-year survival rate with MELD-Na score of < 25 in patients with liver transplantation was 73.9% and 61.6%, respectively, and the difference was not statistically significant (P > 0.05); while in the liver transplantation patients group with MELD-Na score of ≥25, the 1-year survival rate was 79.5%, 80.8%, and 75%, respectively, which was significantly higher than that of non-liver transplantation patients (36.6%, 27.6%, 15.0%) (P < 0.001). Among patients with ACLF grade 3, regardless of the MELD-Na score, the 1-year survival rate was significantly higher in liver transplantation patients than that of non-liver transplantation patients (P < 0.01). Additionally, among patients with non-liver transplantation with an ACLF grade 0~1 and a MELD-Na score of < 30 at admission, 99.4% survived 1 year and still had an ACLF grade 0-1 at discharge, while 70% of deaths progressed to ACLF grade 2-3. Conclusion: Both the MELD-Na score and the EASL-CLIF C ACLF classification are capable of guiding liver transplantation; however, no single model possesses a consistent and precise prediction ability. Therefore, the combined application of the two models is necessary for comprehensive and dynamic evaluation, but the clinical application is relatively complex. A simplified prognostic model and a risk assessment model will be required in the future to improve patient prognosis as well as the effectiveness and efficiency of liver transplantation.

[Interpretation of the essential updates in guidelines for the prevention and treatment of chronic hepatitis B (Version 2022)].

Chinese Society of Hepatology and Chinese Society of Infectious Diseases, Chinese Medical Association update the guidelines for the prevention and treatment of chronic hepatitis B (version 2022) in 2022. The latest guidelines recommend more extensive screening and more active antiviral treating for hepatitis B virus infection. This article interprets the essential updates in the guidelines to help deepen understanding and better guide the clinical practice.

Selenylation to charge transfer improvement at the counter electrode (CE)/electrolyte interface for nanocrystalline Cu1.8S1-xSex CEs.

Pt counter electrodes (CEs) have been widely used in dye-sensitized solar cells (DSSCs) due to their high conductivity and electrocatalytic activity. However, industrialization of DSSCs is limited by shortcomings of Pt CEs such as being expensive, and weak corrosion resistance in electrolytes. Reported in this paper is two simple approaches to Pt-free Cu1.8S1-xSex CEs. Nanocrystalline Cu1.8S1-xSex CEs were fabricated via two processes, that is, a solvothermal process to Cu1.8S1-xSex powder followed by CE fabrication, and a solvothermal process and CE fabrication to Cu1.8S films followed by selenylation to Cu1.8S1-xSex CEs. Photoelectric conversion efficiencies (PCE) of 4.02% and 4.16% were achieved respectively by the as-fabricated Cu1.8S1-xSex CEs. Compared with the cells with Cu1.8S CEs fabricated by the same processes, increases of 19% and 45% were achieved, respectively. The PCE improvement comes from the enhancement of charge transfer at the CE/electrolyte interface induced by the selenylation of the CEs.

[Pediatric SMARCB1/INI1-deficient poorly differentiated chordoma of the skull base: report of five cases and review of literature].

Objective: To investigate the clinicopathological characteristics and differential diagnosis of pediatric SMARCB1/INI1-deficient poorly differentiated chordoma (PDC) of the skull base. Methods: Five cases of SMARCB1/INI1-deficient PDC were identified in 139 cases of chordoma diagnosed in Sanbo Brain Institute, Capital Medical University, Beijing, China from March 2017 to March 2021. The clinical and imaging data of the 5 PDCs were collected. H&E and immunohistochemical staining, and DNA methylation array were used, and the relevant literatures were reviewed. Results: All 5 PDCs were located at the clivus. The average age of the patients was 6.4 years, ranging from 3 to 16 years. Three patients were female and two were male. Morphologically, in contrast with classical chordomas, they presented as epithelioid or spindle tumor cells organized in sheets or nests, with necrosis, active mitoses, and infiltration into surrounding tissue. All cases showed positivity of CKpan, EMA, vimentin and brachyury (nuclear stain), and loss of nuclear SMARCB1/INI1 expression. S-100 protein expression was not frequent (2/5). Ki-67 proliferative index was high (20%-50%). All cases had over-expressed p53. It was necessary to differentiate SMARCB1/INI1-dificient PDC from SMARCB1/INI1-dificient tumors occurring at skull base of children or the tumors with epithelial and spindle cell morphological features. The 3 PDCs with DNA methylation testing showed the methylation profiles different from the pediatric atypical teratoid/rhabdoid tumors. They formed an independent methylation profile cluster. The clinical prognosis of the 5 patients was poor, and the overall survival time was 2-17 months. Conclusions: PDC is a special subtype of chordoma, which often affects children and occurs in the clivus. The PDC shares epithelioid or spindle cell morphologic features which are different from the classic chordoma. Besides the typical immunohistochemical profile of chordoma, PDC also has loss of nuclear SMARCB1/INI1 expression and distinct epigenetic characteristics.

[Clinicopathological characteristics of H3K27-altered diffuse midline glioma and evaluation of NTRK as its therapeutic target].

Objective: To investigate the clinicopathological characteristics of H3K27-altered diffuse midline glioma (DMG), and to analyze DMG's prognostic factors, and subsequently, to study the possibility of using NTRK as a therapeutic target for DMG. Methods: A total of 232 DMG diagnosed at the Sanbo Brain Hospital, Capital Medical University, Beijing, China from July 2016 to March 2021 were collected. Their clinical, radiological and pathological features, the ratio of MGMT promoter methylation, expression of NTRK, and characteristics of NTRK gene fusion were analyzed. The prognostic values of different factors were also studied, including age, tumor location, histological grade, gene and protein expression of NTRK, and postoperative adjuvant therapy. Results: Among the 232 DMG cases, there were 8 patients with both primary and relapse tumors on the record. Thus, a total of 224 patients were analyzed, including 118 males and 106 females. There were 126 adults (>18 years of age) and 98 children (≤18 years of age). Notably, the most frequent location was thalamus (41/126, 32.5%) in adults, but brainstem (59/96, 60.2%) in children. The lesions showed T1 hypointensity or isointensity, and T2 hyperintensity. However, contrast enhancement patterns of the tumors varied, with many tumors lacking contrast-enhancing. The histological grades included grade 2 (9/224, 4.0%), grade 3 (41/224, 18.3%) and grade 4 (174/224, 77.7%). Two hundred and twenty-four DMGs were diffusely positive for H3K27M and negative for H3K27me3. The ratio of MGMT promoter methylation was low (1/45, 2.2%). One hundred and seventy-seven of the 224 cases (177/224, 79.0%) were positive for NTRK. Fifty cases were analyzed using fluorescence in situ hybridization. Among them, five DMGs (positive rate, 10.0%) were NTRK fusion positive. This study showed that there were no differences between adult and pediatric DMGs in histological grading, expression of NTRK, and NTRK gene fusion. One hundred and fifty-nine patients were included in the follow-up analysis (P>0.05). During the follow-up period, 109/159 patients (69.6%) died of the disease, with a median survival time of 12 months (range 1 to 55 months). Univariate log-rank analysis showed that age, location, surgical procedure and postoperative adjuvant therapy were associated with overall survivals of the DMG patients (P<0.05). Conclusions: The prognosis of DMG is poor overall. There are differences between adult and pediatric DMGs in anatomic location and prognosis, but not in other features. NTRK1 gene fusion is detected in 10.0% of the tumors. It suggests that TRK inhibitor might be a choice for treating DMG.

[Interpretation of the American College of Gastroenterology clinical guidelines for acute-on-chronic liver failure].

In January 2022, the American College of Gastroenterology released its first clinical guidelines, integrating the latest research, summarizing the three current definitions characteristics, and proposing recommendations and core viewpoints with important clinical practice value to guide diagnosis, treatment, and management of acute-on-chronic liver failure. This article interprets and summarizes the highlights of the guideline, raises controversial issues, and suggests directions for future research.

[Management of hepatolenticular degeneration during pregnancy].

Hepatolenticular degeneration (Wilson's disease, WD) is a kind of autosomal recessive genetic disease characterized by disorders of copper metabolism. It is caused by mutations in the ATP7B gene, resulting in impaired excretion of copper into the bile, and then pathological deposition in the liver, brain, and other organs. Early diagnosis and treatment can significantly improve the prognosis of patients with WD. However, there is still no clear consensus on the treatment and management of WD during pregnancy. Herein, the clinical management of WD during pregnancy is summarized for clinicians' reference.

[Emphasis on the treatment of biliary liver disease].

Biliary liver disease refers to a group of diseases in which lesions of the biliary system cause liver damage. Early detection and standardized treatment can improve patient outcomes and prolong survival.Treatment includes pharmacological, non-pharmacological, and combination therapy. However, due to the involvement of a variety of diseases and different treatment characteristics, multidisciplinary cooperation is required when necessary, and there are still certain problems to be solved in the treatment.

[Introduction to the recommendations from the European Association for the Study of the Liver clinical practice guidelines on the management of cystic liver disease].

The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.

[Recommendations of EASL clinical practice guidelines on haemochromatosis].

Haemochromatosis is characterised by elevated transferrin saturation (TSAT) and progressive iron loading that mainly affects the liver. Early diagnosis and treatment by phlebotomy can prevent cirrhosis, hepatocellular carcinoma, diabetes, arthropathy and other complications. In patients homozygous for p.Cys282Tyr in HFE, provisional iron overload based on serum iron parameters (TSAT >45% and ferritin >200 µg/L in females and TSAT >50% and ferritin >300 µg/L in males and postmenopausal women) is sufficient to diagnose haemochromatosis. In patients with high TSAT and elevated ferritin but other HFE genotypes, diagnosis requires the presence of hepatic iron overload on MRI or liver biopsy. The stage of liver fibrosis and other end-organ damage should be carefully assessed at diagnosis because they determine disease management. Patients with advanced fibrosis should be included in a screening programme for hepatocellular carcinoma. Treatment targets for phlebotomy are ferritin <50 µg/L during the induction phase and <100 µg/L during the maintenance phase.

[An introduction to multidisciplinary recommendations for Wilson's disease comprehensive diagnosis and management: 2022 practice guidance from the American Association for the Study of Liver Diseases].

The incidence of Wilson's disease (WD) is global, with an estimated prevalence rate of 30 per million or higher. WD clinical manifestations can be liver disease, progressive neurologic deficits (non evident or even absent liver dysfunction), psychiatric disorders, or a combination of these. Children and younger patients are more likely to develop WD as an isolated liver disease than older patients. Symptoms are often vague and can appear at any age. To that end, in 2022, the American Association for the Study of Liver Diseases published the full version of the WD guidelines and recommendations developed by a panel of experts, providing a modern approach for WD diagnosis and management in an effort to assist clinicians in implementing the most recent diagnostic and management strategies.