Objective To report two cases of familial Gitelman syndrome and literature review regarding the updates of relevant genes,classification,treatment,and prognosis.Methods The clinical data of two sisters with Gitelman syndrome were retrospectively analyzed.Results Their blood pressures were within normal range.Hypokalaemic alkalosis,hypomagnesemia,and hypocalciuria were corrected almost completely after three days of intravenous magnesium and potassium infusion,spirolactone and indometacin.However,the maintenance of normal potassium was unsuccessful over one year.Conclusion Hypokalaemic alkalosis,hypomagnesemia,and hypocalciuria were normalized in Gitelman syndrome.There was some debate in regard to using PGE2 synthetase inhibitors.Tolerance of long-term medication will be the big challenge for curative effect.

Direct-acting antiviral agents (DAAs) are the main antiviral therapeutics for hepatitis C virus-related decompensated stage cirrhosis. DAAs of NS3/4A protease inhibitors use is not recommended for patients with decompensated cirrhosis due to characteristics of DAAs metabolism in liver. The recent guidelines have recommended sofosbuvir (SOF)-based plan including pan-genotype plan of sofosbuvir(SOF)/velpatasvir (VEL), sofosbuvir combined with daclatasvir (DCV), genotype 1,4,5,6 specific plan of sofosbuvir (SOF) / ledipasvir (LDV) for 24 weeks or above in combination with ribavirin for 12 weeks because NS5B and NS5A inhibitors has no obvious effect on CYP450 enzyme system and achievement of sustained virological response (SVR) rates at 12/24 weeks is achievable in 88% ~ 100%, and liver reserve function improves in 42% ~ 53% of patients. Furthermore, approximately 15.5% ~ 49% of patients waiting for liver transplantation after treatment with DAAs do not require liver transplantation for short-term and 10.3% ~19.2% of patients receiving SOF/LDV, and SOF combined with DCV not needed liver transplantation. Thus, the clinical application of DAAs provides a safe and reliable antiviral treatment plan for hepatitis C virus-related decompensated stage cirrhosis.

Objective: To explore the clinical value of plasma heme oxygenase 1(HO-1) in the development of non-alcoholic fatty liver disease(NAFLD). Methods: Patients with NAFLD were selected from the Physical examination center and the Department of Traditional and Western Medical Hepatology of Third Hospital of Hebei Medical University. A combination of ultrasound and liver elastography was used to screen NAFLD patients and healthy persons. General clinical characteristics, peripheral blood cell count and liver biochemical test results were collected synchronously, plasma samples were retained, and plasma HO-1 level was detected by enzyme-linked immunosorbent assay. SPSS21.0 statistical software was used for statistical analysis, multivariate logistic regression analyses was used to analyse the independent risk factors affecting the incidence and progression of NAFLD. The diagnostic efficacy of indicators related to development of NAFLD was assessed by the receiver operating characteristic curve(ROC). Results: A total of 328 patients with NAFLD and 113 healthy controls were included. According to the liver biochemical results, the NAFLD group was divided into 148 patients with normal liver enzymes and 180 patients with abnormal liver enzymes. The level of HO-1 in the three groups was 9.09 ± 2.19, 14.38 ± 2.63, 17.00 ± 3.30 ng/ml, and was increased respectively of healthy controls, patients with normal liver enzymes and patients with abnormal liver enzymes. Analyzing plasma HO-1 levels of components associated with metabolic disorders suggests that components without metabolic syndrome(9.83 ± 3.21) < components with 1 metabolic syndrome(13.59 ± 3.72) < components with 2 or more metabolic syndrome(16.09 ± 3.41), P < 0.001. The results of HO-1 level stratification analysis showed that WBC, ALT, AST, GGT, TG increased as HO-1 level increased, and the pairwise difference was statistically significant (P < 0.001). The WBC count of NAFLD is significantly higher than healthy group(6.79 ± 1.62 vs 5.68 ± 1.36, P < 0.001). The univariate and multivariate regression analyses of all the subjects showed that HO-1, TG and BMI were prognostic factors for the occurrence of NAFLD and HO-1, TC, GLU were prognostic factors for the progression of NAFLD, P < 0.05. The ROC analysis showed that HO-1 was reliable markers for predicting the occurrence and progression of NALFD, the sensitivity and specificity were respectively 85.10%, 92.90% and 38.33%, 95.27%. Conclusion Plasma HO-1 can predict the occurrence and progression of NAFLD and is expected to be a novel molecular diagnostic marker for NAFLD and NASH.

Objective:To compare the efficacy and safety of robot-assisted laparoscopic and laparoscopic partial nephrectomy (RAPN and LPN) for patients with completely endophytic renal tumor.Methods:A total of 73 patients with completely endophytic renal tumor receiving RAPN (n=29) or LPN (n=44) in our center between January 2015 and June 2021 were retrospectively collected. There were 21 males and 8 females in RAPN group. The average age was 48.6±13.7 years old, average tumor size was 2.9±0.9 cm with 13 left tumors and 16 right tumors, average R. E.N.A.L. score was 9.2±1.0, and average preoperative eGFR was 82.6±10.7 ml/(min·1.73 m 2). There were 27 males and 17 females in LPN group. The average age was 50.1±12.3 years old, average tumor size was 2.9±0.9 cm with 24 left tumors and 20 right tumors, average R. E.N.A.L. score was 9.1±1.3, and average preoperative eGFR was 81.7±9.6 ml/(min·1.73 m 2). There was no significant difference in above variables between two groups. The operative time, warm ischemia time, blood loss, postoperative complication, postoperative hospital stay and postoperative 3 months renal function of two groups were compared. Results:All 73 patients successfully underwent RAPN or LPN and no patient converted to radical nephrectomy or open surgery. There was no significant difference in operation time [140(80, 160) min vs. 150 (90, 180) min, P=0.264], intraoperative estimated blood loss[150 (100, 200)vs. 180 (120, 200) ml, P=0.576]and postoperative hospital stay (7.0±2.7 vs. 7.4±2.1 days, P=0.480) between two groups. Compared with LPN group, RAPN group had obvious less warm ischemia time (23.1±3.3 vs. 27.6±4.7 min, P<0.001). No obvious complication occurred in RAPN group and one case with postoperative hemorrhage occurred in LPN group. No positive margin occurred in either group. There was no difference in renal function 3 months after operation between the two groups [73.2±6.3 vs.70.5±7.6ml/(min·1.73 m 2), P=0.117]. The median follow-up period was 22.6 months with no tumor recurrence or metastasis. Conclusions:For experienced surgeons, both RAPN and LPN are safe and feasible for patients with completely endophytic renal tumor. Compared with LPN, RAPN has advantages of perioperative curative effect, which could reduce the operating difficulty and shorten the warm ischemia time.

Objective To explore the clinical value of plasma heme oxygenase l(HO-l)in the development of non-alcoholic fatty liver disease(NAFLD).Methods Patients with NAFLD were selected from the Physical examination center and the Department of Traditional and Western Medical Hepatology of Third Hospital of Hebei Medical University.A combination of ultrasound and liver elastography was used to screen NAFLD patients and healthy persons.General clinical characteristics,peripheral blood cell count and liver biochemical test results were collected synchronously,plasma samples were retained,and plasma HO-1 level was detected by enzyme-linked immunosorbent assay.SPSS21.0 statistical software was used for statistical analysis,multivariate logistic regression analyses was used to analyse the independent risk factors affecting the incidence and progression of NAFLD.The diagnostic efficacy of indicators related to development of NAFLD was assessed by the receiver operating characteristic curve(ROC).Results A total of 328 patients with NAFLD and 113 healthy controls were included.According to the liver biochemical results,the NAFLD group was divided into 148 patients with normal liver enzymes and 180 patients with abnormal liver enzymes.The level of HO-1 in the three groups was 9.09±2,19,14.38±2.63,17.00±3.30 ng/ml,and was increased respectively of healthy controls,patients with normal liver enzymes and patients with abnormal liver enzymes.Analyzing plasma HO-1 levels of components associated with metabolic disorders suggests that components without metabolic syndrome(9.83±3.21)

Objective To conduct a scoping review of virtual reality motor sensing exercise in the elderly with dementia,so as to identify the types of intervention,outcome indicators,evaluation tools and application effects of virtual reality motor sensing exercise and provide guidance for future research.Methods A literature search was performed in the PubMed,Embase,CINAHL,Web of Science,Cochrane Library,CNKI,Wanfang,VIP,and CMB databases to identify relevant studies.The search period is from the establishment of databases to October 15,2023.The included studies were summarized and analyzed.Results A total of 18 articles were included in this study,including 14 randomised controlled trials,one class-experimental study and three mixed studies involving a total of 1,054 elderly dementia patients.The types of virtual reality motor sensing exercise included motor sensing interactive games,interactive bicycle riding,remote game exercise,Wii Fit exercise and virtual reality environment exercise.The outcome indicators involve 4 aspects of physical indicators,psychological indicators,cognitive function and quality of life.Conclusion Virtual reality motor sensing exercise is feasible and effective in the elderly with dementia.However,large sample and high-quality randomized controlled trials are still needed in the future to verify the feasibility and application effect of virtual reality exercise in the elderly with dementia,so as to provide evidence-based basis for formulating the best exercise intervention program for the elderly with dementia.

Decellularized matrix transplantation has emerged as a promising therapeutic approach for repairing tissue defects, with numerous studies assessing its safety and efficacy in both animal models and clinical settings. The host immune response elicited by decellularized matrix grafts of natural biological origin plays a crucial role in determining the success of tissue repair, influenced by matrix heterogeneity and the inflammatory microenvironment of the wound. However, the specific immunologic mechanisms underlying the interaction between decellularized matrix grafts and the host immune system remain elusive. This article reviews the sources of decellularized matrices, available decellularization techniques, and residual immunogenic components. It focuses on the host immune response following decellularized matrix transplantation, with emphasis on the key mechanisms of Toll-like receptor, T-cell receptor, and TGF-β/SMAD signaling in the stages of post-transplantation immunorecognition, immunomodulation, and tissue repair, respectively. Furthermore, it highlights the innovative roles of TLR10 and miR-29a-3p in improving transplantation outcomes. An in-depth understanding of the molecular mechanisms underlying the host immune response after decellularized matrix transplantation provides new directions for the repair of tissue defects.

Myositis antibodies are divided into myositis-specific autoantibodies(MSA)and myositis-associated autoantibodies(MAA).There are heterogeneity in the mechanism,clinical features and prognosis of interstitial lung disease(ILD)in the different myositis antibodies.In MSA,anti-melanoma differentiation-related gene 5(MDA5)antibody and anti-aminoacyl synthetase(ARS)antibody are highly correlated with the occurrence of ILD.Patients with MDA5+DM-ILD usually have a rapidly progressive and poor prognosis.The progress of ILD in ARS+DM patients was slow,and the response to treatment is good,but it is easy to relapse.In MAA,anti-Ro52 antibodies often coexist with MSA antibodies,and clinical manifestation is closely related to coexisting antibodies.This review has summarized roles of myositis antibodies in ILD.

AIM:To investigate the effect of Huangqi-Danggui decoction(HQDG)on the brain tissue of rats with cerebral ischemia/reperfusion(I/R)injury for 7 d by regulating macroautophagy and chaperone-mediated autophagy(CMA),and to explore its mechanism.METHODS:Male SD rats were randomly divided into sham group,model group,HQDG group and Xuesaitong(XST)group.Determination of main chemical components of HQDG by liquid chro-matography-mass spectrometry.The model of middle cerebral artery occlusion/reperfusion in rats was established by the left modified thread embolism method,and the changes of cerebral blood flow were observed by laser speckle blood flow imager.Zea Longa score was used to observe the neurological deficit.HE staining was used to observe the degree of nerve cell injury.The changes of neurovascular unit and autophagosomes in brain tissue were observed by transmission electron microscopy.Immunohistochemical method was used to detect the expression of LC3,P62,lysosome-associated membrane protein-2A(LAMP-2A),heat shock protein 70(HSP70)and myocyte enhancer factor 2D(MEF2D)proteins.Western blot was used to detect the expression of autophagy-related proteins P62 and LC3-Ⅱ/LC3-I.RESULTS:Compared with the sham group,the neurological deficit score in model group was significantly higher(P<0.01).A large number of nerve cells showed necrosis and nuclear dissolution,with the cell arrangement being disordered.The number of autophagosomes increased.The protein expression levels of LC3,LAMP-2A,HSP70 and MEF2D in brain tissue increased,while the ex-pression level of P62 protein decreased(P<0.05 or P<0.01).Compared with the model group,the scores of neurological deficit in brain tissue in HQDG and XST groups were significantly lower(P<0.01).Cell damage was significantly re-duced.The number of autophagosomes further increased.The expression levels of LAMP-2A,HSP70,MEF2D and P62 proteins in brain tissue decreased,while the expression levels of LC3-Ⅱ/LC3-I protein increased(P<0.05 or P<0.01).CONCLUSION:HQDG can alleviate cerebral ischemia/reperfusion injury in rats and exert neuroprotective effects by ac-tivating macroautophagy and reducing CMA.

Objective:To study the bacterial pathogen, the optimal plan of antibacterial drugs and the prognostic factors in patients with liver cirrhosis combined with bacterial pneumonia.Methods:Data of 324 cases with liver cirrhosis from the Department of Traditional and Western Medical Hepatology, the Third Hospital of Hebei Medical University were collected, including 217 cases of bacterial pneumonia. Baseline characteristics of the patients, factors affecting the efficacy of antibacterial treatment and prognosis were compared and analyzed. Logistic regression analysis was used to screen and predict the antibacterial efficacy indicators and a prediction model was established. Receiver operating characteristic curve was used to evaluate the value of the established model and Child-Turcortte-Pugh, model for end-stage liver disease, and model for end-stage liver disease combined with serum sodium concentration predict the therapeutic efficacy.Results:Chronic HBV and HCV infections were the main causes of cirrhosis, followed by cryptogenic cirrhosis and alcoholic cirrhosis. Diabetes, cardio-cerebrovascular and chronic obstructive pulmonary disease were susceptible factors for bacterial pneumonia. As infection occurred, the ratio of neutrophils to lymphocytes, serum C-reactive protein, procalcitonin, alanine aminotransferase, and total bilirubin levels had increased significantly. The results of pathogenic analysis showed that the top three pathogenic bacteria were Klebsiella pneumoniae, Acinetobacter baumannii, Pseudomonas aeruginosa and Staphylococcus aureus. The resistance rate of Klebsiella pneumoniae to ceftriaxone was 50.0%, and that of ceftazidime, cefepime, and cefoperazone sulbactam were 27.8%. Imipenem and piperacillin tazobactam containing β-lactamase inhibitors were the most effective antibacterial therapies. Regression analysis showed that age, procalcitonin, and albumin was significantly correlated with antibacterial effects. The PAA model was established and had predicted the efficacy of Child-Turcortte-Pugh, model for end-stage liver disease, and model for end-stage liver disease combined with serum sodium. The specificity and sensitivity of the PAA was confirmed to be 94.12% and 93.62%, which was significantly higher than other models.Conclusion:The main common pathogenic bacterium of cirrhosis combined with bacterial pneumonia is Klebsiella pneumonia (G-bacilli). In addition, gram positive cocci (Staphylococcus aureus) and other are also visible. The elderly, diabetics and patients using hormones are prone to secondary fungal infections. Age, procalcitonin and serum albumin can accurately predict the antibacterial effect, guide clinical treatment and judge the prognosis of the established PAA model.