Autosomal dominant polycystic kidney disease （ADPKD） is a systemic hereditary renal disorder and can affect multiple organs， and congenital hepatic fibrosis is one of the manifestations of liver involvement and is an important complication of ADPKD. Symptomatic management is currently the main treatment method for this disease， and disease-specific drugs such as tolvaptan have limited indications and cannot correct the underlying genetic defect. This article reports a case of ADPKD with congenital hepatic fibrosis， and sirolimus was identified as the individualized treatment regimen based on peripheral blood functional genomic analysis and drug sensitivity prediction platform. The patient achieved significant improvements in symptoms and quality of life after treatment， with a stable kidney volume. This case shows that functional genomics has a potential value in guiding individualized treatment of rare genetic disorders， which provides new treatment ideas and practice paths for similar patients.

Objective To analyze the relationship of total imaging burden score with blood pressure variability(BPV)and cognitive function in elderly patients with cerebral small vessel disease(CSVD).Methods Clinical data of 182 elderly CSVD patients admitted in our hospital from December 2022 to January 2024 were collected and analyzed retrospectively.According to the results of Chinese Guidelines for Diagnosis and Treatment of CSVD-Related Cognitive Dysfunc-tion(2019),Montreal Cognitive Assessment(MoCA)and other tools for their cognitive impair-ment,they were divided into cognitively impaired group(76 cases)and cognitively normal group(106 cases).The total burden score and BPV indicators[24 h systolic blood pressure coefficient of variation(24 h SBPCV),24 h diastolic blood pressure coefficient of variation(24 h DBPCV)]were compared between the two groups.ROC curve was plotted to evaluate the diagnostic value of total burden score,24 h SBPCV and 24 h DBPCV on cognitive impairment in elderly CSVD patients.Multivariate logistic regression analysis was used to identify the risk factors of cognitive impairment in the patients.Based on the MoCA score of the cognitively impaired group,these pa-tients were further assigned into mild and moderate-to-severe cognitive impairment subgroups[with a MoCA score of 18-25(43 cases)and＜18(33 cases),respectively].Then the total burden score,24 h SBPCV and 24 h DBPCV were compared between the two subgroups.Pearson correla-tion coefficient was adopted to evaluate the correlation of severity of cognitive impairment with total burden score,24 h SBPCV and 24 h DBPCV in the elderly CSVD patients.Results The total burden score,24 h SBPCV and 24 h DBPCV were significantly higher in the cognitively impaired group than the cognitively normal group(P＜0.01).ROC curve analysis indicated that total bur-den score(AUC=0.953,95％CI:0.926-0.980,P=0.000),24 h SBPCV(AUC=0.850,95％CI:0.795-0.906,P=0.000)and 24 h DBPCV(AUC=0.761,95％CI:0.690-0.832,P=0.000)had good diagnostic efficiency for cognitive impairment in the elderly CSVD patients,with a cut-off value of 1.5,11.82％,and 8.92％,respectively.Multivariate logistic regression analysis revealed that the above three indicators were risk factors for cognitive impairment in the elderly patients with CSVD(P＜0.05,P＜0.01).Their values were significantly lower in the mild than the moder-ate-to-severe cognitive impairment subgroups(P＜0.01).Pearson correlation analysis displayed that MoCA score was negatively correlated with total burden score,24 h SBPCV and 24 h DBPCV in elderly patients with CSVD(r=-0.755,-0.632,-0.601,P＜0.01).Conclusion Detection of total burden score and BPV indicators is beneficial to the assessment of cognitive impairment in elderly CSVD patients.The higher the total burden score and the greater the BPV indicators,the more severe the cognitive impairment is,which may explore new ideas for clinical diagnosis and treatment of CSVD.

Objective To analyze the relationship of total imaging burden score with blood pressure variability(BPV)and cognitive function in elderly patients with cerebral small vessel disease(CSVD).Methods Clinical data of 182 elderly CSVD patients admitted in our hospital from December 2022 to January 2024 were collected and analyzed retrospectively.According to the results of Chinese Guidelines for Diagnosis and Treatment of CSVD-Related Cognitive Dysfunc-tion(2019),Montreal Cognitive Assessment(MoCA)and other tools for their cognitive impair-ment,they were divided into cognitively impaired group(76 cases)and cognitively normal group(106 cases).The total burden score and BPV indicators[24 h systolic blood pressure coefficient of variation(24 h SBPCV),24 h diastolic blood pressure coefficient of variation(24 h DBPCV)]were compared between the two groups.ROC curve was plotted to evaluate the diagnostic value of total burden score,24 h SBPCV and 24 h DBPCV on cognitive impairment in elderly CSVD patients.Multivariate logistic regression analysis was used to identify the risk factors of cognitive impairment in the patients.Based on the MoCA score of the cognitively impaired group,these pa-tients were further assigned into mild and moderate-to-severe cognitive impairment subgroups[with a MoCA score of 18-25(43 cases)and＜18(33 cases),respectively].Then the total burden score,24 h SBPCV and 24 h DBPCV were compared between the two subgroups.Pearson correla-tion coefficient was adopted to evaluate the correlation of severity of cognitive impairment with total burden score,24 h SBPCV and 24 h DBPCV in the elderly CSVD patients.Results The total burden score,24 h SBPCV and 24 h DBPCV were significantly higher in the cognitively impaired group than the cognitively normal group(P＜0.01).ROC curve analysis indicated that total bur-den score(AUC=0.953,95％CI:0.926-0.980,P=0.000),24 h SBPCV(AUC=0.850,95％CI:0.795-0.906,P=0.000)and 24 h DBPCV(AUC=0.761,95％CI:0.690-0.832,P=0.000)had good diagnostic efficiency for cognitive impairment in the elderly CSVD patients,with a cut-off value of 1.5,11.82％,and 8.92％,respectively.Multivariate logistic regression analysis revealed that the above three indicators were risk factors for cognitive impairment in the elderly patients with CSVD(P＜0.05,P＜0.01).Their values were significantly lower in the mild than the moder-ate-to-severe cognitive impairment subgroups(P＜0.01).Pearson correlation analysis displayed that MoCA score was negatively correlated with total burden score,24 h SBPCV and 24 h DBPCV in elderly patients with CSVD(r=-0.755,-0.632,-0.601,P＜0.01).Conclusion Detection of total burden score and BPV indicators is beneficial to the assessment of cognitive impairment in elderly CSVD patients.The higher the total burden score and the greater the BPV indicators,the more severe the cognitive impairment is,which may explore new ideas for clinical diagnosis and treatment of CSVD.

Objective:To explore the value of magnetic resonance diffusion tensor imaging (DTI) in evaluating cerebrovascular small vessel disease (CSVD) in patients with systemic lupus erythematosus (SLE).Methods:Eighty-two patients with SLE combined with CSVD treated at Yixing People's Hospital from January to December 2022 were selected. They were divided into acute phase infarction group (16 cases), chronic phase infarction group (26 cases), and chronic ischemic lesion group (40 cases) based on routine MRI examination results. All patients underwent DTI examination to obtain the average diffusion coefficient (DCavg) and anisotropy score (FA) of the affected and contralateral normal white matter areas, and the evaluation value of DTI for CSVD in SLE patients was analyzed.Results:The DCavg value on the affected side of 82 patients was significantly higher than that on the healthy side: (11.10 ± 3.48) 10 -3 mm 2/s vs. (8.18 ± 2.42) 10 -3 mm 2/s, and the FA value on the affected side was significantly lower than that on the healthy side: 0.28 ± 0.05 vs. 0.45 ± 0.08, with a statistical significant differences ( P<0.05). The DCavg values of the acute infarction group, chronic infarction group, and chronic ischemic focus group were (11.88 ± 3.50), (9.69 ± 3.24) and (8.52 ± 2.34) 10 -3 mm 2/s, respectively, with statistical significant differences ( P<0.05). The FA values of the acute infarction group, chronic infarction group, and chronic ischemic focus group were 0.28 ± 0.04, 0.33 ± 0.06 and 0.40 ± 0.07, respectively, with statistical significant differences ( P<0.05). The receiver operating characteristic curve was drawn, and the results showed that the area under the curve (AUC) of the acute phase infarction group and the chronic phase infarction group evaluated by DCavg and FA alone and in combination were 0.757, 0.756, and 0.820, respectively. The AUC of the chronic phase infarction group and the chronic ischemic focus group evaluated by DCavg and FA were 0.772, 0.776, and 0.813, respectively. The AUC value of the combined evaluation was relatively large. Conclusions:DTI has good evaluation value for CSVD in SLE patients and can accurately determine the type of CSVD.

This study aims to discuss the therapeutic effect of magnesium isoglycyrrhizinate on hepatitis B virus(HBV)transgenic mouse and its effect on cellular immunity and liver inflammation. The changes of serum aspartate aminotransferase(AST)and alanine aminotransferase(ALT)activity, the difference of serum hepatitis B surface antigen(HBsAg), liver tissue HBsAg mRNA, and the pathological morphological changes of liver tissue were detected to investigate the hepatic inflammatory lesions and the efficacy of magnesium isoglycyrrhizinate in HBV transgenic mouse. Peripheral blood lymphocytes were classified by flow cytometry, and serum cytokines were detected by cytometric bead array(CBA)to explore the mechanism of magnesium isoglycyrrhizinate to reduce hepatic inflammatory lesions in HBV transgenic mouse. After grouping HBV transgenic mouse with serum transaminase activity and 35 days of continuous administration, serum transaminases level in magnesium isoglycyrrhizinate ［15 mg/(kg ·d)］ group was significantly lower than that in control group(P< 0. 05), serum HBsAg protein and liver tissue HBsAg mRNA increased with time, but there was no significant difference between the two groups. The main pathological changes of liver were liver cell swelling, necrosis and focal inflammatory cell infiltration, and the pathological changes of liver in magnesium isoglycyrrhizinate group were lighter than those in control group. The number of CD8+ cells in the blood of magnesium isoglycyrrhizinate group was significantly less than that in the control group(P< 0. 05)and the CD4+/CD8+ cell ratio was significantly higher than that in the control group(P< 0. 05). The content of inflammatory cytokines in serum of magnesium isoglycyrrhizinate group decreased significantly(P< 0. 05). Magnesium isoglycyrrhizinate can regulate the immune function of HBV transgenic mouse, decrease the infiltration of inflammatory cells in hepatic tissue and hepatocyte injury, but do not affect the expression of hepatocyte HBsAg.

In this paper, with B/S application and architecture, an integrated solution of PACS is designed, and the function and application of each part of PACS based on the architecture is introduced. The PACS based on this mode is safe, stable, easy to manage and upgrade and convenient for use in telemedicine.
Computer Systems ; Humans ; Radiology Information Systems ; Research ; Software ; Telemedicine