ObjectiveTo further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. MethodsFive cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. ConclusionRecurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.

Objective To study the correlation between clinicopathological characteristics and lymph node metastasis and prognosis of rectal neuroendocrine tumors (NET),and try to explore the choice of surgical approach.Methods The clinicopathological data of 48 postoperative rectal NET patients were collected.The univariate and multivariate analysis of the correlation between clinicopathologic characteristics and lymph node metastasis were performed by chi-square test and Logistic regression.The prognosis single factor survival analysis was analyzed by Kaplan-Meier method.The survival rates were compared by Log-rank test.The prognosis multivariate survival analysis was performed by the use of Cox model.Results The results of univariate analysis indicated that clinicopathological characteristics related with lymph node metastasis were tumor distance from anal verge less than six cm,tumor diameter over two cm,muscularis invasion,distant metastasis and histological grade 3.The results of multivariate analysis showed that histological grade 3 was an independent risk factor of lymph node metastasis (OR=9.333,95 ％CI:1.054 to 82.635,P=0.045).The results of univariate survival analysis suggested that factors correlated with poor prognosis were tumor distance from anal verge less than six cm,tumor diameter over two cm,muscularis invasion,lymph node metastasis,distant metastasis and histological grade 3.The results of multivariate survival analysis indicated that tumor distance from anal verge less than six cm (HR=0.215,95 ％CI:0.047 to 0.980,P=0.047),distant metastasis (HR=8.788,95％CI:2.612 to 29.571,P＜0.01) and histological grade3 (HR=5.510,95％CI:1.692-17.944,P=0.005) were independent factors associated with poor prognosis.Conclusions For histological grade 1 and 2 rectal NET patients without distant metastasis,radical surgery is recommended when tumor diameter over two cm or muscularis invasion exists.For histological grade 3 rectal NET patients without distant metastasis,radical surgery is recommended.

Objective To investigate the differentiation of neuroendocrine component (NEC) in colorectal adenocarcinoma in relation to its significance by comparing the outcome between patients with or without NEC.Methods The paraffin sections from patients with pathologically confirmed colorectal adenocarcinoma were retrospectively collected and screened for those with NEC by morphological examination and immunohistochemistry with neuroendocrine markers.Control patients (n=54) without NEC were selected from colorectal cancer database and 2: 1 matched on the basis of clinical features with NEC positive patients (n=27).Relative analysis was performed between two groups.Results With a median follow-up of 72 months,the 5-year disease free survival was 58.0% (16/27) in NEC positive group and 79.1% (43/54) in control group (P=0.036).Similarly,the 5-year cancer-specific overall survival was significantly lower in NEC positive group than in control group (58.3% versus 81.1%,P=0.037).Cox regression showed that the 5-year cumulative risks of disease recurrence and cancer-caused death in NEC positive patients were 2.38 and 2.41 times higher than those in control patients,respectively.Conclusions NEC appears to bear a poor prognosis in patients with colorectal adenocarcinoma.

Objective:To review the treatment of life threatening head and neck space infection.Methods:Retrospective was con-ducted to analyse the patient characterization,signs and symptoms at the time of presentation,presence of concomitant systemic co-morbidities,treatment,prognosis and bacteriology.Results:32 patients from 2007.01 to 2013.01(mean age was 57.71 years)were included.The cases were increased year by year.1 1 (34.37%)patients had breathing difficulty requiring tracheostomy and 19 (59.3%)had concomitant systemic comorbidities.23 cases recovered completely,8 with severe mediastinal infection were trans-ferred to the department of thoracic surgery or the department of respiratory medicine.1 patient died.Pus submission rate was 96.87%,the bacteria detection rate was 37.50%.Pus culture showed mixed infection dominated by streptococci.Staphylococcus au-reus,pseudomonas aeruginosa,enterococcus faecalis,prevotella and neisseria were detected.Conclusion:Annually incidence of life threatening head and neck space infection is increasing.Treatment of the infection should include:①Ensuring the airway patency;②Surgical incision and drainage as early as possible;③ Hypoglycemic control,maintenance of hemodynamic,nutritional support,and multi-disciplinary approach are the key points in the management of such infections;④ Systemic antibiotics.

Objective:To compare the effects of low-carbohydrate diet (LCD) and low-fat diet (LFD) in the lifestyle intervention of non-alcoholic fatty liver disease (NAFLD) through a meta-analysis of randomized controlled trials.Methods:PubMed, Embase, Web of Science, Cochrane, CNKI and Wanfang were searched for relevant studies and study references and conference proceedings were manually searched. Two authors independently screened the items retrieved, extracted the data and assessed the quality of included studies. Meta-analysis was performed using R4.4.1 and RevMan5.4.1. Data were pooled using random-effects models and potential sources of heterogeneity were investigated using stratified meta-analysis. Funnel plots and Peters' test were used to assess publication bias.Results:Nine studies with a total of 510 participants met our inclusion criteria. Meta-analysis results showed that LCD and LFD interventions had similar effects on the reduction of intrahepatic lipid content in NAFLD patients ( SMD: -0.31,95% CI: 0.97 to 0.35, P = 0.36). There were no significant differences in changes of alanine aminotransferase ( SMD: -0.25, 95%CI: 0.91 to 0.41, P = 0.45) and aspartate aminotransferase ( SMD: -0.45, 95%CI: 1.63 to 0.72, P = 0.45) levels, either. Subgroup analyses implied that the duration of different interventions might be the cause of heterogeneity across studies. No significant publication bias was showed in the meta-analysis. Conclusion:Current evidence from randomized controlled studies does not support the superiority of LCD over LFD in the treatment of NAFLD.

Objective:To systematically evaluate the effect of hepatitis B virus (HBV) infection on the risk of adverse pregnancy outcomes.Methods:We searched PubMed, Embase, Web of Science, and Cochrane databases. Two researchers independently screened the literature, extracted data, and evaluated the quality. Meta-analysis and cumulative meta-analysis were performed using R4.4.1 software. Fixed/random effects models were used to analyze heterogeneous and non-heterogeneous results. Heterogeneous modifiers were identified by subgroup analysis. Funnel plots and Peters' test were used to analyze potential publication bias.Results:A total of 48 studies involving 92 836 HBsAg-positive pregnant women and 7 123 292 HBsAg-negative pregnant women were included. In terms of adverse pregnancy outcomes, HBV infection was significantly correlated with the occurrence of gestational diabetes mellitus [odds ratio ( OR)=1.34, 95% confidence interval ( CI): 1.17-1.53] and intrahepatic cholestasis ( OR=2.48, 95% CI: 1.88-3.29), with statistically significant differences. In terms of adverse neonatal outcomes, HBV infection was significantly correlated with the occurrence of neonatal asphyxia ( OR=1.49, 95% CI: 1.20-1.86) and preterm birth ( OR=1.22, 95% CI: 1.12-1.33), with statistically significant differences. In addition, the cumulative meta-analysis demonstrated that the risk of gestational diabetes mellitus and preterm birth both tended to be stable in pregnant women with HBV infection following 2009 and 2010, respectively. The supplementary questions answered for repeated studies had limited significance. Conclusion:Intrahepatic cholestasis, gestational diabetes mellitus, neonatal asphyxia, and preterm birth occurrence risk can be raised with HBV infection in pregnant women.

Objective:To investigate the clinicopathological features, immunohistochemical characteristics, differential diagnosis and prognosis of gastric SWI/SNF-complex deficient undifferentiated/rhabdoid carcinomas.Methods:Two cases of gastric SWI/SNF-complex deficient undifferentiated/rhabdoid carcinoma were collected at Fudan University Shanghai Cancer Center, Shanghai, China from 2017 to 2018. The clinicopathological characteristics were analyzed. Hematoxylin and eosin, and immunohistochemical stains were performed, and the relevant literatures were reviewed.Results:The two patients were both male, aged 60 and 74 years, respectively. Their symptoms were both abdominal pain. The tumor arose in the esophagogastric junction in case 1, and the cardia to the fundus and the posterior wall of the upper part of gastric body in case 2. Both tumors were present as an ulcerative mass. The patients died of tumor 11 months and 8 months after surgery, respectively. Histologically, the tumor cells arranged in sheets, nests, cords or trabecular patterns, and pseudoavleolar structure. The tumor cells were epithelioid with uniform morphology, while the tumors showed scant stroma and massive necrosis. Variable rhabdoid cells and multinucleated giant cells were seen in both cases. SMARCA4 encoding protein BRG1 was undetectable in both tumors, while SMARCB1 encoding protein INI1 was detected. The tumor cells were diffusely positive for vimentin and negative for epithelial marker (CKpan), gastrointestinal stromal tumor markers (CD117 and DOG1), myogenic markers (desmin and myogenin), melanoma markers (S-100 protein, SOX10 and HMB45), and lymphohematopoietic markers (LCA and CD20).Conclusions:Gastric SWI/SNF-complex deficient undifferentiated/rhabdoid carcinoma is a rare and highly aggressive tumor with poor prognosis. The detection of subunits protein expression of SWI/SNF complex is important for diagnosis of the tumor.

The determination of kinship requires the probability calculation among a set of individuals in a population supposed in a certain relationship.To make the method of kinship estimation easier to understand and for reasonable application,a general algorithm for parentage testing is presented in this paper.Furthermore,we discussed the reasons for the controversy of disputed-parent cases and proposed suggestions for it.In conclusion,all kinds of parentage testing can be easily solved based on the genetic rule and general algorithm.

ObjectiveTo reveal the targets and molecular mechanisms of the action of Qiangxin Decoction （强心汤） for the treatment of chronic heart failure based on the combination of network pharmacology and molecular docking. MethodsThe active ingredients of Qiangxin Decoction were retrieved from TCMSP database， and the targets of chronic heart failure were screened by searching GeneCards， OMIM， TTD， PharmGkb， and DrugBank databases， and the intersections were taken to obtain the intersecting targets of Qiangxin Decoction for the treatment of chronic heart failure. STRING platform was used to construct the protein-protein interaction network （PPI）， Cytoscape 3.8.0 software was used to calculate the network topology to screen the core targets， and R 4.2.3 was used to construct the “active ingredient-target” network by analyzing the GO enrichment analysis and KEGG pathway enrichment analysis. AutoDock 1.5.7 was used for molecular docking to predict the binding performance of active ingredients and core targets. ResultsSeventy-five intersecting targets were identified for the treatment of chronic heart failure with Qiangxin Decoction， among which the core targets were estrogen receptor 1 （ESR1， degree value=7）， nuclear receptor coactivator 1 （NCOA1， degree value=8）， glucocorticoid receptor （NR3C1， degree value=7）， and nuclear receptor coactivator 2 （NCOA2， degree value=7）. GO enrichment analysis showed that the top 3 items with the smallest P value in molecular function were G protein-coupled amine receptor activity， postsynaptic neurotransmitter receptor activity， and neurotransmitter receptor activity （P＜0.01）； the top 3 items with the smallest P value in biological process were adenylyl cyclase-activated adrenergic receptor signaling pathway， adrenergic receptor signaling pathway， and adenylyl cyclase-regulated G protein-coupled receptor signaling pathway （P＜0.01）； the top 3 items with the smallest P values in cellular composition were components of the postsynaptic membrane， synaptic membrane， and presynaptic membrane （P＜0.01）. KEGG enrichment analysis showed that the top 5 key signaling pathways were neuroactive ligand-receptor interactions， calcium signaling pathway， dopaminergic synapses， cocaine addiction， and cyclic guanosine monophosphate-protein kinase G （cGMP-PKG） signaling pathway. The molecular docking results showed that lignans and isoflavones had lower binding energies and more structural stability with the four core targets （ESR1， NCOA1， NR3C1， NCOA2）. ConclusionThe treatment of chronic heart failure by Qiangxin Decoction was associated with neuroactive ligand-receptor interactions， calcium signaling pathway， dopaminergic synapses， chemoattractant-receptor activation， cGMP-PKG signaling pathway， lipids and atherosclerosis， and cAMP signaling pathway， and lignans and isoflavones may be the core active compounds in its treatment of chronic heart failure.

Although the mTOR-4E-BP1 signaling pathway is implicated in aging and aging-related disorders, the role of 4E-BP1 in regulating human stem cell homeostasis remains largely unknown. Here, we report that the expression of 4E-BP1 decreases along with the senescence of human mesenchymal stem cells (hMSCs). Genetic inactivation of 4E-BP1 in hMSCs compromises mitochondrial respiration, increases mitochondrial reactive oxygen species (ROS) production, and accelerates cellular senescence. Mechanistically, the absence of 4E-BP1 destabilizes proteins in mitochondrial respiration complexes, especially several key subunits of complex III including UQCRC2. Ectopic expression of 4E-BP1 attenuates mitochondrial abnormalities and alleviates cellular senescence in 4E-BP1-deficient hMSCs as well as in physiologically aged hMSCs. These f indings together demonstrate that 4E-BP1 functions as a geroprotector to mitigate human stem cell senescence and maintain mitochondrial homeostasis, particularly for the mitochondrial respiration complex III, thus providing a new potential target to counteract human stem cell senescence.
Mesenchymal Stem Cells/physiology* ; Cellular Senescence ; Homeostasis ; Cell Cycle Proteins/metabolism* ; Adaptor Proteins, Signal Transducing/metabolism* ; Mitochondria/metabolism* ; Electron Transport Complex III/metabolism* ; Humans ; Cells, Cultured