Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a non-atherosclerotic, non-amyloidogenic inherited cerebral small vessel disease caused by mutations in the NOTCH3 gene, typically manifesting in middle age. The diagnosis can be confirmed through the detection of granular osmiophilic material in skin biopsies and NOTCH3 gene mutations identified by genetic testing. However, the pathogenesis of this disease remains unclear, and treatment options are limited. A major contributing factor is that currently available preclinical animal models fail to adequately recapitulate the characteristic pathological features and clinical phenotypes of the disease. Therefore, a comprehensive review of recent advances in CADASIL animal models was conducted, and the advantages and limitations of existing models were systematically analyzed, aiming to offer guidance for selecting appropriate animal models in fundamental research and to propose future directions for developing experimental models.

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a non-atherosclerotic, non-amyloidogenic inherited cerebral small vessel disease caused by mutations in the NOTCH3 gene, typically manifesting in middle age. The diagnosis can be confirmed through the detection of granular osmiophilic material in skin biopsies and NOTCH3 gene mutations identified by genetic testing. However, the pathogenesis of this disease remains unclear, and treatment options are limited. A major contributing factor is that currently available preclinical animal models fail to adequately recapitulate the characteristic pathological features and clinical phenotypes of the disease. Therefore, a comprehensive review of recent advances in CADASIL animal models was conducted, and the advantages and limitations of existing models were systematically analyzed, aiming to offer guidance for selecting appropriate animal models in fundamental research and to propose future directions for developing experimental models.

Objective:To investigate the value of using ultrasound excited contrast agents to assess extracorporeal hydrostatic pressure on the basis of ultrasound contrast imaging.Methods:An extracorporeal hydrostatic pressure evaluation system was established. The changes in contrast intensity was first evaluated for the same concentration of microbubble contrast agent at ambient pressures of 20, 25, 30, 35, 40, and 45 cmH 2O. Contrast agents with the same initial intensity were placed at different pressures for 1 s, 3 s, and 5 s, and the percentage change in contrast agent intensity was analyzed to select the optimal excitation time. Finally, the contrast agent at different pressures was stimulated using an acoustic excitation device, and correlation analysis was performed using the Pearson product moment correlation coefficient. Linear regression analysis was used to establish the relationship between different pressures and the percentage change in intensity. Results:When the ambient pressure was varied under 6 gradients of 20, 25, 30, 35, 40, and 45 cmH 2O, the contrast strength decreased with the pressure increased, and there was a negative correlation between contrast strength and the pressure ( r=-0.971, P<0.001). Under different pressures, the contrast agent intensity showed different degrees of natural decrease in 1 s, 3 s, and 5 s. The difference in the percentage change in contrast agent intensity in each pressure gradient was not statistically significant in 1 s ( P>0.05), whereas the differences in the percentage change in contrast agent intensity in 3 s and 5 s were statistically significant in each pressure gradient (all P<0.05). After microbubble contrast agent was stimulated by ultrasound excitation for 1s, the percentage change in contrast agent intensity was significantly correlated with ambient pressure ( r=-0.976, P<0.001). A linear regression model was fitted with the percentage change in contrast agent intensity after 1 s of stimulation as the independent variable and the pressure as the dependent variable, with the model equation: y=60.075-2.559×x1, where x1 is the percentage change in contrast agent ( R2=0.952, P<0.001). Conclusions:The percentage change in contrast intensity after 1 s of ultrasound excitation of microbubble contrast agent is a favorable predictor of hydrostatic pressure at 6 pressure gradients of 20, 25, 30, 35, 40, and 45 cmH 2O, which may provide a new method for noninvasive monitoring of portal vein pressure for clinicans.

Objective:To establish a nomogram prediction model for recurrence within 2 years after radical resection of hepatocellular carcinoma (HCC) based on clinical and ultrasonographic characteristics.Methods:Clinical data from 405 HCC patients (including 327 males and 78 females), aged 60 (53, 66) years old, who underwent radical hepatectomy in the Zhongshan Hospital, Fudan University, from January to December 2021, were retrospectively collected. The patients were divided into two groups: the training group ( n=283) and the validation group ( n=122). Based on recurrence within 2 years after surgery, the 283 patients in the training group were further categorized into the recurrence group ( n=73) and the non-recurrence group ( n=210). Among the 122 patients in the validation group, 33 had recurrence within 2 years, while 89 did not. Data on age, microvascular invasion, alpha-fetoprotein (AFP), AFP lentil lectin-reactive fraction (AFP-L3), protein induced by vitamin K absence or antagonist-II (PIVKA-II), tumor number, and enhancement homogeneity were collected. Logistic regression analysis was performed on the training group to identify risk factors associated with postoperative recurrence, and a nomogram model for predicting HCC recurrence was constructed based on these factors. Calibration curves were used to compare the consistency between predicted and actual outcomes in both the training and validation groups. Results:Multivariate logistic regression analysis revealed that younger age ( OR=0.976, 95% CI: 0.953-1.000, P=0.004), higher AFP-L3 ( OR=1.066, 95% CI: 1.014-1.120, P=0.012), higher PIVKA-II ( OR=1.000, 95% CI: 1.000-1.001, P=0.042), multiple tumors ( OR=0.399, 95% CI: 0.225-0.706, P=0.038), and heterogeneous enhancement ( OR=0.472, 95% CI: 0.243-0.916, P=0.045) were significant risk factors for recurrence after partial hepatectomy in HCC patients. The nomogram constructed based on these variables had a C-index of 0.87 (95% CI: 0.81-0.93) in the training group and 0.83 (95% CI: 0.77-0.89) in the validation group. The calibration curves for predicting recurrence within 2 years after partial hepatectomy in HCC patients showed a high degree of fit in both the training and validation groups, indicating a good agreement between predicted and actual outcomes. Conclusion:The nomogram model constructed based on preoperative clinical and ultrasonographic characteristics can effectively predict the risk of recurrence within 2 years after radical resection of HCC.

Objective To explore the efficacy of the geriatric interdisciplinary team(GIT)service for elderly patients with multimorbidity and malnutrition.Methods We selected 109 elderly patients with diabetes mellitus(DM),chronic obstructive pulmonary disease(COPD)or chronic heart failure(CHF)and multi morbidity to the Department of Geriatrics of The 2nd People's Hospital of Kunming City from January 2022 to January 2023.The patients were randomly divided into the control group of 53 cases and the observation group of 56 cases.The control group received routine management of multimorbidity and malnutrition,while the observation group used the GIT service model to manage multimorbidity and malnutrition.The disease,nutritional status,and quality of life outcomes of the two groups of elderly patients were evaluated before and 3 months after the intervention.(1)HbA1c was detected in patients with DM in both groups,and the aggravation of patients with COPD or CHF was observed and recorded.(2)Nutrition-related indicators(Tp,ALB and Hb)and Barthel index were evaluated in both groups.Results(1)The HbA1c of patients with DM in the two groups decreased after intervention(P＜0.001),and the decrease in the observation group was more significant than that in the control group(P＜0.001).(2)The number of AECOPEs in patients with COPD in the two groups decreased,and the observation group was less than the control group(P＜0.05).(3)The number of CHF acute exacerbations in patients with CHF in the two groups decreased and the observation group was fewer than the control group(P＜0.05).(4)The TP,ALB,Hb and Barthel index scores of the two groups increased compared with those before intervention,and the observation group increased more significantly than the control group(P＜0.05).Conclusion The geriatric interdisciplinary team service for the elderly has a good effect in the intervention of elderly patients with multimorbidity and malnutrition.

Objective To systematically evaluate the efficacy of β-blocker in pediatric patients with congestive heart failure and congenital heart disease,and to provide evidence for clinician.Methods Before-and-after self-control study and randomized controlled trials were retrieved from PubMed,EMbase,the Cochrane Library,CNKI,WanFang,VIP databases,and the search time was from the establishment of the database to October 31,2023.All outcomes included left ventricular ejection fraction(LVEF),left ventricular fraction shortening,LVFS),left ventricular end-diastolic dimension(LVDD),Left ventricular end-systolic dimension(LVSD),N-terminal proB brain natriuretic peptide(NT-proBNP),heart rate,blood pressure and cardiac function improvement.Results A total of 20 trials involving 1 068 children with heart failure(dilated cardiomyopathy and endocardial fibroelastosis were included.Meta-analysis results showed that the addition of β-blockers(metoprolol succinate,bisoprolol and carvedilol)on the basis of conventional drug therapy for heart failure had significant effects on LVEF[MD=13.06,95%CI(11.67,14.45),P<0.001],LVFS[MD=6.96,95%CI(6.54,7.37),P<0.001],LVDD[MD=-6.43,95%CI(-7.58,-5.28),P<0.001]and LVSD[MD=-8.30,95%CI(-8.83,-7.76),P<0.001]were significantly improved.In addition,blood pressure,heart rate,NT-proBNP and cardiac function could also be improved.Conclusion The combination regimen of β-blockers on the basis of conventional drug therapy for heart failure can improve cardiac function and symptoms of heart failure in children with congestive heart failure.Therefore,it is recommended that β-blockers should be actively used in the conventional treatment regimen for children with congestive heart failure.

Objective To observe the efficacy and safety of medical adhesive occlusion of needle passage for preventing bleeding after CT-guided liver puncture biopsy.Methods Fifty-three patients with liver lesions who underwent CT-guided liver puncture biopsy were retrospectively enrolled.Medical adhesive was used to block the needle passage after puncture sampling.The technical success rate of biopsy and occlusion,as well as the biopsy pathological diagnostic accuracy and incidence of complications were observed and recorded.Results CT-guided liver puncture biopsy were successfully performed in all 53 patients and satisfactory samples were obtained.The technical success rate of biopsy was 100％(53/53),and pathological diagnostic accuracy was 96.23％(51/53).After blocking puncture needle passage with medical adhesive,no obvious bleeding was found in 49 cases(49/53,92.45％),while a small amount of bleeding was noticed in 4 cases(4/53,7.55％)but without subsequent increase during 20-minute observation,and the success rate of occlusion was 100％(53/53).After CT-guided liver puncture biopsy,mild local pain occurred in 5 cases(5/53,9.43％),while slight displacement of the medical adhesive was observed in 12 cases(12/53,22.64％).No serious complications associated with puncture or medical adhesive occurred.Conclusion Medical adhesive occlusion of needle passage was effective and safe for preventing bleeding after CT-guided liver puncture biopsy.

Objective To observe the efficacy and safety of medical adhesive occlusion of needle passage for preventing bleeding after CT-guided liver puncture biopsy.Methods Fifty-three patients with liver lesions who underwent CT-guided liver puncture biopsy were retrospectively enrolled.Medical adhesive was used to block the needle passage after puncture sampling.The technical success rate of biopsy and occlusion,as well as the biopsy pathological diagnostic accuracy and incidence of complications were observed and recorded.Results CT-guided liver puncture biopsy were successfully performed in all 53 patients and satisfactory samples were obtained.The technical success rate of biopsy was 100％(53/53),and pathological diagnostic accuracy was 96.23％(51/53).After blocking puncture needle passage with medical adhesive,no obvious bleeding was found in 49 cases(49/53,92.45％),while a small amount of bleeding was noticed in 4 cases(4/53,7.55％)but without subsequent increase during 20-minute observation,and the success rate of occlusion was 100％(53/53).After CT-guided liver puncture biopsy,mild local pain occurred in 5 cases(5/53,9.43％),while slight displacement of the medical adhesive was observed in 12 cases(12/53,22.64％).No serious complications associated with puncture or medical adhesive occurred.Conclusion Medical adhesive occlusion of needle passage was effective and safe for preventing bleeding after CT-guided liver puncture biopsy.

Objective:To summarize the experience of blood glucose management and nursing for a newborn with repeated hypoglycemia in Beckwith-Wiedemann syndrome.Method:A multidisciplinary team was formed, and formulated an individualized care plan for a large infant with Beckwith-Wiedemann syndrome. A dynamic blood glucose monitoring system was used to closely monitor blood glucose fluctuations for this children, dynamically adjusted the amount of infusion and milk according to the blood sugar situation, detected and dealed with hypoglycemia in time, strengthened the skin care of child and implemented psychological care for the family.Results:After refined treatment and care, the child′s vital signs were stable, and his blood sugar could maintain within a normal range before being discharged from the hospital.Conclusions:The use of dynamic blood glucose monitoring system under the guidance of a multidisciplinary team can effectively monitor and control the blood glucose fluctuations of children with BWS syndrome, which can provide a basis for further treatment of children.

Acupuncture can improve the motor and non-motor symptoms of Parkinson's disease, and the effect of acupuncture combined with drug therapy is better than that of drug therapy alone. The possible mechanism includes inhibiting α-synuclein aggregation, oxidative stress, and neuroinflammation, inhibiting the apoptosis of dopaminergic neurons, and achieving a neuroprotective effect. The points mainly selected for Acupuncture treatment for this disease are Zusanli (ST 36), Yanglingquan (GB 34), Taichong (LR 3), Xuehai (SP 10), and other points. Early use of acupuncture and acupuncture combined with medical treatment strategy is worthy of clinical application.