To support clinicians in making informed decisions regarding the diagnosis and management of inherited hyperbilirubinemia, including Gilbert syndrome, Crigler-Najjar syndrome, Dubin-Johnson syndrome, and Rotor syndrome, the Inherited and Metabolic Liver Disease Collaboration Group of the Hepatology Branch of the Chinese Medical Association convened a panel of Chinese experts in this field. This multidisciplinary consortium developed the present expert consensus by integrating the latest advances in both clinical practice and basic research.

ATP-binding cassette (ABC) transporters are transmembrane proteins involved in the translocation of bilirubin, bile acids, phospholipids, and cholesterol into bile canaliculi. Mutations in particular genes encoding these transporters—including BSEP (ABCB11 gene), MDR3 (ABCB4 gene), sterolin-1 and sterolin-2 (ABCG5/8 genes), and MRP2 (ABCC2 gene)—result in a wide spectrum of liver diseases, ranging from benign conditions such as Dubin-Johnson syndrome to more severe presentations like progressive familial intrahepatic cholestasis. The severity of disease is influenced by many factors, including zygosity, mutation type, and environmental modifiers such as hormones, consanguinity, and founder effects. Homozygous and compound heterozygous mutations typically result in severe and early-onset diseases, while heterozygous single-allelic mutants generally result in milder diseases. Next-generation genetic testing has proven to have high diagnostic value and is important for prognostication. With knowledge of the underlying specific mutations, there is also potential for future targeted therapy for many severe diseases. The aim of this review is to update and discuss the hepatic diseases associated with ABC transporter mutations, the genetic and environmental effects that influence the severity of disease, typical presentations of these cholestatic hepatic diseases, diagnostic considerations, and treatment options.

Terminal ileum intubation is considered the completion step of colonoscopy and is usually performed to assess the ileum. The histological examination of the ileal mucosa, which is acquired during terminal ileum intubation, may allow an accurate diagnosis. However, there is no absolute consensus on when ileoscopy and biopsy should be attempted. As a result, we aimed to evaluate whether terminal ileum intubation and biopsy should be performed routinely.

Systematic searches were performed in the PubMed, EMBASE, and Cochrane Library databases, as well as the Science Citation Index via the Web of Science platform. Reference lists from the identified papers were manually searched. Systematic searches were performed from January 1, 1971, to October 1, 2025. Studies reporting on terminal ileum intubation and biopsy during colonoscopy were included. Case reports, letters, reviews, and animal studies were excluded. The primary outcomes were the diagnostic yield of terminal ileum intubation and the rate of necessitating a change in management. Data were extracted independently by three reviewers.

Thirty-six studies were included. The subtotal diagnostic yield and the rate of necessary change among the selected patients were much greater than those among the unselected patients (5.1% versus 2.5% and 1.5% versus 0.4%, respectively). In addition, the diagnostic yield was found more frequently for inflammatory bowel disease, anemia, abdominal pain, and chronic diarrhea than for the other indications (26.7%, 16.1%, 14.9%, 12.4%, and 3.2%, respectively). The yield of ileal histopathology with a normal endoscopic appearance was low in both unselected and selected patients (3.5% and 2.4%, respectively).

Terminal ileum intubation is recommended as gold standard for completing colonoscopy. Biopsy should be considered in patients with abnormal endoscopic findings or specific high-risk symptoms.

Sedation monitoring is crucial in neurosurgical intensive care units to ensure optimal patient comfort and safety. However, sedation practices vary significantly. This study aimed to evaluate and summarize the evidence related to sedation monitoring in neurocritical care patients, with a focus on identifying best practices for improving monitoring accuracy and patient outcomes.

This study was conducted as an evidence summary, following the evidence summary reporting standards of the Fudan University Evidence-based Nursing Center. The evidence on sedation monitoring management in neurocritical care patients was systematically retrieved using the 6S evidence model, including clinical decisions, best practices, guidelines, expert consensus, evidence summaries, systematic reviews, and more. Searches of domestic and international databases covered all records from the databases’ inception to June 2024. Two researchers independently selected literature that met the inclusion criteria and conducted quality assessment, evidence-level evaluation, and evidence synthesis.

Ten high-quality studies were ultimately included. From these, twenty pieces of best evidence were extracted, covering four categories: monitoring personnel, monitoring targets, monitoring tools, and monitoring timing and content. Among these, fifteen pieces of evidence were classified as strong recommendations, while five were classified as weak recommendations.

This study summarized the best evidence on sedation monitoring for neurocritical care patients, providing guidance for clinical staff to improve sedation monitoring accuracy and patient outcomes in neurosurgical intensive care units.

Metabolism-associated fatty liver disease (MAFLD) is a disease of hepatic fat accumulation resulting from metabolic disorders. Currently, MAFLD is the most common cause of chronic liver disease in children and adolescents. No effective or safe drugs for treating children with MAFLD are available. The traditional Chinese medicine used for treating MAFLD in children is characterized by being holistically regulated, multileveled, multi-targeting, and very safe. In this paper, the progress in research involving treatment using traditional Chinese medicine for MAFLD in children is reviewed.

To observe the clinical efficacy of Jiawei Lizhong Tang combined with dorsal Yu acupoint embedding in patients with non-alcoholic steatohepatitis (NASH).

A total of 118 patients with NASH who attended the Department of Gastroenterology at Liuzhou Hospital of Traditional Chinese Medicine from January 2020 to December 2022 were selected as study subjects. The participants were randomly assigned to either the control group or the observation group using a random number table, with 59 cases in each group. The control group received treatment with Western medicine (compound glycyrrhizin capsule), whereas the observation group received treatment with traditional Chinese medicine combined with thread embedding (Jiawei Lizhong Tang combined with dorsal Yu acupoints embedding). Both groups received a treatment course of 12 weeks. The following anthropometric indicators were measured: weight, waist circumference, and body mass index. Liver function was assessed by measuring alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma-glutamyltransferase (GGT), and total bilirubin (TBIL). Lipid profile indicators included triglycerides (TG), total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), and high-density lipoprotein cholesterol (HDL-C). Blood glucose indicators included fasting blood glucose, fasting insulin (FINS), and insulin resistance index (HOMA-IR). Additionally, liver stiffness measurement (LSM), controlled attenuation parameter (CAP), and liver/spleen CT ratio were assessed. Clinical efficacy was evaluated at the end of the treatment period.

After 12 weeks of treatment, significant improvements were observed in weight, waist circumference, BMI, serum ALT, AST, GGT, TBil, TG, TC, LDL-C, HDL-C, fasting glucose, FINS, HOMA-IR, LSM, CAP, and liver/spleen CT ratio in both groups compared to baseline (P＜0.05). The improvements in these indicators were significantly better in the observation group than in the control group after treatment (P＜0.05).

The treatment of NASH with Jiawei Lizhong Tang combined with dorsal Yu acupoint embedding effectively improves patient condition, reduces liver function damage, regulates blood lipids, insulin resistance, and hepatic steatosis. The therapeutic effect is superior to that of Western medicine therapy.

Infections are frequent and lethal complications of acute-on-chronic liver failure (ACLF). Reliable biomarkers to distinguish fungal from bacterial infections remain limited. Given the central role of immune dysfunction in ACLF, we aimed to evaluate the diagnostic value of serum cytokines in differentiating invasive pulmonary aspergillosis (IPA) from bacterial pneumonia (BP) in HBV-associated ACLF.

This retrospective case-control study enrolled ACLF patients admitted to the Tongji Hospital, between 2018 and 2022. Patients were categorized into IPA, BP, and non-infection groups. The BP and non-infection groups were propensity score-matched to the IPA cases. Serum cytokines levels (IL-1β, sIL-2R, IL-6, IL-8, IL-10, TNF-α) and clinical data were collected, with the diagnostic performance of these cytokines as biomarkers assessed via ROC curves.

A total of 32 IPA, 96 BP, and 96 non-infection patients were enrolled, with balanced baseline characteristics. Compared with the non-infection group, the IPA group had higher sIL-2R (1,606.00 vs. 1,211.50 U/mL, P = 0.019) and IL-6 (69.03 vs. 15.98 pg/mL, P < 0.001) levels, but lower IL-8 levels (62.20 vs. 132.00 pg/mL, P = 0.025). The BP group showed elevated sIL-2R (1,792.00 U/mL), IL-6 (49.42 pg/mL), IL-10 (13.40 pg/mL) levels compared to the non-infection group (all P < 0.001). Also, IL-8 was lower in the IPA group than in the BP group (62.20 vs. 176.00 pg/mL, P < 0.001) and its assessment could best distinguish IPA from BP (AUC = 0.743, cut-off = 76.60 pg/mL; sensitivity = 66.7%, specificity = 82.1%).

Serum IL-8 exhibited superior diagnostic value for IPA in patients with HBV-ACLF and could effectively discriminate Aspergillus infections from bacterial infections.

Chronic hepatitis B (CHB) poses a major global health burden, with China particularly affected. Effective antiviral therapy is crucial to prevent disease progression, but responses may vary by Hepatitis B virus (HBV) genotype. This prospective study aimed to compare genotype-specific responses to 144-week entecavir (ETV) therapy in HBeAg-positive CHB patients, with particular emphasis on histological improvement assessed through paired liver biopsies.

We enrolled 49 treatment-naïve CHB patients (HBV DNA ≥ 20,000 IU/mL, alanine transaminase (ALT) > 2× ULN, and Scheuer system G ≥ 2) who received ETV 0.5 mg/day. HBV genotyping was performed using Polymerase Chain Reaction and fragment length analysis. The primary endpoint was histological improvement (i.e., ≥ 2-grade reduction in necroinflammatory activity without fibrosis progression), evaluated via paired biopsies (baseline and week 144) by blinded pathologists. Secondary endpoints included virological response (i.e., serum HBV DNA < 100 IU/mL), HBeAg seroconversion, and ALT normalization.

The cohort included 24 genotype B and 24 genotype C patients (one genotype A patient was excluded from genotype-specific analyses). Genotype B showed significantly higher histological improvement rates (91.3% vs. 63.2%, P = 0.027) and greater inflammation resolution (0 ≤ G < 1: 56.5% vs. 26.3%, P = 0.048). Virological suppression was excellent in both groups (100% vs. 100%). HBeAg seroconversion trended higher in genotype C (29.2% vs. 50.0%, P = 0.140). All patients achieved ALT normalization by week 48, with no safety concerns.

HBV genotype B demonstrates superior histological responses to ETV therapy compared with genotype C, supporting the clinical value of HBV genotyping for personalized CHB management. These findings highlight the importance of considering viral genotype when evaluating treatment outcomes.

Dysphagia, a severe comorbidity of many neurological diseases, often lacks targeted therapies. Electrical stimulation of cranial nerves represents a novel therapeutic class. This critical review assessed the clinical effectiveness and safety of various approaches for electrical stimulation of the cranial nerves for treating dysphagia, categorized as implantable (directly targeting the nerve), minimally invasive (pharyngeal electrical stimulation), and non-invasive (transcutaneous). A critical literature review was conducted following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The PubMed database was comprehensively searched, and studies were rigorously assessed for inclusion and exclusion criteria. The Newcastle–Ottawa Scale was used to assess the risk of bias. The analysis included 15 clinical studies: four assessing vagus nerve stimulation (including implantable and transcutaneous approaches) and eleven assessing pharyngeal electrical stimulation. Most evaluated studies, particularly for pharyngeal electrical stimulation and transcutaneous vagus nerve stimulation, demonstrated significant beneficial effects on validated dysphagia outcome measures. Importantly, no long-term severe adverse effects were reported across the evaluated stimulation approaches. Cumulative evidence indicates that vagus nerve stimulation and pharyngeal electrical stimulation approaches can effectively alleviate dysphagia symptoms. The different stimulation approaches appear to be complementary, with distinct profiles rendering them suitable for different therapeutic contexts (e.g., short-term hospital-based vs. long-term at-home treatment). Consequently, they represent distinct and valuable options for individualized dysphagia therapy.