Objective:To evaluate the long-term efficacy and safety of recombinant coagulation factor Ⅷ (Octocog alfa) in Chinese patients with hemophilia A (HA) enrolled in the International Antihemophilic Factor Hemophilia A Outcome Database (AHEAD) study (NCT02078427) .Methods:Enrollment of Chinese patients in the AHEAD study was completed by January 2021, and data were collected up to July 15, 2022. This study primarily assessed patients in terms of the Gilbert score, global gait score within the Hemophilia Joint Health Score (HJHS), annualized bleeding rate (ABR), annualized joint bleeding rate, and adverse events.Results:A total of 168 male patients were included in this study, of which 113 received prophylactic treatment and 53 received on-demand treatment. The average age of the patients was 21.4±13.37 years. Compared with baseline, the global gait score within HJHS significantly decreased during the 1-year follow-up in patients with moderately severe HA in the prophylactic treatment group ( P=0.01) and on-demand treatment group ( P=0.008). The mean reduction in Gilbert score was greater in the prophylactic treatment group than in the on-demand treatment group (28.6% vs 8.2%). The average ABR decreased significantly during the 1-year follow-up (3.70 vs 7.78, P=0.01) in the prophylactic treatment group, particularly in patients with severe HA (2.14 vs 8.98, P=0.006) and pediatric patients (2.1 vs 4.73, P=0.03). The ABR score also decreased significantly in the moderate-dose prophylactic treatment group ( P=0.015). During the 1-year follow-up, 25 patients (14.9%) reported 39 adverse events, with only one patient developing treatment-related F Ⅷ inhibitor. Conclusion:Joint mobility improved in patients receiving either prophylactic or on-demand Octocog alfa. Bleeding episodes significantly reduced in patients receiving prophylactic treatment, particularly in pediatric patients and those with severe HA.

Objective:To evaluate the long-term efficacy and safety of recombinant coagulation factor Ⅷ (Octocog alfa) in Chinese patients with hemophilia A (HA) enrolled in the International Antihemophilic Factor Hemophilia A Outcome Database (AHEAD) study (NCT02078427) .Methods:Enrollment of Chinese patients in the AHEAD study was completed by January 2021, and data were collected up to July 15, 2022. This study primarily assessed patients in terms of the Gilbert score, global gait score within the Hemophilia Joint Health Score (HJHS), annualized bleeding rate (ABR), annualized joint bleeding rate, and adverse events.Results:A total of 168 male patients were included in this study, of which 113 received prophylactic treatment and 53 received on-demand treatment. The average age of the patients was 21.4±13.37 years. Compared with baseline, the global gait score within HJHS significantly decreased during the 1-year follow-up in patients with moderately severe HA in the prophylactic treatment group ( P=0.01) and on-demand treatment group ( P=0.008). The mean reduction in Gilbert score was greater in the prophylactic treatment group than in the on-demand treatment group (28.6% vs 8.2%). The average ABR decreased significantly during the 1-year follow-up (3.70 vs 7.78, P=0.01) in the prophylactic treatment group, particularly in patients with severe HA (2.14 vs 8.98, P=0.006) and pediatric patients (2.1 vs 4.73, P=0.03). The ABR score also decreased significantly in the moderate-dose prophylactic treatment group ( P=0.015). During the 1-year follow-up, 25 patients (14.9%) reported 39 adverse events, with only one patient developing treatment-related F Ⅷ inhibitor. Conclusion:Joint mobility improved in patients receiving either prophylactic or on-demand Octocog alfa. Bleeding episodes significantly reduced in patients receiving prophylactic treatment, particularly in pediatric patients and those with severe HA.

Prostate cancer(PCa)is one of the most common malignant tumors in men,marked by the high expression of prostate-specific membrane antigen(PSMA).Radionuclide drug conjugates(RDCs)targeting the PSMA antigen have become a hot topic in PCa research.The development of PSMA-targeted RDCs has progressed rapidly in recent years,significantly contributing to the diagnosis and treatment of PCa.Numerous novel PSMA-related RDCs are now part of clinical research.Due to the special properties of radiopharmaceuticals,it is essential to consider the impact of ionizing radiation in comprehensive clinical evaluation.This article,review the clinical studies from the past decade,and discusses the scope and methodologies of comprehensive clinical evaluation for PSMA-related RDCs.

Objective To explore the value of 18F-FDG PET/CT in diagnosis of retroperitoneal fibrosis (RPF).Methods Totally 12 RPF patients underwent 18 F-FDG PET/CT scanning,and the characteristics of metabolic activity,distribution of retroperitoneal lesions and maximum standardized uptake value (SUVmax) were analyzed retrospectively.Results Among 12 patients,7 were initial evaluation patients,5 were post-treatment patients.Of 7 initial evaluation patients,there were 4 patients with secondary RPF,including prostate carcinoma,breast cancer and IgG4-related disease.Retroperitoneal mass isodense with muscle surrounding the abdominal aorta or the iliac arteries were found in 12 patients,and ureteral involvement were found in 11 paients (11/12,91.67％).SUVmax in initial evaluation patients (4.21±1.76) was higher than that in patients after treatment (1.46±0.25,P＜0.05).According to PET/CT,3 idiopathic RPF patients as initial evaluation with high metabolic activity lesions received immunosuppressive therapy including corticosteroids and tamoxifen,and 4 secondary RPF patients with high metabolic activity lesions received treatment based on causes.Among 5 post-treatment patients,3 continued to accept current corticosteroids maintenance dose,while 2 did not receive any other treatment.Conclusion PET/CT may be used to evaluate the activity and extension of lesions in patients with RPF.

Objective To investigate the characteristics of metabolic foci on 18F-fluorodeoxyglucose (FDG) PET/CT scan in pediatric patients with epilepsy.Methods Twenty-three pediatric patients (15 males,8 females,age range:0.5-13.3 years) with epilepsy were retrospectively reviewed from March 2014 to December 2016.All patients underwent 18F-FDG PET/CT and metabolic foci were found.The visual method and semi-quantitative analysis were used to analyze images.Fourteen of them underwent surgery and were followed up for 3-24 months.Results Glucose hypermetabolism were observed most frequently in the frontal and parietal lobes,with or without surrounding/remote hypometabolism.On the day of PET/CT imaging,8 patients had no seizures,14 patients had seizures,and 1 patient was uncertain.The sites of resection were consistent with the regions of hypermetabolism in 9 patients,among whom the pathological results showed 8 cortical malformations and 1 Rasmussen's syndrome.Follow-up results for the above 9 patients showed that there was 7 Engel class Ⅰ patients and 2 Engel class Ⅲ patients.Conclusion The hypermetabolism may mostly appear in the frontal and parietal lobes of pediatric patients with epilepsy,and malformations of cortical development seem to be the most common pathology results.

Purpose To evaluated the effect of ureteral stent placement before flexible ureteroscopic lithotripsy(FURL).Methods A systematic search of PubMed,Cochrane Library,Embase,Scopus,VIP,CNKI,Wanfang database from databases establishment to February 2017 was performed to identify all clinical trials on the effect of ureteral stenting before flexible ureteroscopic lithotripsy.The outcomes included stone-free rate,mean operative time,success rate of ureteral access sheath placement and postoperative complications.RevMan 5.3 software was used to complete the Meta statistical analysis.Results Three randomized and four non-randomized studies were analyzed,which consisted of 1 176 patients including 788 cases in experimental group,388 cases in control group.Meta-analysis showed significant differences between experimental group and control group in stone-free rate (OR =1.88,95％ CI 1.30-2.71,P ＜ 0.001).There was no statistically significant difference in mean operative time between experimental group and control group (WMD =-0.99,95 ％ CI-10.63-8.65,P =0.84).The success rate of ureteral access sheath placement was significantly higher in experimental group than that in the control group (OR =8.24,95％ CI 3.17-21.45,P ＜ 0.001).In term of postoperative complications,two groups had significant differences (OR =0.57,95 ％ CI 0.33-0.99,P =0.04).Conclusions Preoperative ureteral stenting can increase the stone-free rate and the success rate of ureteral access sheath placement,and reduce complications of FURL.There is no statistically significant difference in mean operative time.

Objective To observe the metabolic changes of subcortical structures in children with intractable epilepsy using 18 F-FDG PET/CT,and to investigate the mechanism of subcortical structure involvement in epileptic seizures and its clinical significance.Methods Features of 18F-FDG PET/CT imaging in 611 intractable epilepsy children were analyzed.The metabolic changes of cortex and subcortical structures (basal ganglia,thalamus and cerebellum) were observed.The children were divided into three groups (young,middle and older groups) according to age,also mild group and severe group according to the number of involved lobar,respectively.The incidence of metabolic abnormalities in subcortical structures of different groups were analyzed.Results Among 611 children,unilateral cortical metabolic abnormality was found in 525,and bilateral cortical metabolic abnormalities were found in 86 children.The involvement of subcortical structures was detected in 190 children,including basal ganglia (n=64),thalamus (n=113) and cerebellum (n=105).The incidence of metabolic abnormality in subcortical structures under different age groups was not statistically different (all P＞ 0.05),while the incidence of metabolic abnormality in subcortical structures of severe group was significantly higher than that of mild group (all P＜0.001).Conclusion 18 F-FDG PET/CT might be able to detect the metabolic abnormalities of subcortical structures,therefore indicating the involvement of cerebral cortex.

Objective To retrospectively analyze the platelet count and related factors in bleeding patients with hematonosis,and to calculate the risk of bleeding when the platelet count is at each exposure level.Methods Retrospective analysis of patients from Department of Hematology Inpatients in Nanjing Drum Tower Hospital,Nanjing First Hospital and Nanjing Jiangning Hospital from July 2013 to June 2017 was collected.And the risk of bleeding for different hematonosis was calculated.Results The tolerance of the 5 categories of hematonosis to low platelet counts is compared:AA and ITP can tolerate lower levels of platelet count;MDS and AML(except M3) are more prone to bleeding;ALL is the most susceptible to bleeding.Conclusion When platelet resources are scarce,priority should be given to ALL,MDS and AML patients,in order to ensure the safety of critically ill patients.For patients with AA and ITP,the platelet infusion threshold may be reduced appropriately,in oder to reduce the incidence of platelet transfusion refractoriness.

Objective To investigate the clinical value of 18 F?FDG PET/CT in staging multiple myeloma ( MM) and evaluating the glucose metabolic activity of MM. Methods A total of 25 MM patients ( 13 males, 12 females, age:39-67 years) from May 2010 to April 2015 were enrolled in this retrospective study. The SUVmax of each patient was recorded. D?S plus staging according to 18 F?FDG PET/CT was com?pared with the traditional D?S staging. The SUVmax and the percentage of plasmacytes of bone marrow of phase Ⅲ and non?phase Ⅲ ( phaseⅠand Ⅱ) according to D?S plus staging were compared. Two?sample t test and Wilcoxon rank sum test were used to analyze the data. Results In 25 MM patients, the range of SUVmax of lesions was 1.8-12?0 and the mean value was 5.15±2.74. According to D?S staging, the numbers of patients with phase Ⅰ,Ⅱ andⅢwere 7, 4 and 14, respectively. While the numbers were 3, 1 and 21 by D?S plus staging. Based on the D?S plus staging system, stages of 7 patients ( 28%, 7/25 ) were changed. According to the D?S plus staging system, the SUVmax between phaseⅢand non?phaseⅢpatients was significantly different (5.75±2.54 vs 3.00±0?70; t=2.12, P<0.05), while the percentage of plasma?cytes of bone marrow between the 2 groups had no significant difference ( 17. 50%( 4. 25%-41. 75%) vs 11?15%(10.25%-36.57%);z=0.05, P>0.05). Conclusion 18F?FDG PET/CT is of clinical importance for MM staging and metabolic activity assessment of MM.

Objective To evaluate the effect of nimodipine on the activity of calcineurin (CaN) in the hippocampus of aged rats.Methods Sixty healthy male Sprague-Dawley rats,aged 18 months,weighing 550-650 g,were divided into 2 groups (n=30 each) using a random number table:surgery group (group S) and nimodipine group (group N).Nimodipine 1 mg/kg was intraperitoneally injected in group N,while the cqual volume of normal saline was given instead in group S,and 30 min later exploratory laparotomy was performed.Ten rats in each group were randomly selected on 1 day before operation and 3 and 7 days after operation,and Morris water maze test was performed.After the end of Morris water maze test,10 rats were selected and sacrificed,brains were removed,and hippocampi were isolated for detection of apoptosis in hippocampal neurons (by flow cytometry) and expression of CaN,phosphor-BAD (p-BAD) and caspase-3 in hippocampal tissues (by Western blot).Apoptotic rate was calculated.Results Compared with the value at 1 day before operation,the escape latency was significantly prolonged,the frequency of crossing the original platform was decreased,the time of staying at the platform quadrant was shortcned,apoptotic rate was increased,and the expression of CaN,p-BAD and caspase-3 in hippocampal tissues was up-regulated at each time point after operation in group S (P＜0.05).Compared with group S,the escape latency was significantly shortened,the frequency of crossing the original platform was increased,the time of staying at the platform quadrant was prolonged,apoptotic rate was decreased,and the expression of CaN,p-BAD and caspase-3 in hippocampal tissues was down-regulated at each time point after operation in group N (P＜0.05).Conclusion The mechanism by which nimodipine inhibits apoptosis in hippocampal neurons and reduces postoperative cognitive dysfunction may be related to inhibition of CaN activation in aged rats.