BACKGROUND:The use of methylprednisolone (MP) is still controversial for hepatitis B virus related acute-on-chronic liver failure (HBV-ACLF). We aimed to explore the change in dendritic cells (DCs) during MP treatment in HBV-ACLF to guide the use of MP to improve patient's prognosis. METHODS:Patients with HBV-ACLF were prospectively allocated to groups given methylprednisolone intravenously (1.5 mg/kg/day for the first 3 days, 1 mg/kg/day for the second 2 days, and 0.5 mg/kg/day for the last 2 days, MP group, n = 36) plus standard treatment or standard treatment only (CM group, n = 34). The phenotype [myeloid and plasmacytoid DCs (mDCs, pDCs)] and function of DCs (IL-12 and IFN-α production) were measured at baseline (0d), 3d, 7d, 10d, 14d, 28d, and then monthly until 3 months. Patients' survival was assessed until day 90. RESULTS:The 3-month survival rate was significantly higher in the MP group than the control (72.0 % vs. 35.5 %，P < 0.01). The phenotype and function of DCs were suppressed in the MP group. The mDCs counts was lower in non-survivors compared to survivors at baseline. Patients with a decline in mDCs counts at the 7th day and a continuous increase in mDCs counts from the 10th day presented a better outcome for patients with MP treatment. Bilirubin was the only relative factor for the restoration of mDCs in the MP group (odds ratio, 0.991; 95 % confidence interval, 0.984-0.999; P = 0.023). CONCLUSIONS:Methylprednisolone could improve the outcome of HBV-ACLF by inhibiting the circulating mDCs counts. And the recovery of mDCs counts after methylprednisolone treatment could represent a favorable response. We can consider monitoring the circulating DCs counts to guide the use of MP in HBV-ACLF in order to improve patient outcomes.

BACKGROUND:Primary biliary cholangitis (PBC) is associated closely with the gut microbiota. This study aimed to explore the characteristics of the gut microbiota after the progress of PBC to cirrhosis. METHOD:This study focuses on utilizing the 16S rRNA gene sequencing method to screen for differences in gut microbiota in PBC patients who progress to cirrhosis. Then, we divided the data into training and verification sets and used seven different machine learning (ML) models to validate them respectively, calculating and comparing the accuracy, F1 score, precision, and recall, and screening the dominant intestinal flora affecting PBC cirrhosis. RESULT:PBC cirrhosis patients showed decreased diversity and richness of gut microbiota. Additionally, there are alterations in the composition of gut microbiota in PBC cirrhosis patients. The abundance of Faecalibacterium and Gemmiger bacteria significantly decreases, while the abundance of Veillonella and Streptococcus significantly increases. Furthermore, machine learning methods identify Streptococcus and Gemmiger as the predominant gut microbiota in PBC patients with cirrhosis, serving as non-invasive biomarkers (AUC = 0.902). CONCLUSION:Our study revealed that PBC cirrhosis patients gut microbiota composition and function have significantly changed. Streptococcus and Gemmiger may become a non-invasive biomarker for predicting the progression of PBC progress to cirrhosis.

Primary biliary cholangitis (PBC) is an autoimmune liver disease. During the diagnostic process, the patient's autoimmune antibodies are routinely examined. Approximately 20% of PBC patients have positive anti-centromere antibody (ACA). We evaluated the clinical characteristics of ACA-positive and ACA-negative PBC patients to explain the differences in disease progression between these two groups. Retrospective data from 961 PBC patients at Beijing Youan Hospital from 2010 to 2019 were gathered and separated into two groups based on ACA positivity. We collected and evaluated clinical laboratory indices, gastroscopy findings, and liver function assessments. In addition, 60 liver biopsies were available for comparison between the 2 groups. Pathologists staged the histological findings using the Ludwig staging criteria and Nakanuma staging and grading. Immunohistochemical staining was also performed on liver biopsies to examine the expression of cytokeratin 7 (CK7) in the tissue. A synthesis of clinical indicators in the large cohort showed that alanine transaminase, aspartate aminotransferase, total bilirubin, IgG, white blood cell, and platelet were significantly lower in the ACA-positive group, indicating that the overall status of liver injury was more moderate in the ACA-positive group. Additionally, ACA-positive patients in the non-cirrhotic group were more likely to present with gastroesophageal varices related to portal hypertension. Finally, analysis of pathologic findings showed that parameters were mostly comparable in the two groups, but CK7 differed and was more significantly lower in the ACA-positive group in albumin-bilirubin grade 2 and 3 patients. In summary, we characterized and compared the clinical features of ACA-positive and ACA-negative PBC patients, corroborating previous studies on the relationship between ACA positivity and portal hypertension cross-sectionally. It suggested that gastroesophageal varices might happen in the earlier course of PBC natural progression in the ACA-positive group.

Background The Albumin-Bilirubin (ALBI) score and grade are widely used to stratify patients with primary biliary cholangitis (PBC) into different disease statuses and risk levels. Recent studies have increasingly highlighted the role of gut microbiota in autoimmune liver diseases. This study aimed to investigate the differences in gut microbiota among PBC patients with varying ALBI grades.Methods Clinical data and stool samples were collected from outpatient and inpatient PBC patients between 2019 and 2022. Gut microbiota profiles were obtained using 16S rDNA sequencing of stool samples. We analyzed alpha diversity, beta diversity, LEfSe analysis and pathway function prediction. Additionally, various machine learning methods-including random forest (RF), lasso, gradient boosting machine (GBM) and support vector machine (SVM)-were employed to identify key features and to build and validate predictive models using bootstrap techniques.Results Clinical characteristics of ALBI grade 1 patients were comparatively better than those of ALBI grade 2 and 3 patients, including multiple laboratory indices. Gut microbiota analysis revealed that species richness and balance were higher in ALBI grade 1 patients. Both the comparison of the most abundant genera and the linear discriminant analysis (LDA) in LEfSe demonstrated that Lachnospira had a higher abundance and better discriminative ability in ALBI grade 1. Pathway function prediction indicated that sulfur metabolism was upregulated in higher ALBI grades. Furthermore, RF identified 10 specific genera, which were then used to build and validate models for discriminating PBC patients according to their ALBI grades. All three models, developed using different machine learning methods, demonstrated good discrimination ability (mean AUC 0.75-0.80).Conclusion This study highlights significant differences in gut microbiota profiles among PBC patients with different ALBI grades. The increased abundance of Lachnospira and upregulation of sulfur metabolism pathways are notable in patients with lower ALBI grades. The machine learning models developed based on gut microbiota features offer promising tools for discriminating between PBC patients with varying disease severities, which could enhance the precision of treatment strategies.

AI-aided clinical diagnosis is desired in medical care. Existing deep learning models lack explainability and mainly focus on image analysis. The recently developed Dynamic Uncertain Causality Graph (DUCG) approach is causality-driven, explainable, and invariant across different application scenarios, without problems of data collection, labeling, fitting, privacy, bias, generalization, high cost and high energy consumption. Through close collaboration between clinical experts and DUCG technicians, 46 DUCG models covering 54 chief complaints were constructed. Over 1,000 diseases can be diagnosed without triage. Before being applied in real-world, the 46 DUCG models were retrospectively verified by third-party hospitals. The verified diagnostic precisions were no less than 95%, in which the diagnostic precision for every disease including uncommon ones was no less than 80%. After verifications, the 46 DUCG models were applied in the real-world in China. Over one million real diagnosis cases have been performed, with only 17 incorrect diagnoses identified. Due to DUCG's transparency, the mistakes causing the incorrect diagnoses were found and corrected. The diagnostic abilities of the clinicians who applied DUCG frequently were improved significantly. Following the introduction to the earlier presented DUCG methodology, the recommendation algorithm for potential medical checks is presented and the key idea of DUCG is extracted.

Background: There is no consensus on the timing of immunotherapeutic strategies for the first- episode anti-myelin oligodendrocyte glycoprotein-IgG (MOG-IgG) associated disorders (MOGAD) presenting with isolated optic neuritis (ON). Objective: To investigate the optimal timing of intravenous methylprednisolone therapy (IVMP) and necessity of immunosuppressive therapy for the first-episode isolated MOG-IgG associated ON (iMOG-ON). Methods: Adult patients with the first-episode iMOG-ON were enrolled. Primary outcomes were best-corrected visual acuity (BCVA) at last follow-up (i.e. final BCVA) and relapse, and their predictors were assessed by multivariate analysis. Results: 62 patients were included. Logistic regression analysis revealed BCVA at the time of IVMP (odds ratio: 0.463 (95 % confidence interval (CI) 0.310-0.714) was a factor predictive of regaining a final BCVA of 0.0 logMAR vision, and its Youden optimal criterion was <0.175 logMAR by plotting the receiver operating characteristic curve. The time-dependent cox proportional hazards model exhibited MMF therapy was not associated with a high likelihood of relapse-free survival (HR = 1.099, 95 % CI 0.892-1.354, P = 0.376) after adjusting for age of onset, gender, and baseline MOG serum titers. Similar analysis exhibited evidently negative association between high MOG-IgG serum titers at baseline and relapse-free survival after adjusting for age of onset, gender, and MMF therapy (HR = 0.339, 95 % CI 0.155-0.741, P = 0.007). Conclusions: During the first episode of iMOG-ON, the optimal timing of IVMP may be a short timeframe before visual acuity decreasing to 0.175 logMAR, and MMF therapy may not be recommended for patients with low MOG-IgG serum titers. Further long-term follow-up studies are required to validate these findings.

Clinical novitiate provided a learning platform for medical students to apply theoretical knowledge to clinical practice,which was an important step for medical students to complete the role transformation to clinicians.At present,the difficulty of clinical teaching of infectious diseases was gradually increasing,which showed that the theoretical learning of online teaching was not stable,the lack of special probation materials,the gradual reduction of typical cases,the teaching method was single,the age gradient of teachers was single,the fear of infectious diseases of students and the poor compliance of patients.In order to improve the quality of clinical practice teaching,a series of reform measures should be taken including building stable online learning platform,establishing the trainee teaching materials and typical cases,improving teaching methods,strengthening the training of the teacher,strengthening medical humanities education,paying attention to training students clinical thinking ability and improving patients’ compliance and so on.Finally,through the clinical novitiate,students could be trained to have certain clinical thinking ability of infectious diseases and build a bridge for medical students to transition into a qualified clinician.

Objective: This study aimed to investigate the prognostic significance of combined serum IL-2 and total bilirubin (TBIL) on liver failure in patients with primary biliary cirrhosis (PBC). Methods: A total of 160 PBC patients who were treated with UDCA therapy was retrospectively analyzed. The demographic data and laboratory parameters at admission were collected, and the COX regression model was used to predict independent risk factors related to the progression of PBC disease. The optimal cut-off values and prognosis effects of serum IL-2 and TBIL were identified based on the time-dependent receiver operating characteristic (ROC) curve. The incidence of liver failure was also analyzed with Kaplan-Meier survival analysis. In addition, a cytokine detection was performed to observe the changes of cytokines (mainly IL-2) with liver tissue and blood samples from 11 patients with end stage PBC liver failure and 5 healthy control patients. Results: Age, IL-2, ALB,γ-GT, ALP, TBIL, Hb, TBA, WBC, and PLT, as well as anti-Sp100, were found to be independent risk factors in PBC patients with liver failure. Patients with decreased serum IL-2 levels and increased TBIL levels have a significant higher incidence of liver failure, a lower survival rate, and a worse prognosis. Patients with advanced liver transplantation with PBC liver failure exhibited a significant decrease in the level of serum IL-2and a relatively immunosuppressed status. Conclusions: The combination of serum IL-2 and TBIL can be a predictor of progression of liver failure in patients with primary biliary cholangitis, and it is likely to be related to the expression of GM-CSF and G-CSF.

目的 探讨肝硬化合并糖尿病患者应用胰岛素强化治疗后血糖及临床指标的变化特点.方法 以首都医科大学附属北京佑安医院2018年11月 ～2021年7月收治的肝硬化合并糖尿病患者共52例为研究对象,分为代偿期肝硬化组(n=16)和失代偿期肝硬化组(n=36),在糖尿病饮食基础上进行胰岛素强化治疗.在基线及强化治疗1周后进行空腹血糖、餐后2h末梢血糖、肝功能、凝血功能和血常规等指标评估.结果 代偿期肝硬化组经过胰岛素强化治疗1周后,与基线比较,lnALT、空腹血糖及餐后2h末梢血糖均明显下降(3.45±0.58IU/L vs 3.71±0.99IU/L,7.32±1.55mmol/L vs 12.29±1.93mmol/L,9.89±1.84mmol/L vs 28.30±1.45mmol/L;P均<0.05).失代偿期肝硬化组经过胰岛素强化治疗1周后,与基线比较,lnALT、空腹血糖及餐后2h末梢血糖均明显下降(3.04±0.89IU/L vs 3.36±1.00IU/L,8.63±2.95mmol/L vs 13.76±3.04mmol/L,12.87±1.77mmol/L vs 27.98±2.14mmol/L;P均<0.05),白蛋白明显上升(32.61±4.49g/L vs 29.39±5.66g/L;P<0.05),但餐后2h末梢血糖仍高于代偿期肝硬化组(P<0.05).两组胰岛素强化治疗前后比较,谷草转氨酶(AST)、总胆红素(TBil)、胆碱酯酶、凝血酶原活动度、白细胞计数、血红蛋白和血小板计数差异无统计学意义(P均>0.05).结论 胰岛素强化治疗能够有效降低肝硬化合并糖尿病患者血糖,对于失代偿期肝硬化患者仍需更长时间的监测及调整才能使血糖达标.

Artificial intelligence (AI)-aided general clinical diagnosis is helpful to primary clinicians. Machine learning approaches have problems of generalization, interpretability, etc. Dynamic Uncertain Causality Graph (DUCG) based on uncertain casual knowledge provided by clinical experts does not have these problems. This paper extends DUCG to include the representation and inference algorithm for non-causal classification relationships. As a part of general clinical diagnoses, six knowledge bases corresponding to six chief complaints (arthralgia, dyspnea, cough and expectoration, epistaxis, fever with rash and abdominal pain) were constructed through constructing subgraphs relevant to a chief complaint separately and synthesizing them together as the knowledge base of the chief complaint. A subgraph represents variables and causalities related to a single disease that may cause the chief complaint, regardless of which hospital department the disease belongs to. Verified by two groups of third-party hospitals independently, total diagnostic precisions of the six knowledge bases ranged in 96.5–100%, in which the precision for every disease was no less than 80%.

Background In primary biliary cholangitis (PBC), the levels of serum IL-2 were involved in liver inflammation and immune changes. This study aimed to investigate the prognostic significance of serum IL-2 combined with total bilirubin (TBIL) in liver failure and cytokine changes during the disease. Methods A total of 160 PBC patients treated with UDCA were included. Parameters at admission were collected, and the COX regression model was used to predict independent risk factors associated with PBC disease progression. We identified the optimal cut-off values and prognosis effects of serum IL-2 and TBIL based on the time-dependent receiver operating characteristic (ROC) curve. We also analyzed the incidence of liver failure with Kaplan-Meier survival analysis. In addition, the changes of cytokines (mainly IL-2) in liver tissues and blood samples from 11 patients with end-stage PBC liver failure and five healthy controls were examined. Results Age, IL-2, ALB, γ-GT, ALP, TBIL, Hb, TBA, WBC, and PLT, as well as anti-Sp100, were found to be independent risk factors in PBC patients with liver failure. Patients with decreased serum IL-2 levels and increased TBIL levels have a significantly higher incidence of liver failure and a worse prognosis. Patients with advanced PBC liver failure after liver transplantation exhibited a significant decrease in levels of serum IL-2 and a relatively immunosuppressed status. Conclusions The combination of serum IL-2 and TBIL can be a predictor of the progression of liver failure in patients with primary biliary cholangitis, and it is likely to be related to the expression of GM-CSF and G-CSF.

BACKGROUND Vitamin D deficiency is universal among patients with chronic liver disease. Vitamin D may be involved in the regulation of immune function of chronic hepatitis B and related to disease progression. METHODS The study was a cross-sectional study. The level of vitamin 25(OH)D was detected in patients with chronic hepatitis B, hepatitis B cirrhosis, hepatitis B cancer, and healthy groups by isotope dilution liquid chromatography tandem mass spectrometry (LC-MS/MS). At the same time, the clinical data, biochemical indexes, and T lymphocyte subsets were collected to study the relationship between vitamin 25(OH)D deficiency and clinical indexes of hepatitis B patients. RESULTS The prevalence of vitamin D deficiency (< 20 ng/mL) was higher in patients with liver cancer group (96.97%, 10.59 ± 3.06 ng/mL) and cirrhosis group (93.18%, 11.85 ± 2.66 ng/mL) than in the healthy group (76.92%, 16.38 ± 5.53 ng/mL) and chronic hepatitis B group (77.83%, 15.06 ± 4.91 ng/mL). There were significant differences in vitamin 25(OH)D levels between the cirrhosis groups and the healthy groups, the liver cancer groups and the healthy groups, the hepatitis B cirrhosis groups and the chronic hepatitis B groups, the liver cancer groups and the chronic hepatitis B groups (p < 0.05). There was no significant difference in vitamin 25 (OH)D level between liver cancer group and hepatitis B cirrhosis group, healthy group and chronic hepatitis B group (p > 0.05). Vitamin 25(OH)D level was correlated with age (r = -0.24, p = 0.015), lymphocyte (r = 0.24, p = 0.015), hemoglobin (r = 0.28, p = 0.005), platelet (r = 0.27, p = 0.006), PTA (r = 0.33, p = 0.001), albumin (r = 0.30, p = 0.002), prealbumin (r = 0.39, p = 0.001), cholinesterase (r = 0.29, p = 0.003), CD3+ (r = 0.20, p = 0.04), CD3+ CD8+ (r = 0.20, p = 0.04), CD45+ (r = 0.24, p = 0.017), but none correlated with liver function and HBV-DNA. CONCLUSIONS Vitamin D deficiency existed in patients with hepatitis B, which was related to the clinical progress of hepatitis B and may be involved in the regulation of immune function in patients with chronic HBV infection.

目的 观察醋酸甲羟孕酮、戊酸雌二醇治疗围绝经期综合征的效果及对雌激素水平、子宫内膜的影响,并评价其安全性.方法 回顾性分析2018年2月—2021年1月收治的围绝经期综合征79例的临床资料,按照治疗方式分为研究组41例和对照组38例,研究组给予戊酸雌二醇治疗,对照组给予醋酸甲羟孕酮治疗.比较2组临床疗效、雌激素水平、子宫内膜厚度及子宫体积,记录2组不良反应发生情况.结果 研究组总有效率高于对照组(P<0.05).治疗后,2组雌二醇均较治疗前升高,卵泡刺激素和黄体生成素均较治疗前下降,且研究组优于对照组(P<0.05,P<0.01).2组治疗后子宫内膜厚度均较治疗前增加(P<0.05).2组不良反应发生率比较差异无统计学意义(P>0.05).结论 围绝经期综合征患者予戊酸雌二醇治疗效果显著,可有效改善雌激素水平,且安全性较高.

新发感染病(emerging infectious disease,EID)是指20世纪70年代以来出现的,存在地域性、突发性、不确定性等特点.目前新发感染病的教学内容存在缺陷,授课教师实践经验不足,传统教学方式不能应对新发感染病的教学需求.因此,亟需进一步改进EID课程的教学方式,改善EID学科的教学效果.本研究对EID的流行病学特征和教学现状进行分析,探索归纳了新的教学方式在EID教学中的优势,以期为EID的教学改进提供新思路.

BackgroundThe human leukocyte antigen (HLA) susceptibility gene is the main genetic risk factor for primary biliary cholangitis (PBC). The prognosis of patients with PBC is linked to gut microbiota dysbiosis. However, whether the HLA alleles are associated with the gut microbiota distribution and disease severity remains unknown. MethodsA cohort of 964 Chinese patients with PBC was enrolled at Beijing YouAn Hospital, Beijing, China. High-resolution genotyping of the HLA class I and class II loci from 151 of these patients was performed using sequence-based PCR. Stool samples were collected from 43 of the 151 fully HLA-typed patients to analyze their microbiota compositions via 16S RNA gene sequencing. ResultsOf the 964 patients, the male:female ratio was 114:850, and 342 of these patients (35.5%) had already developed liver cirrhosis (LC) before enrollment. Patients with PBC showed a significantly higher frequency of HLA DRB1*08:03 than did the controls (21.2% vs. 9.0%, P=0.0001). HLA-DRB1*03:01, DRB1*07:01, DRB1*14:05, and DRB1*14:54 frequencies were also increased but did not reach significance after Bonferroni's correction. Conversely, the DQB1*03:01 frequency was significantly lower in patients with PBC than in the controls (24.5% vs. 39.2%, P=0.0010). The patients' gut microbiota were analyzed from four perspectives. The microbial community abundances were significantly lower in FHRAC-positive patients (patients with a combination of five HLA DRB1 high-risk alleles) than in FHRAC-negative patients (P<0.05). Of the top 10 microbial genera, Lachnospiraceae_incertae_sedis was higher in the FHRAC-positive patients than in the FHRAC-negative patients (P<0.05). linear discriminant analysis (LDA) effect-size (LEfSe) analysis showed different microbes at different levels in the FHRAC-negative patients but not in the FHRAC-positive patients. DQB1*03:01-positive patients contained mostly Lactobacillaceae at the family level. A comparison of the FHRAC-positive patients with and without liver cirrhosis showed that the abundances of Veillonella were significantly higher in patients with cirrhosis and FHRAC than in those without cirrhosis and are FHRAC-negative. ConclusionThe HLA class II genes may influence the gut microbiota compositions in patients with PBC. Differential gut microbiota were expressed at different taxonomic levels. Some bacterial abundances may be increased in FHRAC-positive patients with PBC and cirrhosis.

Background The COVID-19 has caused significant toll over the globe. Pregnant women are at risk of infection. The present study examined the frequency of washing hands with soap and wearing face mask when going out, prevalence of depression and anxiety, and identified their associated factors among pregnant women during the early phase of COVID-19 outbreak in China. Methods A cross-sectional online survey was conducted between 24 February and 3 March 2020. A total of 15 428 pregnant women who were using maternal health care services in China completed a questionnaire which assessed their socio-demographic and pregnancy-related characteristics, contextual, cognitive and social factors related to COVID-19, frequency of washing hands and wearing face masks, and depression and anxiety. Logistics regression analyses were performed to identify the associated factors of preventive behaviours and mental health. Results The prevalence of probable anxiety and depression was 28.2% and 43.6% respectively. 19.8% reported always wearing face mask when going out, and 19.1% reported washing hands with soap for more than 10 times per day. Results from logistic regression analyses showed that older age was associated with lower levels of depression and anxiety ( OR = 0.42–0.67) and higher frequency of washing hands ( OR = 1.57–3.40). Higher level of education level was associated with probable depression ( OR = 1.31–1.45) and higher frequency of wearing face mask ( OR = 1.50–1.57). After adjusting for significant socio-demographic and pregnancy-related factors, place of residence being locked down (a OR = 1.10–1.11), being quarantined (a OR = 1.42–1.57), personally knowing someone being infected with COVID-19 (a OR = 1.80–1.92), perception that COVID-19 would pose long term physical harm to human (a OR = 1.25–1.28) were associated with higher levels of depression and anxiety, while the perception that the disease will be under control in the coming month was associated with lower levels of depression and anxiety (a OR = 0.59–0.63) and lower tendency of always wearing face mask (a OR = 0.85). Social support was associated with lower levels of depression and anxiety (a OR = 0.86–0,87) and higher frequency of washing hands (a OR = 1.06). Conclusions The mental health and preventive behaviours of pregnant women during COVID-19 outbreak was associated with a range of socio-demographic, pregnancy-related, contextual, cognitive and social factors. Interventions to mitigate their mental health problems and to promote preventive behaviours are highly warranted.

目的 分析首诊于眼科的成人非外伤性、非医源性霍纳综合征(Horner syndrome,HS)的临床特点及病因.设计回顾性病例系列.研究对象2018年1月至2020年6月首诊于眼科的HS患者7例.方法 回顾患者的临床及影像学资料,分析其临床表现特点,病变位置及病因.主要指标起病形式、HS三主征、其他临床表现及影像学检查结果.结果 7例患者中6例男性,1例女性为垂体瘤卒中.发病年龄41～60岁.所有患者均有明显的神经眼科体征,突眼2例、动眼神经受累2例、外展神经受累2例、三叉神经第一支受累2例.6例急性或亚急性起病,1例慢性病程.双眼睑裂大小相差2～4 mm,瞳孔大小相差1～1.5 mm.颈交感神经三级神经元的病变累及颈内动脉和海绵窦3例,分别为恶性肿瘤、瘤卒中及动静脉瘘;均无颜面部汗腺分泌异常,有2例伴有明显的局部疼痛.二级神经元受累2例,均为恶性肿瘤,肺尖部病变和甲状腺病变累及颈总动脉各1例,有颜面部汗腺分泌异常,但均无疼痛及神经眼科、神经系统其他体征.交感神经通路一级神经元,即丘脑至颈8～胸2(脊睫中枢)通路病变2例,延髓梗死及神经系统脱髓鞘疾病各1例;均有颜面部汗腺分泌异常,并伴有明显神经系统其他阳性体征.结论 首诊于眼科的非创伤性HS起病形式、症状及其他神经系统受损表现对疾病有重要的定位及定性诊断意义.详细的神经眼科查体及早确认体征对于恶性肿瘤疾病、侵袭动脉及海绵窦的HS尤为重要.

目的:探讨原发性胆汁性胆管炎(PBC)病人能量代谢特点.方法:选取2016年5月至2019年4月首都医科大学附属北京佑安医院肝病科收治的33例PBC病人为研究对象,同时以11例健康体检者为对照.采用间接能量代谢测定仪进行两组能量代谢相关指标测定,包括实测静息能量消耗(REE)、预测REE(pREE)、呼吸商(RQ)、碳水化合物氧化率(CHO％)、蛋白质氧化率(PRO％)及脂肪氧化率(FAT％).结果:PBC组REE显著低于健康对照组[(1110.67±289.43) kcal/d vs(1410.00±272.07)kcal/d,P=0.004].PBC组REE/pREE(％)为(85.55±20.83)％,呈低代谢状态,显著低于健康对照组(P=0.015).PBC组RQ显著低于健康对照组[(0.82±0.06)vs(0.87±0.04),P=0.001].PBC组CHO％显著低于健康对照组(P=0.001),FAT％显著高于健康对照组(P=0.006),PRO％显著高于健康对照组(P=0.005).与Child-PughA级及Child-PughB级病人相比,Child-PughC级病人REE/pREE(％)最低,为(76.67±7.37)％,低代谢状态更为显著.Child-Pugh B级及C级RQ值及CHO％显著低于Child-PughA级,营养物质代谢紊乱更为突出.结论:PBC病人实测REE偏低,呈低代谢状态,存在营养物质代谢紊乱,与肝损伤程度相关,需注意制定适宜的营养支持方案.

Background Hepatitis B virus-related acute-on-chronic liver failure (HBV-ACLF) is a severe condition with high mortality due to lack of efficient therapy. Until now, the use of methylprednisolone (MP) in HBV-ACLF is still controversial. We aimed to evaluate the efficacy and safety of MP in HBV-ACLF. Methods Totally 171 HBV-ACLF patients from three medical centers were randomly allocated into MP group (83 patients treated with MP intravenously guttae for 7 days plus standard treatment: 1.5 mg/kg/day [day 1–3], 1 mg/kg/day [day 4–5], and 0.5 mg/kg/day [day 6–7]) and control group (88 patients treated with standard treatment). The primary endpoints were 6-month mortality and prognostic factors for 6-month survival. The survival time, cause of death, adverse events, liver function, and HBV DNA replication were analyzed. Results The 6-month mortality was significantly lower in MP group than control group [32.4% vs. 42.5%, P = 0.0037]. MP treatment was an independent prognostic factor for 6-month survival [HR (95% CI) 0.547(0.308–0.973); P = 0.040]. Factors associated with reduced 6-month mortality in MP group included HBV DNA and lymphocyte/monocyte ratio (LMR) ( P < 0.05). Based on ROC curve, LMR+MELD had a better predictive value for prognosis of HBV-ACLF under MP treatment. No significant difference in HBV DNA replication was observed between groups ( P > 0.05). Conclusions MP therapy is an effective and safe clinical strategy in HBV-ACLF, increasing the 6-month survival rate. Clinical trials registered at http://www.chictr.org.cn as ChiCTR-TRC-13003113 registered on 16 March 2013.