Objective:To evaluate the hemodynamic situation of the patients with moyamoya disease using MR perfusion imaging,and to explore the relationship between compensatory collateral circulation and perfusion. Methods:Seventy-two hospitalized patients with moyamoya disease were selected as typical moyamoya disease group,including 37 males and 35 females,aged 10 - 62 years old,all patients underwent cerebral angiography (DSA)and MR perfusion imaging.And 20 patients with out neurological history were used as control group.With mean transit time (MTT)image as a standard,the abnormal perfusion ranges were classified as region of interest (ROI),and the corresponding perfusion parameter values,including cerebral blood flow (CBF),cerebral blood volume (CBV),MTT and time to peak (TTP)were recorded,respectively.The cerebellum was used as a reference in this study,the perfusion parameters were standardized,and the relative ratios of the perfusion parameters (rMTT,rTTP,rCBF,rCBV)were obtained.Results:Compared with control group,the rMTT and rTTP of the patients in typical moyamoya disease group were prolonged and the rCBF was reduced (P <0.05 or P < 0.01), but the rCBV had no obvious difference (P >0.05).②Compared with the contralateral side,the rMTT and rTTP of the suffered side were prolonged,and the rCBF and rCBV were reduced (P < 0.05 or P < 0.01).Compared with chronic onset group,the rCBV and rCBF of the patients in acute onset group were reduced (P <0.05 or P <0.01),but the rMTT and rTTP had no statistically significant difference (P > 0.05).There were no significant differences in all parameters between hemorrhagic moyamoya disease group and ischemia group (P > 0.05 ). Conclusion:MR perfusion imaging can accurately evaluate the hemodynamic condition of moyamoya disease;MTT and TTP hve higher sensitivities than CBF and CBV.MR perfusion imaging can evaluate the compensation of collateral circulation of moyamoya disease and provide the objective basis for the clinician to select the proper surgical timing and the best operation methods.

Objective To investigate the relationship of serum adiponectin (APN) with left ventricular hypertrophy (LVH) and carotid athcrosclerosis (AS),and to observe the change of serum adiponectin levels in patients with hypertension and obesity.Methods Patients with essential hypertension (50 cases) were divided into simple hypertension group (23 cases) and hypertension with obesity group(27 cases),and 45 normotensives were divided into control group (24 cases) and simple obesity group (21 cases).The levels of serum adiponectin were determined by enzyme immunoassay.Common carotid artery intima-media thickness (CCA-IMT),carotid artery diameter (CADIA) and left ventricular relevant index were measured by color doppler ultrasonography,and left ventricular mass index (LVMI) was calculated.Results In control group,simple obesity group,simple hypertension group and hypertension with obesity group,serum APN levels were decreased by turn,CADIA reduced gradually,LVMI values were increased by turn,CCA-IMT values were increased gradually (F=28.34,10.26,36.52,14.73,all P＜0.01).Correlation analysis showed that serum APN levels were significantly negatively correlated with LVMI and CCA-IMT(r=-0.870,-0.710,both P＜0.01),and were positively related with CADIA (r=0.742,P＜0.00).APN,SBP and BMI were the main influencing factors on left ventricular hypertrophy (β =-0.909,0.126,-0.053).Conclusions The presence of both hypertension and obesity may play a synergistic role in accelerating the process of left ventricular hypertrophy and atherosclerosis.Lower APN levels and/or rising BMI may accelerate occurrence and development of LVH and AS in aged patients with hypertension and obesity.

Objective To study the effect of contusion and exhaustive exercise on satellite cells' activation and Pax7/CBF1/DAPT contents in skeletal muscles of rats,and reveal the repair mechanism of the skeletal muscle injury.Methods Twenty-four seven-week-old male Sprague-Dawley(SD)rats were randomly divided into a control(C)group,an immediately after exhaustive exercise(E0)group,a 24 hours after exhaustive exercise(E24)group and a 48 hours after exhaustive exercise(E48)group,each of 6.Other 18 SD rats were randomly divided into 3 groups,6 rats in each group:an immediately after contusion group(D0),a 24h post-contusion group(D24)and a 48h post-contusion group(D48).All groups were killed at different time points after exhaustive exercise and the contusion respectively while the control group was at resting state,and their serum was extracted.The right gastrocnemius muscles were resected and divided into 2 parts:one was used immediately for culturing satellite cells,while the other was stored in the bridge at-80℃.All the serum and gastrocnemius muscles were tested for Pax7,CBF1 and DAPT contents using the enzyme-linked immunosorbent assay(ELISA).Moreover,three-day-old newborn rats were also slaughtered for the above experiment.Results The results of culture in vitro showed that one day after the culture began a small amount of spindle satellite cells could be seen in the newborn rats,but the satellite cells of other groups were growing slowly.However,on the third day and the fifth day,the spindle satellite cells of each group began to proliferate in quantity and reached the peak.Then all the satellite cells had a good proliferation until the seventh day after serial passage,and some patial fusions to microtubules occurred.The biggest cell number and proliferation rate was in newborn rats,but no skeletal muscle satellite cell was found in the control group.At the same time,the number and the proliferation rate of skeletal muscle satellite cells in contusion groups were significantly higher than the exhaustive exercise groups.The results of Elisa showed that the contents of CBF1 and Pax7 in skeletal muscles in exhaustive groups and contusion groups,were significantly upregulated,compared to the control group(P＜0.05).Amomg them,the CBF1 content in group D0,together with the Pax7 content in group D24 and D48,was significantly higher than that of the other groups(P＜0.05).On the other hand,the content of DAPT in skeletal muscles of exhaustive groups and contusion groups,compared to the control group,was significantly downregulated (P＜0.05).To be more specific,the value of group D0 was significantly lower than that of the other groups (P＜0.05).However,there was no significant difference in it among group E0,E24 and E48.Conclusions Contusion and exhaustive exercise can activate the skeletal muscle satellite cells to proliferate and differentiate.The number of activated skeletal muscle satellite cells in the contusion groups is significantly larger than that of exhaustive groups maybe due to the stronger stimulating by contusion.Both contusion and exhaustive exercise increase the contents of Pax7 and CBF1 in skeletal muscles,but decrease that of DAPT.The Pax7 and CBF1 may play a positive regulating role in terms of activating skeletal satellite cells and repairing skeletal muscle injury,while DAPT may play a negative regulating role.

Objective:To systematically evaluate the relationship between sedentary behavior (SB) and the risk of developing gestational diabetes mellitus (GDM).Methods:PubMed, Medline, EMBASE, CINAHL, the Cochrane Library, the Rehabilitation & Sports Medicine Source, the Sedentary Behavior Research Database (SBRD), Wanfang Database, the China Journal full-text database, and the China Biomedical Literature Database were searched for cohort studies, case-control studies, and cross-sectional studies concerning the risk of SB and GDM from the establishment of the database to January 2021. Two researchers screened the literature, extracted data, evaluated the quality of the data according to the inclusion and exclusion criteria, and, finally, produced a descriptive analysis of the results.Results:A total of 11 studies were included, including eight cohort studies, two cross-sectional studies, and one case-control study. The overall quality of the studies was moderate. The prevalence of GDM was 5.04%-26.81%, and the prevalence of SB before and during pregnancy was 39.47%-40.21% and 28.86%-93.50%, respectively. In terms of the prevalence of SB before pregnancy, four moderate-quality studies reported no association with GDM risk. In terms of SB during pregnancy, four moderate-quality studies reported no association with GDM risks, and four studies (one of high quality and three of moderate quality) reported an association with GDM risks, two of which focused on the second trimester. There was considerable heterogeneity between studies on the diagnosis of GDM and SB.Conclusion:SB before pregnancy is not associated with the risk of GDM, while the relationship between SB and the risk of GDM is unclear, but the level of SB in the second trimester of pregnancy may be positively associated with the risk of GDM.

OBJECTIVETo retrospectively analyze the experience of one-stage surgical correction for children with congenital heart diseases and tracheal stenosis and further to clear the principle of treatment for these patients.

METHODSFrom January 2006 to June 2013, 48 patients with congenital heart diseases and tracheal stenosis underwent surgical correction. There were 36 male and 12 female patients. The mean age at operation was (23 ± 27)months (range: 3-72 months) and the mean weight was (12 ± 8) kg (range: 3.4-46.0 kg). The underlying diagnoses were pulmonary sling in 33 patients, double aortic arch in 3, tetrology of Fallot in 6, ventricular septal defect in 4, double outlet right ventricle in 1, and pulmonary atresia in 1 patient. Among them, short tracheal stenosis was present in 15, long tracheal stenosis in 25 and bridging bronchus in 8 patients. Repairs with autogenous tracheal tissue were performed in 6, and end-to-end anastomosis in 11 and slide tracheoplasty in 31 patients. Two patients had granulation tissue growing in the airway postoperatively and were re-operated by autogenous rib tissue. All of patients were followed up after operation 1, 3, 6, 12 months and if the patient had symptoms that should be examined by bronchoscopy. The patients should be examined by CT post-operation one year.

RESULTSThere were 7 deaths in all 48 cases and the early mortality was 14.6%. Two deaths were not related to tracheal stenosis, and other 5 were associated with long segment tracheal stenosis. Forty-one patients were followed for 2 months to 6 years, and no patients required re-operations. Clinical symptoms of tracheal stenosis disappeared and the results of CT scan were satisfied.

CONCLUSIONSOne stage surgical repair of the patients with congenital heart diseases and tracheal stenosis have a good effect. Slide tracheoplasty is the effective surgical method for long segment tracheal stenosis. Postoperative granulation tissue growing in the airway is the leading cause of death.

Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Infant ; Male ; Retrospective Studies ; Tracheal Stenosis ; complications ; surgery

To evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center. Methods    From January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed. Results    All patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ. Conclusion    According to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.

Objective:To analyze the risk factors that affect the prognosis of patients with hypopharyngeal squamous cell carcinoma（HPSCC） and to compare the efficacy of surgical resection followed by adjuvant radiotherapy（SR） with that of neoadjuvant therapy consisting of platinum-based chemotherapy and fluorouracil combined with either cetuximab or nimotuzumab, followed by SR. The study also aimed to evaluate the overall survival（OS） of patients, their postoperative eating function, tracheostomy decannulation rate, and tumor response to the two neoadjuvant chemotherapies. Methods:A retrospective analysis was performed on the medical records of HPSCC patients who received SR or neoadjuvant therapy followed by SR treatment at the Shanghai General Hospital from 2012 to 2019 and had not undergone any prior treatment. The prognostic factors were analyzed, and the survival analysis of patients who underwent SR treatment with two neoadjuvant chemotherapy regimens was performed. Results:A total of 108 patients were included in the study. The results of the univariate analysis showed that gender（P=0.850） had no significant correlation with the survival rate of HPSCC patients who underwent SR. However, age, smoking history, alcohol consumption history, platelet-to-lymphocyte ratio（PLR）, neutrophil-to-lymphocyte ratio（NLR）, T stage, N stage, neoadjuvant therapy with either cetuximab or nimotuzumab combined with platinum-based chemotherapy and fluorouracil, and histological grade were significantly associated with prognosis（P<0.05）. The multivariate analysis revealed that smoking history, histological grade, and neoadjuvant therapy with either cetuximab or nimotuzumab combined with platinum-based chemotherapy and fluorouracil were independent risk factors affecting the prognosis of HPSCC（P<0.05）. Patients who received neoadjuvant therapy had longer OS than those who underwent SR only（P<0.001）. There was no significant difference in tumor response to the two neoadjuvant therapies and in OS（P>0.05）, and there was no significant difference in the rate of oral feeding and tracheostomy decannulation among the three treatment groups（P>0.05）. Conclusion:Univariate analysis showed that age at tumor onset, smoking history, alcohol consumption history, NLR, PLR, T stage, N stage, whether receiving neoadjuvant chemotherapy, and pathological grade were associated with the prognosis of HPSCC patients receiving SR treatment. Multivariate analysis showed that smoking history, pathological grade, and neoadjuvant chemotherapy were independent risk factors affecting the prognosis. Neoadjuvant chemotherapy with cetuximab or nimotuzumab can prolong the OS of patients, providing a certain basis and reference for the treatment of HPSCC.
Humans ; Neoadjuvant Therapy ; Squamous Cell Carcinoma of Head and Neck ; Cetuximab/therapeutic use* ; Retrospective Studies ; China ; Prognosis ; Fluorouracil ; Head and Neck Neoplasms

Objective:To investigate the current situation of the use of transjugular intrahepatic portosystemic shunt (TIPS) for portal hypertension, which should aid the development of TIPS in China.Methods:The China Portal Hypertension Alliance (CHESS) initiated this study that comprehensively investigated the basic situation of TIPS for portal hypertension in China through network research. The survey included the following: the number of surgical cases, main indications, the development of Early-TIPS, TIPS for portal vein cavernous transformation, collateral circulation embolization, intraoperative portal pressure gradient measurement, commonly used stent types, conventional anticoagulation and time, postoperative follow-up, obstacles, and the application of domestic instruments.Results:According to the survey, a total of 13 527 TIPS operations were carried out in 545 hospitals participating in the survey in 2021, and 94.1% of the hospital had the habit of routine follow-up after TIPS. Most hospitals believed that the main indications of TIPS were the control of acute bleeding (42.6%) and the prevention of rebleeding (40.7%). 48.1% of the teams carried out early or priority TIPS, 53.0% of the teams carried out TIPS for the cavernous transformation of the portal vein, and 81.0% chose routine embolization of collateral circulation during operation. Most of them used coils and biological glue as embolic materials, and 78.5% of the team routinely performed intraoperative portal pressure gradient measurements. In selecting TIPS stents, 57.1% of the hospitals woulel choose Viator-specific stents, 57.2% woulel choose conventional anticoagulation after TIPS, and the duration of anticoagulation was between 3-6 months (55.4%). The limitation of TIPS surgery was mainly due to cost (72.3%) and insufficient understanding of doctors in related departments (77.4%). Most teams accepted the domestic instruments used in TIPS (92.7%).Conclusions:This survey shows that TIPS treatment is an essential part of treating portal hypertension in China. The total number of TIPS cases is far from that of patients with portal hypertension. In the future, it is still necessary to popularize TIPS technology and further standardize surgical indications, routine operations, and instrument application.

Objective    To analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations. Methods    We retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation. Results    The top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation. Conclusion    Series operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.

  Objective  To study the demographic and clinical characteristics, correlation of genotype and phenotype and treatment of Blau syndrome to facilitate early diagnosis and timely treatment of Blau syndrome.  Methods  Seventy-two patients with Blau syndrome from 11 centers from May 2006 to April 2022 were retrospectively analyzed, and their general information, clinical data, laboratory examination and treatment medication were collected.  Results  The distribution of patients with Blau syndrome was uniform in geographical north and south of China, and there was no obvious gender bias. The mean age of onset was (14.30±12.81) months, and the age of diagnosis was (55.18±36.22) months. 35% of patients with Blau syndrome happened before 1 year old, and all patients developed before 5 years old. 87.50% (63/72) had granulomatous arthritis, 65.28% (47/72) had rash, 36.11% (26/72) had ocular involvement, 27.78% (20/72) had fever, and 15.28% (11/72) had pulmonary involvement. Arthritic manifestations of Blau syndrome were most at risk, followed by rash, ocular involvement, and fever.The first 25 months of the disease, the risk of developing a rash was the greatest. The risk of developing arthritis was the greatest between 25 months and 84 months. The main mutations were p.R334Q and p.R334W, and patients with p.R334Q mutation had relatively high incidence of fever (35.71%[5/14] vs. 14.29%[1/7], P=0.43) and ocular involvement (42.86%[6/14]vs. 28.57%[2/7], P=0.51). There was a relatively high incidence of rash (85.71%[6/7] vs. 64.29%[9/14], P=0.59) in patients with the p.R334W mutation. Forty-five patients(62.50%)were treated with a combina-tion of glucocorticoid and methotrexate. Twenty-two patients were treated with tumor necrosis factor antagonist in addition to glucocorticoid and methotrexate.  Conclusions  The risk of different clinical manifestations of Blau syndrome from high to low was arthritis, followed by rash, ocular involvement and fever. The main treatment was glucocorticoid combined with methotrexate, to which biological agents could be added.