In 2015,the International Ascites Club proposed a new definition of hepatorenal syndrome-acute kidney injury based on the progression of hepatorenal syndrome,and studies are still being conducted to explore the exact pathogenesis of hepatorenal syndrome-acute kidney injury.Intestinal barrier plays an important bridging role in liver-kidney connection,and intestinal flora disturbance,bacterial translocation,and endotoxins entering the blood cause damage to the kidneys by releasing proinflammatory cytokines and activating immune-related cells.The entrance of bile acid into the circulation system also directly or indirectly lead to the development and progression of hepatorenal syndrome-acute kidney injury.This article reviews the crosstalk mechanism of hepatorenal syndrome-acute kidney injury from the perspective of the intestinal barrier and further clarifies the key role of the liver-gut-kidney axis in the pathogenesis of this disease,in order to provide new treatment ideas.

Clinical research on rare diseases has always faced multiple challenges in clinical research design and implementation due to small sample sizes of patients,high heterogeneity,and limited research resources.The rapid development of digital intelligence technology has provided innovative solutions for rare disease research.This article systematically explores the current status and response strategies of clinical research on rare diseases in the digital intelligence age.On the one hand,the efficiency of rare disease research has been optimized through adaptive design,mixed trial mode,and precision medicine stratification methods.On the other hand,solutions based on digital technology have been proposed to address the practical challenges of recruitment difficulties and underrepresentation of rare disease clinical research patients,data management and technical barriers,and insufficient coverage of natural medical history and baseline databases through digital intelligence technology.By combining international collaboration,intelligent screening,and remote experiments,a multidisciplinary collaboration and international cooperation,adaptive design,digital data platform,and patient-centered remote research model have been constructed as the core implementation strategies.Typical cases demonstrate that digital intelligence technology not only effectively shortens the drug development cycle,but also significantly enhances patient benefits,providing a replicable practical paradigm for global rare disease research.The practice of digital platforms represented by the International Rare Disease Research Alliance and the China Rare Disease Diagnosis and Treatment Collaboration Network has further verified the feasibility and promotional value of the digitalization path.In summary,digital intelligence technology has shown considerable promise in overcoming the clinical research challenges of rare diseases and accelerating the development of treatment plans,providing systematic references for researchers,regulatory agencies,and patient organizations.It is expected to drive the clinical research of rare diseases towards a more efficient and accurate future.

In 2015,the International Ascites Club proposed a new definition of hepatorenal syndrome-acute kidney injury based on the progression of hepatorenal syndrome,and studies are still being conducted to explore the exact pathogenesis of hepatorenal syndrome-acute kidney injury.Intestinal barrier plays an important bridging role in liver-kidney connection,and intestinal flora disturbance,bacterial translocation,and endotoxins entering the blood cause damage to the kidneys by releasing proinflammatory cytokines and activating immune-related cells.The entrance of bile acid into the circulation system also directly or indirectly lead to the development and progression of hepatorenal syndrome-acute kidney injury.This article reviews the crosstalk mechanism of hepatorenal syndrome-acute kidney injury from the perspective of the intestinal barrier and further clarifies the key role of the liver-gut-kidney axis in the pathogenesis of this disease,in order to provide new treatment ideas.

Clinical research on rare diseases has always faced multiple challenges in clinical research design and implementation due to small sample sizes of patients,high heterogeneity,and limited research resources.The rapid development of digital intelligence technology has provided innovative solutions for rare disease research.This article systematically explores the current status and response strategies of clinical research on rare diseases in the digital intelligence age.On the one hand,the efficiency of rare disease research has been optimized through adaptive design,mixed trial mode,and precision medicine stratification methods.On the other hand,solutions based on digital technology have been proposed to address the practical challenges of recruitment difficulties and underrepresentation of rare disease clinical research patients,data management and technical barriers,and insufficient coverage of natural medical history and baseline databases through digital intelligence technology.By combining international collaboration,intelligent screening,and remote experiments,a multidisciplinary collaboration and international cooperation,adaptive design,digital data platform,and patient-centered remote research model have been constructed as the core implementation strategies.Typical cases demonstrate that digital intelligence technology not only effectively shortens the drug development cycle,but also significantly enhances patient benefits,providing a replicable practical paradigm for global rare disease research.The practice of digital platforms represented by the International Rare Disease Research Alliance and the China Rare Disease Diagnosis and Treatment Collaboration Network has further verified the feasibility and promotional value of the digitalization path.In summary,digital intelligence technology has shown considerable promise in overcoming the clinical research challenges of rare diseases and accelerating the development of treatment plans,providing systematic references for researchers,regulatory agencies,and patient organizations.It is expected to drive the clinical research of rare diseases towards a more efficient and accurate future.

Hepatosplenic candidiasis (HSC) is a rare type of candidiasis that can occur in patients with hematologic malignancies, hematopoietic stem cell transplantation. At present, there is still a lack of studies on HSC in patients with hematologic disorders. Based on The Chinese Guidelines for the Diagnosis and Treatment of Invasive Fungal Disease in Patients with Hematological Disorders and Cancers (the 6th revision), We retrospectively analyzed the clinical characteristics and prognosis of patients with HSC treated in Peking University Institute of Hematology from 2008 to 2022. Finally, eighteen patients were included, with 1 (5.6%) proven, 2 (11.1%) probable, and 15 (83.3%) possible HSC. Among them, 3 (16.7%) patients occurred after haploid hematopoietic stem cell transplantation and 15 (83.3%) patients occurred after chemotherapy. 6 (33.3%) patients had positive blood cultures, including 4 cases of Candida tropicalis and 2 cases of Candida albicans. At 4 weeks of antifungal therapy, 10 (58.8%) patients achieved partial response (PR), At 8 weeks, 1 (6.3%) patients achieved complete response and 10 (62.5%) patients achieved PR. At 6 months after diagnosis, 3 (16.7%) patients died of hematopoietic recurrence, and none of them died of HSC. As a rare fungal infection disease, HSC has a low positive rate of microbiological and histological examinations, a persistent treat cycle, and has difficulty in remission, reminding us of the need for vigilance in patients with hematopoietic disorders and persistent fever.

Objective:To evaluate the efficacy and safety of matched sibling donor allogeneic hematopoietic stem cell transplantation (allo-HSCT) for the treatment of myelofibrosis (MF).Methods:In this case series, the clinical data of 18 patients with MF who received allo-HSCT in the Department of Hematology, Peking University People′s Hospital from December 2008 to December 2023 were retrospectively studied. Kaplan-Meier survival analysis and competitive risk model were used to evaluate the probabilities of 3-year overall survival (OS), disease-free survival (DFS), cumulative incidence of relapse (CIR), and transplant related mortality (TRM). The transplant related complications were also analyzed.Results:Among the 18 patients included, there were 12 males and 6 females, with a median age of 50 (range: 28-64) years. All 18 patients achieved neutrophil engraftment, and the time of neutrophil engraftment [ M ( Q1, Q3)] was 16.0 (11.8, 18.0) days. Twelve patients achieved platelet engraftment, and the platelet engraftment time was 21.0 (16.2, 43.2) days. Six patients had grade Ⅱ to Ⅳ acute graft-versus-host disease (GVHD), and six patients had chronic GVHD. The 3-year OS rate and DFS rate after transplantation were 62.2% and 52.2%, respectively. The 3-year CIR and TRM were 29.7% and 24.6%, respectively. Four patients died during follow-up, with the main cause of death being infections. Conclusion:Matched sibling allo-HSCT is a feasible option for the treatment of MF.

Objective: To evaluate the efficacy and safety of oXiris hemofilter for septic shock patients. Methods: Clinical data of septic shock patients receiving continuous renal replacement therapy (CRRT) with oXiris hemofilter in department of surgical intensive care unit (SICU) of the First Affiliated Hospital of Xi'an Jiaotong University from March 1st, 2018 to July 20th, 2019 were retrospectively analyzed. The heart rate (HR), mean arterial pressure (MAP), oxygenation index (PaO₂/FiO₂), lactate (Lac), platelet count (PLT), serum procalcitonin (PCT), interleukin-6 (IL-6) and C-reactive protein (CRP), noradrenaline (NE) dosage, acute physiology and chronic health evaluationⅡ(APACHEⅡ) and sequential organ failure score (SOFA) were compared before and after oXiris treatment and the prognosis were also analyzed. Results: Six patients with septic shock were included [5 males, the average age was (56.3±11.8) years old]. A total of 13 oXiris hemofilter sets were performed during treatment. Compared with before treatment, the HR, IL-6 and CRP levels were significantly decreased after treatment [HR (bpm): 93.8±9.7 vs. 133.5±18.3, IL-6 (ng/L): 509.2±169.6 vs. 3 739.8±618.2, CRP (mg/L): 169.1±148.3 vs. 277.8±68.7, all P < 0.05], MAP, PaO₂/FiO₂ and PLT were significantly increased [MAP (mmHg, 1 mmHg = 0.133 kPa): 73.3±2.2 vs. 63.3±1.6, PaO₂/FiO₂ (mmHg): 166.8±40.4 vs. 95.1±56.2, PLT (×10⁹/L): 73.3±27.5 vs. 41.2±21.4, all P < 0.05]; meanwhile, NE dosage, APACHEⅡ and SOFA scores were significantly decreased [NE (μg·kg^-1·min^-1): 0.4±0.3 vs. 1.2±0.7, APACHEⅡ: 18.8±6.9 vs. 30.0±7.3, SOFA: 11.7±4.2 vs. 17.3±2.1, all P < 0.05]. Although Lac and PCT decreased after treatment, there was no significant difference [Lac (mmol/L): 3.5±2.1 vs. 6.1±3.2, PCT (μg/L): 37.7±48.3 vs. 85.1±32.8, both P > 0.05]. At the end, 3 of the 6 patients survived and the others were discharged again medical advice. The length of SICU stay was 3 to 23 days, with an average of (13.0±8.5) days. No adverse events occurred during the treatment. Conclusion oXiris hemofilter can effectively remove inflammatory mediators in circulation, significantly improve hemodynamic status and severity, and may be considered as a safe and reliable treatment modality for septic shock patients.

Objective To evaluate the efficacy and safety of oXiris hemofilter for septic shock patients. Methods Clinical data of septic shock patients receiving continuous renal replacement therapy (CRRT) with oXiris hemofilter in department of surgical intensive care unit (SICU) of the First Affiliated Hospital of Xi'an Jiaotong University from March 1st, 2018 to July 20th, 2019 were retrospectively analyzed. The heart rate (HR), mean arterial pressure (MAP), oxygenation index (PaO2/FiO2), lactate (Lac), platelet count (PLT), serum procalcitonin (PCT), interleukin-6 (IL-6) and C-reactive protein (CRP), noradrenaline (NE) dosage, acute physiology and chronic health evaluation Ⅱ(APACHEⅡ) and sequential organ failure score (SOFA) were compared before and after oXiris treatment and the prognosis were also analyzed. Results Six patients with septic shock were included [5 males, the average age was (56.3±11.8) years old]. A total of 13 oXiris hemofilter sets were performed during treatment. Compared with before treatment, the HR, IL-6 and CRP levels were significantly decreased after treatment [HR (bpm): 93.8±9.7 vs. 133.5± 18.3, IL-6 (ng/L): 509.2±169.6 vs. 3739.8±618.2, CRP (mg/L): 169.1±148.3 vs. 277.8±68.7, all P < 0.05], MAP, PaO2/FiO2 and PLT were significantly increased [MAP (mmHg, 1 mmHg = 0.133 kPa): 73.3±2.2 vs. 63.3±1.6, PaO2/FiO2 (mmHg): 166.8±40.4 vs. 95.1±56.2, PLT (×109/L): 73.3±27.5 vs. 41.2±21.4, all P < 0.05]; meanwhile, NE dosage, APACHEⅡ and SOFA scores were significantly decreased [NE (μg·kg-1·min-1): 0.4±0.3 vs. 1.2±0.7, APACHEⅡ:18.8±6.9 vs. 30.0±7.3, SOFA: 11.7±4.2 vs. 17.3±2.1, all P < 0.05]. Although Lac and PCT decreased after treatment, there was no significant difference [Lac (mmol/L): 3.5±2.1 vs. 6.1±3.2, PCT (μg/L): 37.7±48.3 vs. 85.1±32.8, both P > 0.05]. At the end, 3 of the 6 patients survived and the others were discharged again medical advice. The length of SICU stay was 3 to 23 days, with an average of (13.0±8.5) days. No adverse events occurred during the treatment. Conclusion oXiris hemofilter can effectively remove inflammatory mediators in circulation, significantly improve hemodynamic status and severity, and may be considered as a safe and reliable treatment modality for septic shock patients.

Objective To evaluate the clinical efficacy and safety of continuous renal replacement therapy (CRRT) in patients with severe acute pancreatitis (SAP) receiving percutaneous drainage (PCD). Methods Clinical data of SAP patients receiving PCD admitted to department of hepatobiliary surgery of the First Affiliated Hospital of Xi'an Jiaotong University from November 11th 2015 to May 13th 2018 were retrospectively analyzed. The patients were divided into CRRT group and control group according to whether or not receiving CRRT. Demographic data, relevant variables before and after PCD, complication and outcome were all compared. Results A total of 75 patients were included in the study, 30 were treated with application of CRRT and 45 without CRRT. ① There was no significant difference in gender, age, body mass index (BMI), medical history (smoking, drinking), complications (cardiovascular disease, chronic lung disease, diabetes, chronic renal insufficiency), etiology (gallstone, alcohol abuse, hyperlipidemia and others), or white blood cell count (WBC), C-reactive protein (CRP), serum procalcitonin (PCT), fluid resuscitation, mechanical ventilation, vasoactive agent or intra-abdominal pressure within 48 hours after admission between the two groups. However, acute physiology and chronic health evaluationⅡ(APACHEⅡ) score within 48 hours after admission of CRRT group was significantly higher than that of control group (18.3±4.5 vs. 12.8±6.2, P < 0.05). ② There was no significant difference in WBC, PCT, APACHEⅡ score or computed tomography severity index (CTSI) before PCD between the two groups. There was no significant difference in the position or times of PCD procedure between the two groups, but the time interval of PCD in the CRRT group was significantly longer than that in the control group (days: 19.4±5.4 vs. 12.8±2.2, P < 0.05). Meanwhile, there was no significant difference in drainage of fluid properties, incidence of abdominal bleeding, infection, gastrointestinal fistula, endoscopic removal of necrotic tissue, laparotomy for removal of necrotic tissue or the time from PCD to endoscopy or laparotomy between two groups. However, the length of intensive care unit (ICU) stay and the length of hospital stay in the CRRT group were significantly longer than those in the control group (days: 23.2±8.5 vs. 15.3±12.1, 51.2±21.2 vs. 31.2±14.0, both P < 0.01). ③ Kaplan-Meier survival analysis showed that there was no significant differences in 1-year or 3-year cumulative survival rates between the two groups (χ21 = 0.097, P1 = 0.755; χ22 = 0.013, P2 = 0.908). Conclusions CRRT is safe and feasible in the treatment of SAP patients receiving PCD procedure. It does not increase the risk of bleeding and may delay the time interval of PCD intervention. However, it may prolong the length of ICU stay and the length of hospital stay. It should be worthy of much attention for clinicians.

Objective To get an understanding of the patient experience in public hospitals nationwide, and to evaluate the implementation of the Action Plan to Improve Health Care. Methods Supported by the mobile technology, from September 6, 2017 to December 15, 2018, the authors conducted an online survey that measured the satisfaction of both inpatients and outpatients at secondary and tertiary hospitals across the country. 15 questions from six dimensions including registration experience, patient-doctor communication, nurse-patient communication, the healthcare signage system, responsiveness of care providers and privacy protection were prepared for outpatients, while 20 questions from nine aspects such as nurse-patient communication, patient-doctor communication, pain management, medication communication, admission and discharge information, responsiveness of care-givers, food service, friendliness to patient family, and the healthcare signage system were directed at inpatients. Descriptive statistical analysis was used to describe the basic features of the data. Results 9.18 million valid responses from outpatients and another 5.38 million from inpatients were obtained. The overall satisfaction rate with outpatient services had reached a score of 90.45 points where nurse-patient communication stands out as the top-rated dimension and privacy protection gets the lowest rating. On the other hand, the inpatient satisfaction stands at a score of 93.01 with friendliness to patient family receiving the top score and patient-doctor communication the lowest. Conclusions Despite the positive feedback Chinese patients give on the outpatient care they receive, we should make efforts to improve the outpatient care environment, the wayfinding system, privacy protection, and responsiveness of care-givers.