Objective:To investigate the relationship between 1p/16q loss of heterozygosity (LOH) and 1p gain in Wilms tumor and their clinicopathologic characteristics and prognosis.Methods:A total of 175 Wilms tumor samples received from the Department of Pathology, Beijing Children′s Hospital from September 2019 to August 2022 were retrospectively analyzed. The histopathologic type and presence of lymph node involvement were evaluated by two pathologists. The clinical data including patients′gender, age, tumor location, preoperative chemotherapy, and tumor stage were summarized. Fluorescence in situ hybridization (FISH) was done to detect 1p/16q LOH and 1p gain and their correlation with the clinicopathological features and prognosis were analyzed.Results:Among the 175 samples, 86 cases (49.1%) were male and 89 (50.9%) were female. The mean age was (3.5±2.9) years, and the median age was 2.6 years. There were 26 (14.9%) cases with 1p LOH, 28 (16.0%) cases with 16q LOH, 10 (5.7%) cases of LOH at both 1p and 16q, and 53 (30.3%) cases with 1q gain. 1q gain was significantly associated with 1p LOH ( P<0.01) and 16q LOH ( P<0.01). There were significant differences ( P<0.01) between 1q gain, 1p LOH and 16q LOH among different age groups. The rate of 16q LOH in the high-risk histopathological subtype (50.0%) was significantly higher than that in the intermediate-risk subtype (13.6%, P<0.05). The frequency of 1q gain, 1p LOH, and 16q LOH in children with advanced clinical stages (Ⅲ and Ⅳ) was significantly higher than that in children with early clinical stages (Ⅰ and Ⅱ). 1q gain, 1p LOH, and 16q LOH showed no significant correlation with gender, unilateral or bilateral disease, chemotherapy, or lymph node metastasis. The progression-free survival (PFS) time for patients with 1q gain and 1p LOH was significantly shorter than those without these aberrations ( P<0.05). Additionally, the PFS time of patients with 16q LOH was slightly shorter than those with normal 16q, although the difference was not statistically significant. Patients with stage Ⅲ to Ⅳ disease exhibiting 1q gain or 1p LOH had a significantly higher relative risk of recurrence, metastasis, and mortality. Conclusions:1p/16q LOH and 1q gain are associated with age, high-risk histological type, and clinical stage in Wilms tumor. 1q gain and 1p LOH are significantly correlated with the prognosis of Wilms tumor.

The gastrointestinal tract is the largest digestive organ and the largest immune organ and detoxification organ, which is vital to the health of the body. Drosophila is a classic model organism, and its gut is highly similar to mammalian gut in terms of cell composition and genetic regulation, therefore can be used as a good model for studying gut development. target of rapmaycin complex 1 (TORC1) is a key factor regulating cellular metabolism. Nprl2 inhibits TORC1 activity by reducing Rag GTPase activity. Previous studies have found that nprl2 mutated Drosophila showed aging-related phenotypes such as enlarged foregastric and reduced lifespan, which were caused by over-activation of TORC1. In order to explore the role of Rag GTPase in the developmental defects of the gut of nprl2 mutated Drosophila, we used genetic hybridization combined with immunofluorescence to study the intestinal morphology and intestinal cell composition of RagA knockdown and nprl2 mutated Drosophila. The results showed that RagA knockdown alone could induce intestinal thickening and forestomach enlargement, suggesting that RagA also plays an important role in intestinal development. Knockdown of RagA rescued the phenotype of intestinal thinning and decreased secretory cells in nprl2 mutants, suggesting that Nprl2 may regulate the differentiation and morphology of intestinal cells by acting on RagA. Knockdown of RagA did not rescue the enlarged forestomach phenotype in nprl2 mutants, suggesting that Nprl2 may regulate forestomach development and intestinal digestive function through a mechanism independent of Rag GTPase.
Animals ; Drosophila/genetics* ; Mechanistic Target of Rapamycin Complex 1/metabolism* ; Mammals/metabolism* ; Carrier Proteins ; Tumor Suppressor Proteins/metabolism* ; Drosophila Proteins/genetics*

A novel structural dynamics test method and device were designed to test the biomechanical effects of dynamic axial loading on knee cartilage and meniscus. Firstly, the maximum acceleration signal-to-noise ratio of the experimental device was calculated by applying axial dynamic load to the experimental device under unloaded condition with different force hammers. Then the experimental samples were divided into non-specimen group (no specimen loaded), sham specimen group (loaded with polypropylene samples) and bovine knee joint specimen group (loaded with bovine knee joint samples) for testing. The test results show that the experimental device and method can provide stable axial dynamic load, and the experimental results have good repeatability. The final results confirm that the dynamic characteristics of experimental samples can be distinguished effectively by this device. The experimental method proposed in this study provides a new way to further study the biomechanical mechanism of knee joint structural response under axial dynamic load.
Animals ; Cattle ; Biomechanical Phenomena ; Knee Joint/physiology* ; Meniscus ; Mechanical Phenomena ; Weight-Bearing

Objective:To investigated the efficacy and safety of transurethral bladder neck incision and laparoscopic modified bladder neck Y-V plasty in the treatment of bladder neck contracture (BNC)after transurethral resection of prostate (TURP).Methods:The clinical data of 57 patients with BNC after TURP who were treated in the Department of Urology, Sixth People's Hospital, Shanghai Jiaotong University School of Medicine from January 2013 to December 2022 were retrospectively analyzed.And the patients were divided into two groups based on the different surgical approaches. There were 22 cases in the transurethral bladder neck incision group, with an average age of (73.75±7.62) years and the preoperative urinary flow Q max of (3.92±2.73) ml/s. The preoperative International Prostate Symptom Score (IPSS) was (26.92±3.34) points, and the quality of life (QOL) score was (4.83±0.72) points. There were 35 cases in laparoscopic modified bladder neck Y-V plasty group, with an average age of (68.57±9.31) years and the preoperative urinary flow Q max of (2.56±1.27)ml/s. The preoperative IPSS was (27.08±3.06) points, and the QOL score was (5.08±0.84) points. The patients underwent transurethral bladder neck incision: Scar tissue was incised at 3, 9, and 12 o'clock in the bladder neck, and the incision depth reached the external fat of the bladder neck at 3 and 9 o'clock. Patients with significantly elevated bladder neck were treated with plasma electrosurgical resection to remove scar tissue. The patients underwent laparoscopic modified bladder neck Y-V plasty: After proper exposition of the bladder neck, the scar tissue was excised. the anterior bladder wall was incised in an inverted Y-shaped manner, the apex of the V-shaped flap was sutured to the distal urethrotomy to create a widened bladder neck. The postoperative urinary flow Q max, IPSS, and QOL of the two groups were compared. Results:All patients underwent surgeries successfully, with a one-time success rate of 94.3% (33/35) in the laparoscopic modified bladder neck Y-V plasty group, which was higher than the one-time success rate of 68.2% (15/22) in the transurethral bladder neck incision group( P<0.01). There were statistically significant difference in operation time [(31.75±12.81)min vs. (68.57±22.36)min] and postoperative hospital stay [(1.73±0.94)d vs. (5.17±2.12)d] between the transurethral bladder neck incision group and the laparoscopic modified bladder neck Y-V plasty group ( P<0.05). The median follow-up period was 12.6 (7.3, 27.8) months. The IPSS of the transurethral bladder neck incision group and the laparoscopic modified bladder neck Y-V plasty group were (9.92±2.56) points and (7.16±2.21) points, respectively. The QOL was (2.76±1.24) points and (1.31±0.95) points, respectively. The urinary flow Q max at 6 months after operation was (15.13±4.68)ml/s and (19.96±4.17)ml/s, respectively. There was statistical significance( P<0.05). Conclusions:Both laparoscopic modified bladder neck Y-V plasty and transurethral bladder neck incision are safe and effective in the treatment of BNC after TURP, and laparoscopic modified bladder neck Y-V plasty has a better clinical therapeutic effect.

OBJECTIVE： To mine and evaluate the post-marketing safety alert signals of pegaspargase （PEG-ASP） and L-asparaginase （L-ASP），and compare the safety differences between them ，so as to provide reference for clinical safe and rational drug use. METHODS ： The adverse drug event （ADE） reports of PEG-ASP and L-ASP issued by FDA adverse event reporting system from Jan. 1st，2004-Jun. 30th，2020 were retrieved. BCPNN method was used to mine the safety signals of these two drugs under the condition that the lower limit of information component （IC－2SD）＞0 and the number of events ≥3. The medium and strong signals of two drugs with IC －2SD≥1.5 were evaluated and compared in 8 system organ class，such as gastrointestinal system ，hepatobiliary system ，blood and lymphatic system ，blood vessels and lymphatic vessels ， nervous system ，immune system ，metabolism and nutrition ，various examinations. IC value of specific ADE signal and its 95％ confidence interval were analyzed by time scanning spectrum. RESULTS & CONCLUSIONS ：The reports of PEG-ASP and L-ASP as suspected drugs were 2 324 and 3 824；67 and 68 medium and strong signals were included ，respectively. In gastrointestinal system，the common strong signal of PEG-ASP and L-ASP was necrotic pancreatitis. In hepatobiliary system ，both of them showed strong signal in venoocclusive liver disease ，and this ADE was not included in the drug instruction. In blood and lymphatic system ， common strong signals of the two drugs were febrile neutropenia ，coagulation disorder ，neutropenia and febrile bone marrow regeneration disorder ；in blood vessels and lymphatic vessels ，in addition to haemodynamic instability ，IC values of other signals of L-ASP were higher than those of PEG-ASP. In nervous system ，IC values of other signals of L-ASP were higher than those of PEG-ASP except for intracranial haemorrhage. In immune system ，anaphylactic reaction was a medium signal for L-ASP but was a strong signal for PEG-ASP. In metabolism and nutritional diseases ，except for tumor lysis syndrome ，IC values of other signals of L-ASP were higher than those of PEG-ASP. The results of time scanning spectrum showed that the signals of necrotic pancreatitis and coagulation disorder of PEG-ASP were stable ，while the signals of veno occlusive liver disease and hypersensitivity were unstable and needed to be observed ；above four signals of L-ASP were stable signals. When using PEG-ASP or L-ASP clinically ， close attention should be paid to the safety problems such as hypersensitivity ，coagulation disorder ，thrombosis，necrotic pancreatitis，venoocclusive liver disease and hypoproteinemia.

Objective: To explore the recovery time and risk factors of coagulopathy caused by rodenticide poisoning through analyzing and following up the confirmed cases, and to provide more useful guidance information for the clinic practice. Methods: A total of 96 cases with coagulation dysfunction caused by anticoagulant rodenticide poiso-ning in Children′s Hospital, Chongqing Medical University from January 2014 to December 2016, were analyzed retrospectively.The recovery time of coagulation function and the relationship between recovery time and drug involved way, dysfunction organs and poison concentration were studied respectively. Results: (1) A total of 96 patients were hospitalized because of severe coagulopathy caused by the poisoning of second generation anticoagulant rodenticide.Brodifacoum was detected from 33 blood samples and the median concentration was 364 μg/L (55-4 654 μg/L). Bromadiolone was detected from 7 blood samples and the median concentration was 130 μg/L (18-652 μg/L). Brodifacoum and Bromadiolone were both detected from 8 cases and the median concentration was 741 μg/L (63-6 000 μg/L) and 11 μg/L (3-3 694 μg/L), respectively.(2) A total of 57 cases of the patients were successfully followed up.A total of 18 cases were confirmed with oral poisoning, 16 cases with dermal poisoning, while 23 cases denied any involved ways of poisoning, and 7 cases had organs dysfunction.The follow-up time was 12-54 months.All the hospitalized patients were given specific antidote Vitamin K treatment and recovered successfully without any sequelae.(3) The median recovery time of coagulopathy caused by rodenticide poisoning was 2.5 months.(4) The recovery time of coagulation function was positively correlated with the plasma concentration of Brodifacoum(r=0.619, P<0.01). (5) There was no significant correlation between recovery time and organ dysfunction or drug involved ways of poisoning involvement (all P>0.05). Conclusions The recovery time of coagulation dysfunction caused by anticoagulant rodenticide in children is much longer.The higher the concentration of Brodifacoum poison is, the longer the recovery time.Enough supplementation course of Vitamin K should be given according to the follow-up of coagulation function.

Objective: To analyze the diagnostic value of skeletal muscle biopsy in patients with rhabdomyolysis. Methods: Clinical and pathological data of 26 patients with rhabdomyolysis from January 2002 to December 2018 undergoing muscle biopsy were collected. Results: Eighteen males and 8 females were finally recruited with median age of 6-73 (37.3±19.6) years. The average time from onset to biopsy was 44 days (median course was 30 days). All patients had acute manifestations with muscle pain and/or weakness. Serum creatine kinase was between 1 648-92 660 U/L. Muscle biopsies showed nonspecific changes in 12 cases (a few with type 2 muscle fiber atrophy, slight deposition of lipid droplets), 10 cases with necrotizing myopathy (muscle fiber necrosis and regeneration). Toxic neurogenic damages were seen in 2 cases (type 1 and type 2 angular atrophic muscle fibers with group change), lipid storage disease in 1 case (lipid droplets deposit significantly) and idiopathic inflammatory myopathy in 1 case (muscle fiber necrosis and regeneration, with lymphocyte infiltration). The etiology of non-specific pathological changes included short-term strenuous exercise in 6 patients, poisoning in two, chronic kidney disease in one, viral infection in one, hypothyroidism in one and unknown reason in one. As to patients with necrotizing myopathy, seven were poisoning or drug-related, one with hyperthyroidism, two with unknown reason. Conclusions Among the numerous causes of rhabdomyolysis, exercise usually links nonspecific skeletal muscle changes and poisoning or drug-related disorders are commonly associated with necrotic myopathy. Rhabdomyolysis induced by primary myopathy is rare.

Objective To investigate the feasibility of short-segment percutaneous pedicle screw non-fusion fixation plus electro-acupuncture for the treatment of single thoracolumbar fracture.Methods Forty-three patients with single thoracolumbar fracture were treated with short-segment percutaneous pedicle screw fixation without fusion,and then were given electro-acupuncture on points such as Jiaji (EX-B2),Huantiao (GB30),and Zhibian (BL54) after the operation.Before and after operation and during the follow-up,X-ray images of the spine at positive position and lateral position were taken for the measurement of vertebral anterior and posterior diameter ratio and the vetebral kyphosis Cobb's angle.Lumbago visual analogue scale (VAS) scores and Oswestry scores for lumbago function disorder were used for the evaluation of clinical efficacy.Results (1) All of the 43 patients were followed-up,and the follow-up period lasted for 10-27 months.(2) After the operation and at the end of follow-up,vertebral anterior and posterior diameter ratio and Cobb's angle were much improved (P ＜ 0.01compared with those before the operation),and the two indexes at the end of follow-up were improved as compared with those after the operation (P ＜ 0.05).(3) One week after the operation and at the end of follow-up,VAS scores and Oswestry scores were obviously improved as compared with those before treatment (P ＜ 0.01),and the two kinds of scores at the end of follow-up were also improved as compared with those after the operation (P ＜0.01).Conclusion Short-segment percutaneous pedicle screw fixation without fusion plus electro-acupuncture at points such as Jiaji is effective for the treatment of single thoracolumbar fracture by relieving pain in the waist and back,which ensures the satisfactory effect of operation.

Objective To study the microscopic changes of white matter and the relationship between white matter changes and cognitive impairment in Alzheimer's disease (AD) using voxel-based analysis of DTI. Methods Thirty-sev?en patients with probable AD,and 32 normal controls(NC) were all examined by MMSE scores, and underwent a diffusion tensor imaging. The value of FA changes and the correlations between FA and MMSE scores were investigated. Results FA reduction was detected in the right frontal, temporal and parietal lobes as well as the thalamus, the bilateral cingu?lum, corpus callosum, precuneus, inferior parietal lobule, inferior patieto gyrus, supramaginal gyrus and hippocampus in AD. FA values in the right cingulum, left corpus callosum, left inferior temporal gyrus and the bilateral inferior parietal lobule, inferior patieto gyrus and precuneus were significantly decreased in AD than in the health control groups (P<0.05, FWE corrected). There was a positive correlation between the values of FA and MMSE scores(P<0.001,uncorrect?ed). Conclusion AD patients have significant reduction of FA values in the specific regions. There is a positive correla?tion between white matter changes and impairments of cognitive function.

Objective To observe the expression of AdipoQ in Sj?gren's syndrome (SS) mice and its role in inflammation was investigated by recombinant gene transfection study in vivo. Methods As spon-taneous SS mice model, a total of 30 NOD mice were divided into 3 groups randomly: recombinant adenovirus (rADV-AdipoQ) group, normal saline control and simple adenoviruses (control group). The submandibular gland morphology, histopathological grading and the level of serum tumor necrosis factor-α (TNF-α), AdipoQ was compared between the three groups. The expression of AdipoQ on mice submandibular gland was assessed by means of semi-quantitative reverse transcriptase polymerase chain reaction. Results The submandibular glands of the mice of the control group were destructed, with focal lymphocytic infiltration and acinus atrophy. Compared with control model groups, the serum TNF-α and salivary gland AdipoQ expression was significantly down-regulated in the rADV-AdipoQ group [(248 ±30) vs (162 ±73) ng/ml] (P<0.05). Conclusion AdipoQ gene transfected SS mice can significantly improve the morphological features of tissues and decrease the concentration of TNF-αin serum, in addition, it can effectively inhibit inflammation, decrease the degree of protein and AdipoQgene expression. So the AdipoQ may be the protective gene in SS mice.