Hepatolenticular degeneration， also known as Wilson disease （WD）， is a hereditary disease caused by mutations in the ATP7B gene， leading to copper metabolism disorders. Gene mutations result in impaired synthesis of copper-binding protein， and abnormal excretion of copper through bile leads to pathological deposition of copper in various organs， ultimately causing multi-organ damage. The insidious onset and low specificity of symptoms make it difficult to diagnose this disease. On the basis of existing studies and the theory of latent toxin， this paper proposes that latent toxin in kidney collaterals is the main pathogenesis of renal injury in WD. It is pointed out that health Qi deficiency and latent pathogen are the premises for the occurrence of this disease， and the transformation of latent pathogen into toxin is the ley pathological process. Toxin damaging kidney collaterals is the ultimate result. According to the pathogenesis， this paper proposes the treatment principle of reinforcing healthy Qi and resolving toxin and treatment based on syndrome differentiation. This review provides new ideas for the diagnosis and treatment of renal injury in WD with traditional Chinese medicine.

ObjectiveTo observe the clinical efficacy of Gandouling decoction combined with neuromuscular electrical stimulation （NMES） in the treatment of dysphagia in Wilson disease （WD） with combined phlegm and stasis. MethodsA total of 80 WD patients with dysphagia due to combined phlegm and stasis treated in the Department of Encephalopathy， the First Affiliated Hospital of Anhui University of Chinese Medicine were randomized into a control group and an observation group， with 40 patients in each group. In addition， 40 healthy volunteers were recruited as the normal group. The control group was treated with basic copper drainage combined with NMES. The observation group was treated with Gandouling Decoction on the basis of the therapy in the control group. Each course of treatment lasted for 8 days， and the patients were treated for a total of 4 courses. All subjects underwent video fluoroscopic swallowing study （VFSS） before and after treatment. During the examination， contrast agents with 4 different characters were used for the swallowing action， and the passing time was recorded. The TCM syndrome score， water swallow test score， standard swallowing assessment （SSA） score， and 24-h urinary copper level before and after treatment were analyzed. ResultsWhen performing VFSS， the passing time of contrast agents of different characters in the oral stage was longer in the WD group than in the normal group （P<0.01）， while it had no significant difference in the pharyngeal stage. After treatment， the passing time in the oral stage shortened in the control and observation groups （P<0.01）， and the observation group outperformed the control group （P<0.01）. After treatment， both the control and observation groups showed declines in TCM syndrome score and SSA score （P<0.01） and an increase in water swallow test score （P<0.01）， and the changes were more obvious in the observation group than in the control group （P<0.01）. In addition， the treatment in the control and observation groups elevated the 24-h urinary copper level （P<0.01）， and the elevation in the observation group was more obvious than that in the control group （P<0.01）. Neither group showed obvious adverse reaction. ConclusionGandouling decoction combined with NMES can significantly ameliorate dysphagia in WD patients with the syndrome of combined phlegm and stasis regarding the TCM syndrome score， water swallow test score， and SSA score， demonstrating definite clinical efficacy and high safety.

Digital technologies have become an integral part of complete denture restoration. With advancement in computer-aided design and computer-aided manufacturing (CAD/CAM), tools such as intraoral scanning, facial scanning, 3D printing, and numerical control machining are reshaping the workflow of complete denture restoration. Unlike conventional methods that rely heavily on clinical experience and manual techniques, digital technologies offer greater precision, predictability, and efficacy. They also streamline the process by reducing the number of patient visits and improving overall comfort. Despite these improvements, the clinical application of digital complete denture restoration still faces challenges that require further standardization. The major issues include appropriate case selection, establishing consistent digital workflows, and evaluating long-term outcomes. To address these challenges and provide clinical guidance for practitioners, this expert consensus outlines the principles, advantages, and limitations of digital complete denture technology. The aim of this review was to offer practical recommendations on indications, clinical procedures and precautions, evaluation metrics, and outcome assessment to support digital restoration of complete denture in clinical practice.
Humans ; Denture, Complete ; Computer-Aided Design ; Denture Design/methods* ; Consensus ; Printing, Three-Dimensional

Background and aims:Studies indicate that the gut microbiota and its metabolites are involved in the progression of cardiovascular diseases,and enterohepatic circulation plays an important role in this progression.This study aims to identify potential probiotics for the treatment of unstable angina(UA)and elucidate their mechanisms of action.Methods:Initially,the gut microbiota from patients with UA and control was analyzed.To directly assess the effects of Bifidobacterium supplementation,10 patients with UA were enrolled and administered Bifidobacterium(630 mg per intake twice a day for 1 month).The fecal metagenome,serum trimethyl-amine N-oxide(TMAO)levels,and other laboratory parameters were evaluated before and after Bifido-bacterium supplementation.Results:After supplementing with Bifidobacterium for 1 month,there were statistically significant dif-ferences(P＜0.05)in TMAO,aspartate aminotransferase,total cholesterol,and low-density lipoprotein compared to before.Additionally,the abundance of Bifidobacterium longum increased significantly,although the overall abundance of Bifidobacterium did not reach statistical significance.The gut micro-biota,metabolites,and gut-liver axis are involved in the progression of UA,and potential mechanisms should be further studied.Conclusions:Metagenomic analysis demonstrated a reduced abundance of Bifidobacterium in patients with UA.Supplementation with Bifidobacterium restored gut dysbiosis and decreased circulating TMAO levels in patients with UA.This study provides evidence that Bifidobacterium may exert cardiovascular-protective effects through the gut-liver-heart axis.

Objective To explore the prognostic value of quantitative parameters of intra-voxel incoherent motion-diffusion weighted imaging(IVIM-DWI)in predicting the prognosis of patients with Kirsten rats arcomaviral oncogene homolog(KRAS)mutation non-small cell lung cancer(NSCLC)undergoing first-line concurrent chemoradiotherapy.Methods A total of 139 patients with KRAS-mutated NSCLC were selected as the research subjects,and all of them received first-line concurrent chemoradiotherapy.Based on follow-up results(with all-cause mortality within one year defined as the endpoint event),patients were divided into favorable prognosis group and poor prognosis group.General data and IVIM-DWI quantitative parameters[apparent diffusion coefficient(ADC),diffusion coefficient(D),pseudo-diffusion coefficient(D*)and perfusion fraction(f)]were compared be-tween the two groups.Cox regression analysis was employed to identify factors influencing poor prognosis in patients with KRAS-mutated NSCLC.Receiver operating characteristic(ROC)curves were plotted to evaluate the prognostic predictive value of IVIM-DWI quantitative parameters.Kaplan-Meier(K-M)survival curves and the Log-rank test were used to analyze the relationships between IVIM-DWI quantitative parameters and prognosis in patients with KRAS-mutated NSCLC.Results Among the 139 patients,four were lost during follow-up,resulting in a survival rate of 69.63％(94/135).The favorable prognosis group comprised 94 patients,while the poor prognosis group included 41 pa-tients.There were statistically significant differences in the types of KRAS mutations,the best thera-peutic effect after treatment,and smoking history between the two groups(P＜0.05).After adjus-ting for confounding factors,ADC,D,D* and f values were identified as influencing factors for poor prognosis in patients with KRAS-mutated NSCLC(P＜0.05).The area under the curve(AUC)for the combined prediction of prognosis using ADC,D,D* and f values was 0.933,which was higher than the AUC for each parameter alone.Patients with low ADC,D,D* and f values exhibited sig-nificantly lower survival rates compared to those with high values(P＜0.05).Conclusion IVIM-DWI quantitative parameters,including ADC,D,D* and f values,are influencing factors for the prognosis of first-line concurrent chemoradiotherapy in patients with KRAS-mutated NSCLC.The combined predictive performance of these parameters is high,offering guidance for clinical diagnosis and treatment and potentially improving disease prognosis.

This paper introduces the construction and application of the medical quality evaluation indicator system in clinical departments of a large general hospital.It describes the indicator system's constitution and score setting,summarize the characteristics and application practice of the assessment system,and proposes some ideas for the following improvement,which in order to provide some reference for other hospitals.

The present jaw relationship record tray is designed in an upper and lower integrated type,which can not accurately ob-tain important information of maxillary occlusal plane,the position about 1 mm below the upper lip and the support degree of the upper lip during clinical work.Therefore,the base-plate and occlusal-rim composite solid(diagnostic denture)is not accurate and the accu-rate jaw relationship can not be tranfered in the following steps.This paper introduces the design,clinical application process and key points of a split-type jaw relationship record tray.It can accurately obtain important information such as the occlusal plane,the position about 1mm below the upper lip,and the degree of support of the upper lip.It provides reference for the accurate recording and transfer of the jaw relationship.It also lays a foundation for the direct fabrication of full denture based on the information obtained by the split-type jaw relationship record tray after the digital impression obtained by intraoral scanning.

Objective To investigate the clinical efficacy of endoscopic assisted minimally invasive trans foraminal lumbar interbody fusion(MIS-TLIF)and traditional open TLIF in the treatment of senile degenerative spondylolisthesis.Methods From January 2020 to March 2021,100 elderly patients with degenerative spondylolisthesis were selected and divided into MIS-TLIF group and TLIF group according to the surgical method,with 50 cases in each group.The preoperative and postoperative VAS,Japanese Orthopedic Association score(JO A)and Oswestry disability index(ODI),and the rate of interbody fusion and the incidence of complications were compared and analyzed between the two groups.Results In MIS-TLIF group,the operative time,intraoperative blood loss,incision length,postoperative drainage volume and hospital stay were(167.5±54.2)minutes,(173.8±47.1)ml,(3.5±0.7)cm,(69.6±16.3)ml,and(8.3±2.7)days,respectively.In the TLIF group,it was(136.3±38.9)minutes,(281.0±50.3)ml,(10.0±2.1)cm,(148.4±28.2)ml,and(11.2±3.1)days,respectively.The difference between the two groups was statistically significant(P＜0.05).The VAS,JO A and ODI scores of MIS-TLIF group were(2.17±0.62)points,(21.72±3.14)points and(13.22 ±2.43)points,respectively.The results in TLIF group were(3.24±1.06)points,(17.06±2.85)points and(16.83±2.87)points,respectively,and there was statistical significance between the two groups(P＜0.05).There was no significant difference in the rate of interbody fusion between the two groups at 12 months after operation(P＞0.05).The incidence of postoperative complications in MIS-TLIF group(8.00％)was significantly lower than that in TLIF group(24.00％,P＜0.05).Conclusion MIS-TLIF assisted by endoscope has the advantages of less trauma,faster recovery and less postoperative complications,and the short-term curative effect is more satisfactory than TLIF.

Humans ; Consensus ; Pancreatic Neoplasms/therapy* ; Neuroendocrine Tumors/therapy*

OBJECTIVE: To analyze the clinical and genetic characteristics of a child with clinical manifestations of hypoplasia, epilepsy and abnormal face. METHODS: The clinical data of the child were collected. The peripheral blood samples of the patient and his parents were extracted for high-throughput sequencing, and Sanger sequencing verification and bioinformatics analysis were performed to detect suspected pathogenic variants. RESULTS: The clinical manifestations of the child were overall developmental backwardness, seizures, autism, and special facial appearance. High throughput sequencing showed that there was a heterozygous mutation of exon 11: c.1920_c.1927delCCTCTACC (p.Ser641Rfs*31) of the DYRK1A gene. The same variant was found in neither of her parents, suggesting that it has a denovo origin. CONCLUSION The exon11: c.1920_c.1927delCCTCTACC (p.Ser641Rfs*31) mutation in DYRK1A gene was the genetic etiology of the case, which enriches the pathogenic gene spectrum of DYRK1A and provides the basis for clinical diagnosis and genetic counseling.
Arthrogryposis ; Child ; Facies ; Female ; Heterozygote ; Humans ; Intellectual Disability/genetics* ; Mutation