Objective:To assess health-related quality of life and disease burden of Chinese Phenylketonuria(PKU)patients and inform optimized management and support strategies.Methods:A cross-sectional survey is conducted to explore questionnaires.The Delphi method was applied to form a standardized questionnaire.Results:A total of 263 valid questionnaires were collected.The average patient age was 7.6 years.Younger patients reported better quality of life.Mean total medical cost per patient was 238 461.9 yuan,exceeding the 2024 national per capita disposable income.Conclusion:PKU patients are facing significant challenges in both quality of life and economic burden.

Objective:To analyze the changes of peripheral blood CD 4+ T lymphocyte subsets caused by nanosecond pulse ablation in C57BL/6J liver cancer mice and its effect on the immunity of liver cancer mice. Methods:According to the randomallocation, 32 female C57BL/6J mice weighing 18-20 g and aged 8-10 weeks were divided into the blank control group (Group A), the liver cancer control group (Group B), the radical liver lobectomy group (Group C), and the nanosecond pulse ablation treatment group (Group D), with 8 mice in each group. The in situ liver cancer models for mice in groups B, C, and D were established using Hepa 1-6 hepatoma cell line. On the 7th day after modeling, group C underwent liver lobectomy, and group D underwent nanosecond pulse ablation. After 7 days of treatment, the subtypes Th1, Th2, Th17 and Treg cell levels of CD 4+ T cells in the peripheral blood of the four groups of mice were detected by flow cytometry. Results:Flow cytometry was used to detect and analyze the proportions of CD 4+ T cell subsets Th1, Th2, Th17 and Treg in the peripheral blood of four groups of mice. Comparisons of each index among the groups showed statistically significant differences (all P<0.001). The proportion of Th1 cells detected by flow cytometry was (8.4±1.1) % in group A, (8.5±1.5)% in group D, (3.5±0.5)% in group B, and (2.4±0.5)% in group C. Both group A and Group D were higher than group B and group C, and the differences were statistically significant (all P<0.001). The proportion of Th2 cells detected by flow cytometry was (5.1±0.8)% in group A, (5.1±1.3)% in Group B, (5.4±0.9)% in group C, and (3.6±0.9)% in group D. The proportion of Th2 cells in groups A, B, and C was higher than that in group D, and the differences were statistically significant (all P<0.05). The proportion of Th17 cells detected by flow cytometry was (1.5±0.6)% in group A, (8.6±1.3)% in group B, (8.2±1.5)% in group C, and (1.7±0.3)% in group D. The proportion of Th17 cells in both group B and group C was higher than that in group A and group D, and the differences were statistically significant (all P<0.001). The proportion of Treg cells detected by flow cytometry was (7.0±0.9)% in group B, (6.8±0.9)% in group C, (3.8±0.8)% in group D, and (0.9±0.2)% in group A. The proportion of Treg in group B and group C was higher than that in group A and group D, and the difference was statistically significant (all P<0.001). Conclusion:Nanosecond pulse ablation for liver cancer in mice can regulate CD 4+ T cell subsets in peripheral blood, enhance the immune response of Th1 cells, inhibit the expression of Th2, Th17 and Treg cells, and improve the immunosuppressive state caused by liver cancer.

Objective To explore the effect of collateral status on prognosis in elderly patients with acute ischemic stroke due to large vessel occlusion(AIS-LVO)after Solitaire stent retriever in combination with the intracranial support catheter aspiration for mechanical thrombectomy(SWIM),and construct a prediction model for prognosis.Methods A retrospective analysis was performed on 240 elderly AIS-LVO patients who underwent SWIM technique in our hospital be-tween February 2019 and February 2024.According to gender,age,occlusion sites and TOAST classifications,they were divided into a modeling group(180 cases)and a verification group(60 cases)in a ratio of 3∶1.Based on the results of modified Rankin scale(mRS)at 3 months after surgery,the patients in the modeling group were further divided into good prognosis subgroup(mRS score:0-2,97 cases)and poor prognosis subgroup(mRS score:3-6,83 cases).Multivari-ate logistic regression analysis was applied to evaluate the relationship between preoperative col-lateral circulation status and prognosis and to identify the influencing factors for prognosis.Then a prediction model for prognosis was constructed,and its performance was evaluated by ROC curve analysis.Results In the modeling group at 3 months of follow-up,the poor prognosis subgroup had significantly larger proportions of posterior circulation occlusion,cardiogenic embolism,ASITN/SIR grades 3-4 and hemorrhage transformation,higher NIHSS score at admission and longer interval from onset to vascular recanalization,while lower ASPECTS score at admission when compared with the good prognosis subgroup(P＜0.01).Multivariate logistic regression analysis showed that occlusion site,TOAST classification,NIHSS score at admission,interval from onset to vascular recanalization and hemorrhage transformation were independent risk fac-tors for poor prognosis,while ASPECTS score at admission and collateral circulation were protec-tive factors of good prognosis in the elderly AIS-LVO patients after SWIM technique(P＜0.01).Hosmer-Lemeshow test showed that the regression equation obtained goodness of fit in the mod-eling group(P=0.435).ROC curve analysis revealed that the AUC,sensitivity and specificity of then constructed prediction model for poor prognosis was 0.855[95％CI(0.797-0.913)],81.93％and 79.38％,respectively.The model was further verified in the data of the verification group(34 cases in good prognosis and 26 cases with poor prognosis),the AUC value,sensitivity and speci-ficity was 0.839[95％CI(0.732-0.947)],84.62％and 79.41％,respectively.Conclusion Our pre-diction model constructed based on screened risk factors for poor prognosis has good validity in patients with AIS-LVO after SWIM technique,which can identify the patients at high risk for poor prognosis.

Objective:To assess health-related quality of life and disease burden of Chinese Phenylketonuria(PKU)patients and inform optimized management and support strategies.Methods:A cross-sectional survey is conducted to explore questionnaires.The Delphi method was applied to form a standardized questionnaire.Results:A total of 263 valid questionnaires were collected.The average patient age was 7.6 years.Younger patients reported better quality of life.Mean total medical cost per patient was 238 461.9 yuan,exceeding the 2024 national per capita disposable income.Conclusion:PKU patients are facing significant challenges in both quality of life and economic burden.

Objective To explore the effect of collateral status on prognosis in elderly patients with acute ischemic stroke due to large vessel occlusion(AIS-LVO)after Solitaire stent retriever in combination with the intracranial support catheter aspiration for mechanical thrombectomy(SWIM),and construct a prediction model for prognosis.Methods A retrospective analysis was performed on 240 elderly AIS-LVO patients who underwent SWIM technique in our hospital be-tween February 2019 and February 2024.According to gender,age,occlusion sites and TOAST classifications,they were divided into a modeling group(180 cases)and a verification group(60 cases)in a ratio of 3∶1.Based on the results of modified Rankin scale(mRS)at 3 months after surgery,the patients in the modeling group were further divided into good prognosis subgroup(mRS score:0-2,97 cases)and poor prognosis subgroup(mRS score:3-6,83 cases).Multivari-ate logistic regression analysis was applied to evaluate the relationship between preoperative col-lateral circulation status and prognosis and to identify the influencing factors for prognosis.Then a prediction model for prognosis was constructed,and its performance was evaluated by ROC curve analysis.Results In the modeling group at 3 months of follow-up,the poor prognosis subgroup had significantly larger proportions of posterior circulation occlusion,cardiogenic embolism,ASITN/SIR grades 3-4 and hemorrhage transformation,higher NIHSS score at admission and longer interval from onset to vascular recanalization,while lower ASPECTS score at admission when compared with the good prognosis subgroup(P＜0.01).Multivariate logistic regression analysis showed that occlusion site,TOAST classification,NIHSS score at admission,interval from onset to vascular recanalization and hemorrhage transformation were independent risk fac-tors for poor prognosis,while ASPECTS score at admission and collateral circulation were protec-tive factors of good prognosis in the elderly AIS-LVO patients after SWIM technique(P＜0.01).Hosmer-Lemeshow test showed that the regression equation obtained goodness of fit in the mod-eling group(P=0.435).ROC curve analysis revealed that the AUC,sensitivity and specificity of then constructed prediction model for poor prognosis was 0.855[95％CI(0.797-0.913)],81.93％and 79.38％,respectively.The model was further verified in the data of the verification group(34 cases in good prognosis and 26 cases with poor prognosis),the AUC value,sensitivity and speci-ficity was 0.839[95％CI(0.732-0.947)],84.62％and 79.41％,respectively.Conclusion Our pre-diction model constructed based on screened risk factors for poor prognosis has good validity in patients with AIS-LVO after SWIM technique,which can identify the patients at high risk for poor prognosis.

Objective:To analyze the changes of peripheral blood CD 4+ T lymphocyte subsets caused by nanosecond pulse ablation in C57BL/6J liver cancer mice and its effect on the immunity of liver cancer mice. Methods:According to the randomallocation, 32 female C57BL/6J mice weighing 18-20 g and aged 8-10 weeks were divided into the blank control group (Group A), the liver cancer control group (Group B), the radical liver lobectomy group (Group C), and the nanosecond pulse ablation treatment group (Group D), with 8 mice in each group. The in situ liver cancer models for mice in groups B, C, and D were established using Hepa 1-6 hepatoma cell line. On the 7th day after modeling, group C underwent liver lobectomy, and group D underwent nanosecond pulse ablation. After 7 days of treatment, the subtypes Th1, Th2, Th17 and Treg cell levels of CD 4+ T cells in the peripheral blood of the four groups of mice were detected by flow cytometry. Results:Flow cytometry was used to detect and analyze the proportions of CD 4+ T cell subsets Th1, Th2, Th17 and Treg in the peripheral blood of four groups of mice. Comparisons of each index among the groups showed statistically significant differences (all P<0.001). The proportion of Th1 cells detected by flow cytometry was (8.4±1.1) % in group A, (8.5±1.5)% in group D, (3.5±0.5)% in group B, and (2.4±0.5)% in group C. Both group A and Group D were higher than group B and group C, and the differences were statistically significant (all P<0.001). The proportion of Th2 cells detected by flow cytometry was (5.1±0.8)% in group A, (5.1±1.3)% in Group B, (5.4±0.9)% in group C, and (3.6±0.9)% in group D. The proportion of Th2 cells in groups A, B, and C was higher than that in group D, and the differences were statistically significant (all P<0.05). The proportion of Th17 cells detected by flow cytometry was (1.5±0.6)% in group A, (8.6±1.3)% in group B, (8.2±1.5)% in group C, and (1.7±0.3)% in group D. The proportion of Th17 cells in both group B and group C was higher than that in group A and group D, and the differences were statistically significant (all P<0.001). The proportion of Treg cells detected by flow cytometry was (7.0±0.9)% in group B, (6.8±0.9)% in group C, (3.8±0.8)% in group D, and (0.9±0.2)% in group A. The proportion of Treg in group B and group C was higher than that in group A and group D, and the difference was statistically significant (all P<0.001). Conclusion:Nanosecond pulse ablation for liver cancer in mice can regulate CD 4+ T cell subsets in peripheral blood, enhance the immune response of Th1 cells, inhibit the expression of Th2, Th17 and Treg cells, and improve the immunosuppressive state caused by liver cancer.

Background::Although overnight fasting is recommended prior to endoscopic retrograde cholangiopancreatography (ERCP), the benefits and safety of high-carbohydrate fluid diet (CFD) intake 2 h before ERCP remain unclear. This study aimed to analyze whether high-CFD intake 2 h before ERCP can be safe and accelerate patients’ recovery.Methods::This prospective, multicenter, randomized controlled trial involved 15 tertiary ERCP centers. A total of 1330 patients were randomized into CFD group ( n = 665) and fasting group ( n = 665). The CFD group received 400 mL of maltodextrin orally 2 h before ERCP, while the control group abstained from food/water overnight (>6 h) before ERCP. All ERCP procedures were performed using deep sedation with intravenous propofol. The investigators were blinded but not the patients. The primary outcomes included postoperative fatigue and abdominal pain score, and the secondary outcomes included complications and changes in metabolic indicators. The outcomes were analyzed according to a modified intention-to-treat principle. Results::The post-ERCP fatigue scores were significantly lower at 4 h (4.1 ± 2.6 vs. 4.8 ± 2.8, t = 4.23, P <0.001) and 20 h (2.4 ± 2.1 vs. 3.4 ± 2.4, t= 7.94, P <0.001) in the CFD group, with least-squares mean differences of 0.48 (95% confidence interval [CI]: 0.26–0.71, P <0.001) and 0.76 (95% CI: 0.57–0.95, P <0.001), respectively. The 4-h pain scores (2.1 ± 1.7 vs. 2.2 ± 1.7, t = 2.60, P = 0.009, with a least-squares mean difference of 0.21 [95% CI: 0.05–0.37]) and positive urine ketone levels (7.7% [39/509] vs. 15.4% [82/533], χ2 = 15.13, P <0.001) were lower in the CFD group. The CFD group had significantly less cholangitis (2.1% [13/634] vs. 4.0% [26/658], χ2 = 3.99, P = 0.046) but not pancreatitis (5.5% [35/634] vs. 6.5% [43/658], χ2 = 0.59, P = 0.444). Subgroup analysis revealed that CFD reduced the incidence of complications in patients with native papilla (odds ratio [OR]: 0.61, 95% CI: 0.39–0.95, P = 0.028) in the multivariable models. Conclusion::Ingesting 400 mL of CFD 2 h before ERCP is safe, with a reduction in post-ERCP fatigue, abdominal pain, and cholangitis during recovery.Trail Registration::ClinicalTrials.gov, No. NCT03075280.

Objective By using high-resolution magnetic resonance angiography to display the vascular wall imaging so as to evaluate the relationship between plaque characteristics and postoperative in-stent restenosis(ISR)in patients with middle cerebral artery stenosis.Methods A total of 66 patients with symptomatic atherosclerotic middle cerebral artery stenosis,who were admitted to the Affiliated Hospital of Chengde Medical College of China from January 2019 to March 2023,were enrolled in this study.Before stent implantation,all the 66 patients completed high-resolution magnetic resonance angiography.According to the postoperative imaging follow-up results,the patients were divided into ISR group and non-ISR group.The preoperative plaque characteristics,which were assessed by high-resolution magnetic resonance imaging,were compared between the two groups.Results ISR group had 14 patients and non-ISR group had 52 patients.Most of the plaques were located in the inferior lateral wall(37.8％)and the ventral lateral wall(28.7％),in which no statistically significant difference existed between the two groups(P＞0.05).Compared with non-ISR group,in ISR group the negative remodeling degree was obviously higher and the difference between the two groups was statistically significant(x2=6.026,P=0.049).The plaque load in ISR group and non-ISR group was 79.09±8.82 and 69.46±10.49 respectively,and the difference between the two groups was statistically significant(t=3.143,P=0.003).The homocysteine level in ISR group and non-ISR group was(16.02±4.24)mol/L and(12.05±3.34)mol/L respectively,and the difference between the two groups was statistically significant(t=3.717,P＜0.001).In ISR group,there were more significantly contrast-enhanced plaques(78.5％vs.42.3％),with statistically significant difference(x2=6.311,P=0.043).Multivariate logistic regression analysis showed that plaque load(OR=1.225,95％CI:1.040-1.443,P=0.015)and homocysteine level(OR=1.676,95％CI:1.150-2.442,P=0.007)were the independent risk factors for ISR.ROC curve analysis showed that in predicting ISR,the AUC,specificity and sensitivity of the plaque load were 0.765(95％CI:0.622-0.908,P=0.002),0.731 and 0.714 respectively,which of the homocysteine level were 0.767(95％CI:0.623-0.911,P=0.002),0.942 and 0.500 respectively.The combination use of plaque load and homocysteine level could achieve the best predictive effect,its AUC,specificity and sensitivity were 0.887(95％CI:0.794-0.981,P＜0.001),0.904 and 0.714 respectively.Conclusion The plaque load assessed by high-resolution magnetic resonance imaging and the homocysteine level have higher specificity and sensitivity in predicting ISR in patients with middle cerebral artery stenosis.

OBJECTIVES: Primary trigeminal neuralgia (PTN) is a common cranial nerve disease in neurosurgery, which seriously endangers the physical and mental health of patients. Percutaneous balloon compression (PBC) has become an effective procedure for the treatment of PTN by blocking pain conduction through minimally invasive puncture. However, the recurrence of facial pain after PBC is still a major problem for PTN patients. Intraoperative balloon shape, pressure and compression time can affect the prognosis of patients with PBC after surgery. The foramen ovale size has an effect on the balloon pressure in Meckel's lumen. This study aims to analyse the predictive value of foramen ovale size for postoperative pain recurrence of PBC by exploring the relationship between foramen ovale size and postoperative pain recurrence of PBC. METHODS: A retrospectively analysis was conducted on the clinical data of 60 patients with PTN who were treated with PBC in Department of Neurosurgery, Affiliated Hospital of Chengde Medical College from November 2018 to December 2021. We followed-up and recorded the Barrow Neurological Institute (BNI) pain score at 1, 3, 6 and 12 months after operation. According to the BNI pain score at 12 months after surgery, the patients were divided into a cure group (BNI pain score I to Ⅱ) and a recurrence group (BNI pain score Ⅲ to Ⅴ). The long diameter, transverse diameter and area of foramen ovale on the affected side and the healthy side of the 2 groups were measured. Receiver operating characteristic (ROC) curve and area under the curve (AUC) were used for analysis the relationship between the recurrence of pain and the long diameter, transverse diameter, area of foramen ovale on the affected side, and aspect ratio, transverse diameter ratio, area ratio of foramen ovale on the affected side to healthy side in the 2 groups. RESULTS: At the end of 12 months of follow-up, 50 (83.3%) patients had pain relief (the cured group), 10 (16.7%) patients had different degrees of pain recurrence (the recurrence group), and the total effective rate was 83.3%. There were no significant differences in preoperative baseline data between the 2 groups (all P>0.05). The long diameter of foramen ovale on the affected side, the long diameter ratio and area ratio of foramen ovale on the affected/healthy side in the cured group were significantly higher than those in the recurrence group (all P<0.05), and there were no significant differences in the transverse diameter and area of foramen ovale on the affected side and the transverse diameter ratio of foramen ovale on the affected/healthy side between the 2 groups (all P>0.05). The ROC curve analysis showed that the AUC of the long diameter of foramen ovale on the affected side was 0.290 (95% CI 0.131 to 0.449, P=0.073), and the AUC of aspect ratio of foramen ovale on the affected side to healthy side was 0.792 (95% CI 0.628 to 0.956, P=0.004). The AUC of area ratio of foramen ovale on the affected side to healthy side was 0.766 (95% CI 0.591 to 0.941, P=0.008), indicating that aspect ratio and area ratio of foramen ovale on the affected side to healthy side had a good predictive effect on postoperative pain recurrence of PBC. When aspect ratio of foramen ovale on the affected side to healthy side was less than 0.886 3 or area ratio of foramen ovale on the affected side to healthy side was less than 0.869 4, postoperative pain recurrence was common. CONCLUSIONS Accurate evaluation of the foramen ovale size of skull base before operation is of great significance in predicting pain recurrence after PBC.
Humans ; Retrospective Studies ; Foramen Ovale ; Treatment Outcome ; Trigeminal Neuralgia/surgery* ; Pain, Postoperative/etiology* ; Recurrence

Objective:To investigate characteristics of the 18F-flurodeoxyglucose ( 18F-FDG) uptake intensity and ranges in distinct hepatic alveolar echinococcosis lesions. Methods:The clinical data of 39 patients with position emission tomography during Jan 2017 to Dec 2019 in the First Affiliated Hospital of Xinjiang Medical University were enrolled. Among them, there were 17 males and 22 females, aging from 15 to 65 years (median 34 years). Lesions were classified into six groups based on heterogenic scales of calcification and liquefaction: A. non-calcified and non-liquefied ( n=7); B. obvious calcified and non-liquefied ( n=7); C. partial calcified and partial liquefied( n=10); D. obvious calcified and partial liquefied ( n=5); E. partial calcified and subtotal liquefied ( n=5); F. obvious calcified and subtotal liquefied ( n=5). Tumor to background ratio (TBR) and width (W) of lesion infiltrative boundary were measured and calculated. Statistical comparison using Mann-Whitney U test as well as correlation analysis was performed. Results:TBR values [ M( Q1, Q3)] for each group were 4.40(3.66, 7.03), 2.55(1.69, 3.60), 3.73(3.37, 5.21), 2.90(2.75, 3.60), 3.80(3.49, 6.36), 2.49(2.21, 3.97), among which A>B, A>D, A>F, C>B, E>B ( U=3.0, 4.0, 4.5, 11.0, 5.0, all P<0.05); From the perspective of the calcification in each group, it was found that the lighter the calcification was, the greater the TBR value was. W values [ M( Q1, Q3)] for each group were [12.5(10.0, 19.5), 11.2(10.5, 12.5), 12.2(10.9, 13.2), 7.8(7.3, 9.3), 10.0(7.3, 13.4), 7.3(6.8, 7.6)] mm, among which A>D, A>F, B>D, B>F, C>D, C>F (all U=0, all P<0.05); According to the degree of calcification and liquefaction of lesions in each group, the lighter the calcification was, the greater the W value was; The heavier the liquefaction was, the smaller the W value was. A mild strength linear correlation has been observed between the TBR value and W value ( r=0.4136, P<0.05). Conclusions:Less calcification and liquefaction implicated higher 18F-FDG uptake intensity and wider range. Radical resection margins and tissue sampling should be individualized based on different lesion features in surgical treatment.