Objective To discuss the clinical value of tumor components of renal angiomyolipoma(AML)in predicting the efficacy of selective arterial embolization(SAE).Methods The clinical data of 20 patients with AML,who received SAE treatment at the General Hospital of Ningxia Medical University of China between August 2019 and April 2023,were retrospectively analyzed.The pre-SAE and post-SAE total tumor volume,fat volume(FV),non-fat volume(NFV),proportion of FV,proportion of NFV were calculated.Pearson correlation analysis was used to analyze the relationship between the initial volume of each tumor component and the tumor volume reduction rate.Multiple linear regression analysis was used to analyze the factors affecting the tumor volume reduction rate.Results The postoperative tumor volume,FV,and NFV were all significantly reduced when compared with their preoperative values(all P＜0.01).The postoperative proportion of FV was increased,and the postoperative proportion of NFV was decreased(P＜0.05).The postoperative tumor volume reduction value was closely correlated with the volume of tumor components and the presence of rupture(P＜0.05).Multivariate regression analysis revealed that the proportion of NFV was the independent risk factor for reduced tumor size.Conclusion After SAE,the proportion of NFV in AML is decreased.The preoperative measurement of this index can help clinicians to predict the postoperative tumor volume reduction ratio and to evaluate the postoperative efficacy of patients.

Objective:To investigate the incidence and prothrombotic risk factors of postoperative venous thromboembolism(VTE) in Cushing′s disease and to further develop an assessment model to identify those at high risk of postoperative VTE events.Methods:A retrospective study was performed in 82 patients who were admitted to Huashan Hospital, Fudan University during January 2019 and January 2020 and diagnosed with Cushing′s disease. These patients underwent the evaluation about their clinical, hormonal, and coagulation parameters, as well as ultrasonography and pulmonary angio-CT when necessary. The least absolute shrinkage and selection operator(LASSO) regression analysis was used to screen independent risk factors, and a nomogram model for postsurgical VTE risk assessment in Cushing′s disease was initially established, and Bootstrap method was used for internal verification. Finally, the predictive model was evaluated for calibration and clinical applicability in the study cohort.Results:Nineteen patients(23.17%) developed VTE events, with 14 cases occurring after endoscopic transsphenoidal surgery. Compared to patients without VTE, those in the VTE group were older( P<0.001), had longer postoperative bed rest, higher rates of current infection, higher HbA 1C levels, and more severe glucose tolerance impairment(all P<0.05). Through LASSO regression analysis, two independent risk factors for postoperative VTE were identified: Age and current infection. Then a VTE risk assessment nomogram model was established to predict the patients at high risk of VTE. In the nomogram model for VTE risk assessment, the area under the receiver operating characteristic curve was 0.868(95% CI 0.787-0.949), with the calibration curve closely aligning with the ideal diagonal line and the clinical decision curve exceeding the two extreme curves. Conclusions:Advanced perioperative assessment needs to be taken to screen those with high VTE risks in patients diagnosed with Cushing′s disease. Additionally, during the perioperative period, patients with Cushing′s disease should undergo mandatory physical activity or prophylactic anticoagulant therapy.

Objective:To explore the independent influencing factors of patients with spontaneous rupture hemorrhage of primary liver cancer (PLC).Methods:A retrospective cohort study was conducted. The clinical data of 128 patients with PLC spontaneous rupture hemorrhage in Ningxia Medical University General Hospital from January 2017 to March 2022 were analyzed, including 108 males and 20 females, aged (53.4±10.6) years. According to different treatment, 128 patients were divided into liver resection group (LR, n=28), interventional group [ n=39, transcatheter arterial chemoembolization (TACE) and transcatheter arterial embolization (TAE)], and conservative group ( n=61). Univariate and multivariate Cox regression was performed to analyze prognostic factors. The LR and TACE groups were subdivided into LR (aLR, n=15), TACE/TAE (aTACE, n=33) and LR+ TACE ( n=19) groups. Kaplan-Meier analysis was performed, and the survival rate was compared by log-rank test. Results:The median survival time of LR group and TACE group was 23 months and 21 months, respectively, with no statistical significance ( P>0.05). The median survival time (38 months) in LR+ TACE group was significantly longer than that in aLR group (10 months) and aTACE group (9 months), and the difference was statistically significant ( P<0.05). Univariate analysis showed that Barcelona Clinical Liver Cancer (BCLC)staging, tumor length ≥10.0 cm, vascular invasion, α-fetoprotein ≥400 μg/L, total bilirubin, prothrombin time and treatment affected overall survival of PLC spontaneous rupture hemorrhage patients (all P<0.05). Multivariate analysis showed that BCLC staging, tumor length ≥10.0 cm, Child-Pugh grade and treatment were independent influencing factors for overall survival of PLC spontaneous rupture hemorrhage patients (all P<0.05). Conclusion:BCLC stage, tumor length ≥10.0 cm, Child-Pugh grade and treatment method are independent predictors of overall survival in patients with spontaneous rupture of PLC. LR combined with TACE therapy can improve the survival and prognosis of patients with spontaneous rupture of primary liver cancer.

The incidence of each of the rare disease is very low. The complexity and diagnosis difficulty of the rare disease lead to the difficulties in the clinical research and development (R&D) of drugs for rare diseases. There is an urgent clinical need for the drug development of rare diseases in China. Encouraging R&D of new drugs, particularly the innovative drugs with China's own independent intellectural property is the basis for solving the predicament in drug shortage in China.. In order to further improve the efficiency of clinical R&D of drugs for rare diseases, the National Medical Products Administration (NMPA), Center for Drug Evaluation (CDE) issued Technical Guidance for Clinical Research and Development of Drugs for Rare Diseases. This is the first guidance for rare diseases in China that is drafted from the standpoint of the clinical technology research and development.The guidance is the scientifitc thinking and framework for the drug developing enterprises to research and develop drugs for rare disease efficiently and appropriately by following drug developing protocols and relating to the special features of rare disease.This paper presents the concepts and rationale in the guidance for the appraisal of rare disease drug research and development.

Objective:To investigate the safety and efficacy of transcatheter genicular artery embolization (GAE) for moderate to severe knee osteoarthritis (KOA).Methods:This prospective study included 13 patients (17 knees) with KOA who were treated with GAE from October 2020 to March 2021. The Kellgren-Lawrence (K-L) grade was 2-3 for 11 knees, and 4 for 6 knees. The Western Ontario and McMaster Universities osteoarthritis index (WOMAC) and the Whole-Organ Magnetic Resonance Imaging Score (WORMS) assessments were performed for all the subjects before operation. The success rate, clinical efficacy and complications were recorded after operation. Clinical outcomes were evaluated at 1 day, 1week and 1, 3, 6 months after the operation.Results:The success rate of GAE in 17 cases was 100%, and the success rate of target artery superselection was 98.4%(63/64). The baseline WOMAC pain score was 11(10, 13) and total score was 44(38, 58) for 17 knees. Post-operation follow-up WOMAC pain score were 4(3, 7), 2(1, 5), 2(1, 6) and 4(2, 6) at 1 day, 1 week, 1 month, and 3 months. Post-operation follow-up WOMAC total score were 22 (15, 34),20 (12, 24),17 (12, 26) and 20 (12, 31) at 1 day, 1 week, 1 month, and 3 months. There were 16 knees with 6 month follow-up assessment, with the WOMAC pain score of 2.5(2, 5), and the total score of 15(12, 26). Significant difference was found in the WOMAC pain score between baseline and the 1 day, 1 week, 1, 3 and 6 months follow up ( Z=-3.631, -3.623, -3.622, -3.622, -3.532, all P<0.001); also, the total score was statistically significant different between the baseline and the 1 day, 1 week, 1, 3 and 6 months follow up ( Z=-3.639, -3.634, -3.646, -3.527, -3.532, all P<0.001). At 3 months follow-up, there was 1 knee recognized clinical failure. Post-operative adverse reaction in this group included skin ecchymosis in femoral artery puncture area ( n=3), knee joint stiffness and pain within 1 week ( n=4),and clanging joints during postoperative activities ( n=6). Conclusion:GAE is a feasible and safe procedure with obvious short-term curative effect, which can alleviate pain symptoms and improve restricted movement in patients with KOA.

Objective:To investigate the clinical application value of blood oxygen level dependent functional magnetic resonance imaging (BOLD-fMRI) to the protection of language function in patients with unilateral frontal and temporal lobes glioma receiving postoperative intensity modulation radiation therapy (IMRT).Methods:A total of 27 patients with unilateral frontal and temporal lobe gliomas were treated with postoperative radiotherapy. The planning CT and BOLD-fMRI were performed before radiotherapy, and the language functional areas were delineated based on the fused images of 3D T1 and CT. IMRT technology was used to develop radiotherapy plans with and without language function area protection, naming conventional and protective radiotherapy plans respectively. The maximum radiation dose ( Dmax), average radiation dose ( Dmean), target conformal (CI) and dose uniformity (HI) of PTV of the two plans were compared and analyzed to ensure that the protective radiotherapy plan could meet the radiotherapy standard. Then, the Dmax and Dmean of the language function area were compared and analyzed to evaluate whether the Dmax and Dmean of the language function area were decreased in the protective radiotherapy plan. Results:There were no significant differences in CI, HI, Dmax and Dmean of PTV between the conventional radiotherapy plan and protective radiotherapy plan ( P>0.05). There were statistically significant differences in Dmax and Dmean of Wernicke′s and Broca′s (healthy side and affected side) between the conventional radiotherapy plan and protective radiotherapy plan ( t=3.073-12.707, P<0.05). Dmax and Dmean of Wernicke′s and Broca′s (healthy side and affected side) were decreased in the protective radiotherapy plan compared with the conventional radiotherapy plan, and the decrease was significant in the healthy side. Conclusions:BOLD-fMRI combined with IMRT can not only guarantee the target dose of patients with glioma receiving postoperative radiotherapy, but also reduces the radiation dose to the language function area. Chinese reading task and paragraph comprehension task are the stimulation mode of language function in patients after brain tumor surgery. These tasks are simple and the effect is accurate.

Objective:To analyze the hepatic hemodynamics changes after partial splenic embolization in patients with cirrhosis and hypersplenism.Methods:A total of 26 patients with cirrhosis and hypersplenism who underwent partial splenic embolization in the General Hospital of Ningxia Medical University from April 2018 to June 2019 were included in this study. The clinical data was retrospectively studied. The study objects consisted of 19 males and 7 females with an average of 55 years. Whole-liver perfusion enhanced CT imaging scan was performed for all patients. Changes in laboratory indexes and the blood perfusion of the liver sections including hepatic arterial perfusion, portal venous perfusion, total liver perfusion and hepatic arterial perfusion indexes before PSE and postoperative 1 month were compared.Results:The postoperative leukocyte and platelet counts increased, and the prothrombin time decreased in the 26 patient, and the differences were significant ( P<0.05). The hepatic arterial perfusion of the left lateral section, left medial section, right anterior section and right posterior section increased in postoperative 1 month, from 10.0 (7.0, 13.5) ml·min -1·(100 ml) -1, 9.3 (5.4, 12.8) ml·min -1·(100 ml) -1, 8.0 (6.0, 11.2) ml·min -1·(100 ml) -1, 10.7 (7.1, 13.8) ml·min -1·(100 ml) -1 to 7.7 (4.2, 11.0) ml·min -1·(100 ml) -1, 6.9 (2.6, 10.2) ml·min -1·(100 ml) -1, 7.1 (4.1, 8.7) ml·min -1·(100 ml) -1, 5.9 (4.4, 8.5) ml·min -1·(100 ml) -1, respectively. The differences were all significantly different ( P<0.05). There were no significant difference in portal venous perfusion and total liver perfusion before and after operation ( P>0.05). The hepatic arterial perfusion index of left lateral section, right anterior section and right posterior section increased after operation ( P<0.05). Conclusion:For patients with cirrhosis and hypersplenism who underwent partial splenic embolization, the state of hypersplenism was relieved, the hepatic arterial blood flow increased, and the liver function improved.

Objective:To explore the correlation of cognitive impairment with abnormal brain regions having low-frequency fluctuation (ALFF) by resting-state blood oxygen level-dependent functional magnetic resonance imaging (BOLD-fMRI) in patients with mild hepatic encephalopathy (MHE).Methods:Fifty-nine patients (28 patients with chronic cirrhosis and 31 patients with MHE, according to results of digital symbol test [DST] scores/digital connection test A [NCT-A] scores) admitted to our hospital from March 2018 to January 2020 were enrolled; 33 healthy control subjects matched in gender, age and years of education were chosen as controls. Resting-state BOLD-fMRI was performed in all subjects, and the original images were processed to obtain ALFF brain map. Statistical methods were used to obtain the brain regions with different ALFF values in the three groups, and the brain regions enjoying correlations between ALFF values and venous blood ammonia content/DST scores/NCFA scores in patients from the MHE group.Results:As compared with the control group, patients in the MHE group exhibited significantly lower ALFF values in the right central posterior gyrus, bilateral cuneiform lobe, left lateral occipital gyrus, left occipital gyrus, and left pars opercularis, and significantly increased ALFF values in the right thalamus, left parahippocampal gyrus and surrounding ventricle (bilateral caudate nucleus head)( P<0.05). Bivariate correlation analysis suggested that ALFF values were negatively correlated with blood ammonia in the left pars opercularis, left margin superior gyrus, bilateral precuneus, left superior temporal gyrus, right middle frontal gyrus and surrounding area, and right insula ( P<0.05); ALFF values were positively correlated with DST scores in left medial cingulate gyrus and right cerebellar hemisphere; ALFF values in the left frontal lobe and left posterior cerebellar lobe were positively correlated with NCT-A scores, and ALFF values in the left parahippocampal gyrus were negatively correlated with NCT-A scores ( P<0.05). Conclusion:The ALFF differences in multiple brain regions of patients with MHE may be related to cognitive impairment.

Objective To analyze the magnetic resonance imaging (MRI) images of patients with adult Japanese encephalitis (JE),and to investigate the diagnostic value of MRI for the disease.Methods Thirty-two adult JE patients who underwent cranial MRI at General Hospital of Ningxia Medical University between August 2016 and September 2018 were enrolled.All patients had disease onset between August and September and they aged 17 to 83 years old.The clinical data,laboratory results,MRI signal characteristics of each scanning sequence and the distribution of the brain lesions were retrospectively analyzed.Results Of the 32 adult JE patients,29 (90.6％)cases had acute onset,28 (87.5％) cases had unconsciousness and cognitive impairment,26 (81.2％) cases had intracranial hypertension,3 (9.4％) cases had meningeal irritation,3 (9.4％) cases had Parkinson-like symptoms,10 (31.2％) cases had epilepsy,and 15 (46.9％) cases had decreased muscle strength.Twenty patients were positive for JE virus-specific IgM antibodies.Twenty-eight patients underwent cerebrospinal fluid examination,15 (53.6％) cases showed intracranial pressure ≥180 mmH2O (1 mmH2O =0.009 8 kPa),7 (25％) cases developed lymphocyte reaction,and 16 (57.1％) cases showed mixed cell reaction.Twenty-three cases (71.9％) showed lesions of brain on MRI,including thalamus (17 cases,73.9％),hippocampus (13 cases,56.5％),cerebral peduncle (6 cases,26.1％),cortical and subcortical (4 cases,17.4％),basal ganglia (2 cases,8.7％),brainstem (1 case,4.3％) and splenium of corpus callosum (1 case,4.3％).Positive T1 weight image (T1WI) and T2 weight image (T2WI) results were found in 21 patients,respectively,23 patients had positive T2-fluid attenuated inversion recovery (FLAIR) images,and 20 patients had positive diffusion weighted imaging (DWI) images.Among them,T2-FLAIR and DWI images showed more lesions,wider range of lesions and clearer boundary of cortical involvement range than T1WI and T2WI images.Conclusions Bilateral thalamus and hippocampus are often involved in adult JE.T2-FLAIR and DWI sequences are more sensitive to detect lesions.Combining MRI images with epidemiological characteristics,clinical manifestations,and laboratory tests is of great assistance for early diagnosis of JE.

Objective To investigate the clinical value of blood oxygenation level-dependent functional magnetic resonance imaging(BOLD-fMRI)in the protection of visual function during postoperative radiotherapy for occipital lobe gliomas. Methods Twenty-eight patients with occipital lobe gliomas receiving postoperative radiotherapy from 2014 to 2016 were enrolled as subjects. All patients underwent computed tomography(CT) simulation,conventional MRI,and BOLD-fMRI before radiotherapy. The location and scope of the visual cortex on 3DT1anatomical images were used to guide the labeling of the visual cortex on simulated CT images. A visual cortex protective radiotherapy plan and a conventional radiotherapy plan were made by intensity-modulated radiotherapy. The feasibility of the visual cortex protective radiotherapy plan was evaluated using conformity index(CI),homogeneity index(HI),Dmax,and Dmeanfor planning target volume(PTV)and Dmaxand Dmean to the visual cortex. Results For the two plans, if the effective dose to target volume was guaranteed and the doses to conventional organs at risk were acceptable, there were no significant differences in CI or HI for PTV between them(P=0.874,P=0.602).Compared with the conventional radiotherapy plan,the Dmaxand Dmeanto the ipsilateral visual cortex were reduced by 8.40% and 9.25%, respectively, while the Dmaxand Dmeanto the contralateral visual cortex were reduced by 13.26% and 14.77%, respectively, in the protective radiotherapy plan. Conclusions With a guaranteed prescribed dose to target volume and BOLD-fMRI used as a guide, the visual cortex protective radiotherapy, compared with the conventional plan, can reduce the dose to the visual cortex and protect the corresponding functional areas.