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Objective To compare the diagnostic value of smear acid-fast staining,TB-DNA,X-pert MTB/RIF and culture of Mycobacterium tuberculosis.Methods Four methods were used to detect the perifocal pus of the patients with bone destruction in orthopaedics department within one year,and the results were analyzed statistically,the indexes included sensitivity,specificity,positive predictive value and negative predictive value.Results The sensitivity,specificity,positive predictive value,negative predictive value,and Youden index were 31.75%,100.00%,100.00%,53.74%and 0.32 respectively.TB-DNA had a sensitivity of 88.89%,a specificity of 98.00%,a positive predictive value of 98.25%,a negative predictive value of 87.50%,and a Youden Index of 0.87.Xpert MTB/Rif had a sensitivity of 95.23%,a specificity of 68.00%,and a positive predictive value of 78.95%,the negative predictive value was 91.90%,the Youden index was 0.63.The sensitivity,the specificity,the positive predictive value,the negative predictive value and the Youden index were 41.27%,100.00%,100.00%,57.47%and 0.41 respectively,(χ2 = 77.354,P<0.005).Conclusion Among the four methods,TB-DNA has a good Sensitivity and specificity,Xpert mtbrif has a good sensitivity,TB-DNA and Xpert mtbrif ha a good authenticity,and both positive and negative predictive values are high,it has good value in the diagnosis of bone tuberculosis.
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Objective To investigate the mechanism of iron death induced by TRPC6/NF-κB in glomerular podiocytes mediated by high homocysteine(Hcy).Methods Mouse glomerulopocytes were cultured in vitro and divided into Control group(0 μmol/L Hcy)and Hcy group(80 μmol/L Hcy).After 48h of intervention,Western blot was used to detect the expression levels of iron death related proteins GPX4 and SLC7A11 and TRPC6 and NF-κ B.Real-time quantitative fluorescence PCR(qRT-PCR)and immunofluorescence were used to detect the expression of TRPC6.The level of podocyte apoptosis was detected by flow cytometry.Malondialdehyde(MDA)assay kit was used to determine intracellular MDA levels.After transfection of TRPC6 interference fragment and TRPC6 negative control(NC),qRT-PCR was divided into Control,si-NC and si-TRPC6(Si-TRPC6-1,Si-TRPC6-2,Si-TRPC6-3).Western Blot was divided into Control,Hcy,si-NC+Hcy,si-TRPC6+Hcy.The expression of TRPC6 mRNA was detected by qRT-PCR.The expression levels of GPX4,SLC7A11,NF-κB and TRPC6 were detected by Western Blot.The level of podocyte apoptosis after interference was detected by flow cytometry.Results(1)Compared with Control group,the expression levels of iron death related proteins GPX4 and SLC7A11 in Hcy group were decreased,and the apoptosis rate was increased(P<0.05).(2)Compared with Control group,TRPC6 protein,mRNA levels and immunofluorescence expression were increased in Hcy group.The level of MDA and the expression of NF-κB signaling pathway protein increased in Hcy group,and the comparison between the two groups had statistical significance(P<0.05).(3)Compared with the si-NC group,the mRNA expression level of TRPC6 in si-TRPC6(Si-TRPC6-1,Si-TRPC6-2,Si-TRPC6-3)group was decreased,and the interference effect of Si-TRPC6-3 was the best(P<0.05).After transfecting TRPC6 NC and TRPC6 interference fragment and administering Hcy,there was no difference in GPX4,SLC7A11,NF-κB and TRPC6 expression in si-NC+Hcy group compared with Hcy group.Compared with the si-NC+Hcy group,the si-TRPC6+Hcy group had higher expression of iron death related proteins,GPX4 and SLC7A11,lower expression of NF-κB and TRPC6,and decreased apoptosis rate(P<0.05).Conclusion This study confirmed that TRPC6/NF-κB can regulate iron death of renal podocytes under the induc-tion of Hcy,which is one of the mechanisms leading to kidney injury.
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Objective:To investigate the prediction of the tumor proliferation antigen(Ki-67) expression status in breast cancer patients based on ultrasound radiomics combined with clinicopathologic features.Methods:Breast cancer patients who underwent 2D ultrasound and Ki-67 examination from January 2018 to February 2022 in Changzhou Second People′s Hospital, Nanjing Medical University were retrospectively analyzed. Among them, 427 patients from Chengzhong campus were randomly divided into training and validation sets in the ratio of 8∶2, and 229 patients from Yanghu campus were used as an independent external test set. Radiomics features were extracted from the region of interest of 2D ultrasound images, and the Mann-Whitney U test, recursive feature elimination, and minimum absolute shrinkage and selection operators were used to perform feature dimensionality reduction and to establish a radiomics score(Rad-score). Subsequently, single/multifactor logistic regression regression analyses were used to construct a joint prediction model based on Rad-score and clinicopathological features. Model performance and utility were assessed using the subject operating characteristic area under the curve (AUC), calibration curve, and decision curve analyses. Results:The AUCs of the joint model for predicting Ki-67 expression status in breast cancer in the training, validation, and test sets were 0.858, 0.797, and 0.802, respectively, which were superior to those of the radiomics (0.772, 0.731, and 0.713) and clinical models (0.738, 0.750, and 0.707). Calibration curve and decision curve analyses indicated that the joint model had good calibration and clinical value.Conclusions:A joint model based on ultrasound radiomics and clinicopathological features can effectively predict the Ki-67 expression status of breast cancer, which is expected to become a non-invasive tool for Ki-67 detection and provide clinicians with an important auxiliary diagnostic and therapeutic decision-making basis.
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Objective:To summarize the best evidence of psychological intervention for patients receiving assisted reproductive technology (ART).Methods:All evidence on psychological interventions for patients receiving ART was searched in UpToDate, BMJ Best Practice, Joanna Briggs Institute, GIN, NICE, NGC, SIGN, CMA Infobase, Cochrane Library, PubMed, Web of Science, CINHAL, Embase, psycINFO, China Guideline Clearinghouse (CGC), guides in medlive.cn, CNKI, Wanfang Data, SinoMed, and VIP databases. The evidence retrieved included clinical practice, recommendations, guidelines, expert opinions, consensus, systematic reviews and meta-analyses, and the search time limit was from the establishment of the database to January 2023. Two researchers conducted evidence extraction, aggregation, and quality evaluation.Results:A total of 797 references were obtained at the initial retrieval, and 27 papers were finally included, including 1 clinical best practice, 2 guidelines, 1 recommended practice, and 23 systematic evaluations. The evidence summary summarized 16 best evidences, including four aspects of psychological assessment, psychological intervention, drug treatment and personnel training for ART patients, of which 50% were class 1 evidences and 87.5% of the evidences were recommended with strength A.Conclusions:Enhanced personnel training in assisted reproduction facilities and early identification of the psychological state of infertile patients by medical staff and targeted psychological intervention or medication are the key to improving the psychological status of ART patients.
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Objective To analyze the relationship between acute exacerbation of chronic obstructive disease (COPD) hospitalization and climate change in Enshi, Hubei province, and to provide theoretical basis for the prevention and treatment of COPD in Enshi . Methods Patients with acute COPD in Enshi district of Hubei Province from January 2019 to December 2019 were selected, and the clinical data of all patients on admission, including symptoms, signs, onset month, lung function indicators and serum inflammatory factors, were analyzed . FEV1% predictive value, FEV1/FVC and other lung function indexes were determined by pulmonary function detector. CRP and WBC levels were determined by biochemical detector. At the same time, meteorological factors data in Enshi , were collected , including monthly mean temperature, monthly mean humidity, monthly mean wind speed , monthly mean sunshine in Hubei province , etc. Spearman linear correlation was used to analyze the meteorological factors affecting the number of hospitalized AECOPD patients . Results The age of patients with ACUTE exacerbation of COPD was mainly 70-79 years old (34.63%) in Enshi area of Hubei Province , followed by 60-69 years old (22.49%) , and the lowest was 40-49 years old (13.75%). The incidence time mainly concentrated to may for the peak in March , November to January for the secondary peak , June to October for a relatively small number of cases. The main symptoms of COPD acute recombination are frequent cough , shortness of breath , increased sputum volume, etc., while the main signs are emphysema , wheezing , dry and wet rales . The predictive value of FEV1 and FEV1/FVC value of acute recombinant COPD were significantly lower than those of non-acute recombinant COPD (P1=-0.519 , r2=-0.428, P1=0.124 , r2=0.176, P>0.055) . Conclusion The number of patients with ACUTE exacerbation of COPD shows obvious seasonality, and the occurrence of AECOPD should be actively prevented in months with great changes in temperature and humidity.
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Objective:To investigate the safety and feasibility of the CHESS endoscpic ruler (CHESS ruler), and the consistency between the measured values and the interpretation values by endoscopic physician experience.Methods:From January 2021 to January 2022, a total of 105 liver cirrhosis patients with portal hypertension were prospectively enrolled from General Hospital, Xixia Branch Hospital, Ningnan Hospital of People′s Hospital of Ningxia Hui Autonomous Region (29 cases), and the First People′s Hospital of Yinchuan (25 cases), General Hospital of Ningxia Medical University (18 cases), Wuzhong People′s Hospital (10 cases), the Fifth People′s Hospital of Ningxia Hui Autonomous Region (10 cases), Shizuishan Second People′s Hospital (6 cases), Yinchuan Second People′s Hospital (5 cases), and Zhongwei People′s Hospital (2 cases) 8 hospitals. The clinical characteristics of all the patients, including gender, age, nationality, etiolog of liver cirrhosis, and Child-Pugh classification of liver function were recorded. A big gastroesophageal varices was defined as diameter of varices ≥5 mm. Endoscopist (associated chief physician) performed gastroscopy according to the routine gastroscopy procedures, and the diameter of the biggest esophageal varices was measured by experience and images were collected, and then objective measurement was with the CHESS ruler and images were collected. The diameter of esophageal varices of 10 randomly selected patients (random number table method) was determined by 6 endoscopists (attending physician or associated chief physician) with experience or measured by CHESS ruler. Kappa test was used to test the consistency in the diameter of esophageal varices between measured values by CHESS ruler and the interpretation values by endoscopic physician experience.Results:Among 105 liver cirrhosis patients with portal hypertension, male 65 cases and female 40 cases, aged (54.8±12.2) years old, Han nationality 82 cases, Hui nationality 21 cases and Mongolian nationality 2 cases. The etiology of liver cirrhosis included chronic hepatitis B (79 cases), alcoholic liver disease (7 cases), autoimmune hepatitis (7 cases), chronic hepatitis C (2 cases), and other etiology (10 cases). Liver function of 32 cases was Child-Pugh A, Child-Pugh B 57 cases, and Child-Pugh C 16 cases. All 105 liver cirrhosis patients with cirrhotic portal hypertension were successfully measured the diameter of gastroesophageal varices by CHESS ruler, and the success rate of application of CHESS ruler was 100.0% (105/105). The procedure time from the CHESS ruler into the body to the exit of the body after measurement was (3.50±2.55) min. No complications happened in all the patients during measurement. Among 105 liver cirrhosis patients with cirrhotic portal hypertension, 96 cases (91.4%) were recognized as big gastroesophageal varices by the endoscopists. Totally 93 cases (88.6%) were considered as big gastroesophageal varices by CHESS ruler. Eight cases were recognized as big gastroesophageal varices by the endoscopist, however not by the CHESS ruler; 5 cases were recognized as big gastroesophageal varices by the CHESS ruler, but not by the endoscopists; 4 cases were not recognized as big gastroesophageal varices both by the endoscopists and CHESS ruler; 88 cases were recognized as big gastroesophageal varices both by the endoscopists and CHESS ruler. The missed diagnostic rate of big gastroesophageal varices by the endoscopists experience was 5.4% (5/93), and the Kappa value of consistency coefficient between the measurement by the CHESS ruler and the interpretation by endoscopists experience was 0.31 (95% confidence interval 0.03 to 0.60). The overall Kappa value of consistency coefficient by 6 endoscopists measured by CHESS ruler in big gastroesophageal varices diagnosis was 0.77 (95% confidence interval 0.61 to 0.93).Conclusion:As an objective measurement tool, CHESS ruler can make up for the deficiency of subjective judgment by endoscopists, accurately measure the diameter of gastroesophageal varices, and is highly feasible and safe.
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Objective:To investigate the diagnostic value of metagenomic next-generation sequencing (mNGS) in AIDS patients complicated with Pneumocystis jirovecii ( P. jirovecii) infection. Methods:This is a retrospective study. From January 2019 to June 2021, the respiratory tract and other body fluid samples of 236 cases of AIDS co-infected patients diagnosed in the AIDS Department of Changsha First Hospital were collected, along with corresponding medical histories. Traditional etiological hexamine silver staining and serum 1,3-β-D glucan (BDG) were performed simultaneously with mNGS detection, and Fisher′s exact test was used to analyze the results and compare the diagnostic performances of mNGS with those of hexamine silver staining and serum G test.Results:A total of 236 cases of AIDS patients with pulmonary infection were collected and tested. Seventy-seven cases were clinically diagnosed with Pneumocystis jiroveci pneumonia and 159 cases with non- Pneumocystis jiroveci pneumonia. Among the 236 AIDS patients with pulmonary infection, mNGS detected 77 [32.63%(77/236)] positive cases of Pneumocystis jiroveci, while hexamine silver staining detected 10[4.24%(10/236)] and serum BDG detected 146 [61.86% (146/236). Based on these clinical diagnostic results, the sensitivity of mNGS detection was 100% (77/77) for the 77 patients with Pneumocystis pneumoniae, significantly higher than that of silver hexamine staining [12.99% (10/77), P=0.046] and serum BDG [58.44% (45/77), P=0.038]. The mNGS showed good specificity, which was the same as that of hexamine silver staining [100% (159/159)] and significantly higher than that of serum BDG [36.48% (58/159), P=0.026]. With therapeutic clinical diagnosis as the reference method, the accuracy of mNGS detection was 100% (236/236). Conclusions:This study evaluated the diagnostic value of mNGS detection in AIDS patients with Pneumocystis jirovecii infection. The results showed that the sensitivity and specificity of mNGS detection were high, and it had exceptional clinical application value in the pathogenic detection of infectious diseases.
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Lung cancer is the most threatening tumor disease to human health. Early detection is crucial to improve the survival rate and recovery rate of lung cancer patients. Existing methods use the two-dimensional multi-view framework to learn lung nodules features and simply integrate multi-view features to achieve the classification of benign and malignant lung nodules. However, these methods suffer from the problems of not capturing the spatial features effectively and ignoring the variability of multi-views. Therefore, this paper proposes a three-dimensional (3D) multi-view convolutional neural network (MVCNN) framework. To further solve the problem of different views in the multi-view model, a 3D multi-view squeeze-and-excitation convolution neural network (MVSECNN) model is constructed by introducing the squeeze-and-excitation (SE) module in the feature fusion stage. Finally, statistical methods are used to analyze model predictions and doctor annotations. In the independent test set, the classification accuracy and sensitivity of the model were 96.04% and 98.59% respectively, which were higher than other state-of-the-art methods. The consistency score between the predictions of the model and the pathological diagnosis results was 0.948, which is significantly higher than that between the doctor annotations and the pathological diagnosis results. The methods presented in this paper can effectively learn the spatial heterogeneity of lung nodules and solve the problem of multi-view differences. At the same time, the classification of benign and malignant lung nodules can be achieved, which is of great significance for assisting doctors in clinical diagnosis.
الموضوعات
Humans , Lung/pathology , Lung Neoplasms/pathology , Neural Networks, Computer , Tomography, X-Ray Computed/methodsالملخص
Objective:To construct the prognostic prediction model and scoring tool by using severe trauma patients’ physiological indicators on admission, and to verify the clinical application effect and provide a reference for the early evaluation of severe trauma patients.Methods:This study was a retrospective study which adopted cluster sampling. Patients who met the inclusion and exclusion criteria in the emergency department of the First Affiliated Hospital of Soochow University from September 2019 to November 2020 were included. Patients were randomly assigned into the modeling group and the validation group in a ratio of 7:3 based on their outcome in the emergency department. Logistic regression analysis was performed to construct a prediction model, which was simplified as a scoring tool. The model was verified by using validation group and two months’ prospective validation. The efficiency of the simplified scoring tool was compared with that of the revised trauma score (RTS) and the injury severity score (ISS).Results:Totally 863 patients were included in this study, including 604 patients in the modeling group and 259 patients in the validation group. The model included systolic blood, SpO 2 and AVPU score. The AUC for predicting the death of severe trauma patients was 0.938. The AUC of the prediction model was 0.933, the best cut-off point was 5, the sensitivity was 86.7%, the specificity was 94.2%; the AUC of the validation was 0.885, the sensitivity was 83.3%, the specificity was 93.7%; and the AUC of prospective validation was 0.919, the sensitivity was 100%, and the specificity was 76.7%. The AUC of the RTS and ISS were 0.800 and 0.833, respectively. The AUC of RTS was lower than that of the simplified scoring tool constructed in this research. Conclusions:The prediction model and simplified scoring tool are better than RTS in predicting the outcome of emergency severe trauma patients, which are convenient for emergency medical staff to evaluate the severity of trauma patients.
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Objective:To summarize and analyze the clinical characteristics of different treatment in children with esophageal foreign bodies.Methods:This study collected 246 children with esophageal foreign bodies in our hospital from January 2016 to January 2020, which was divided into endoscopic group and operative group.The general and clinical data of children treated with different treatment were collected and statistical analyzed.Results:There were 222 children in endoscopic group and 24 children in operative group, respectively.The rate of surgery was 9.75%.There were no significant differences in gender and location of esophageal foreign bodies.However, the average age of operative group was(2.92±2.67) years, which significantly younger than that in endoscopic group(4.12±3.37)years( P=0.049). The residence time in operative group(median 29.10 h)was remarkable longer than that in operative group(median 11.80 h)( P<0.001). The proportion of sharpness(50.00%) and corrosive(45.83%) foreign bodies in operative group were more than those in endoscopic group[16.22% and 8.11%( P<0.001)]. Moreover, the occurrence rate of major complication in operative group was 83.33%, which was dramatically higher than that in endoscopic group(0.90%)( P<0.001). Conclusion:The younger and longer residence time of esophageal foreign bodies in children contribute to the rate of operative treatment.Additionally, the sharpness and corrosive foreign bodies increase the risk of surgery and serious complications.
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Objective:To explore the effects of clinical characteristics and dosimetric factors on the survival and prognosis of patients with locally advanced thoracic esophageal squamous cell carcinoma after concurrent chemoradiotherapy (CCRT).Methods:A total of 158 patients with locally advanced thoracic esophageal squamous cell carcinoma undergoing CCRT in Shandong Cancer Hospital, Anyang Cancer Hospital of Henan Province, Tengzhou Central People′s Hospital of Shandong Province and the First Affiliated Hospital of China Medical University from August 2015 to October 2018 were selected as the research subjects. These patients were divided into standard-dose group (50.0-50.4 Gy, n=59) and high-dose group (>50.4 Gy, n=99) according to the radiotherapy dose. The overall survival (OS) and progression-free survival (PFS) of the two groups after treatment were compared. Kaplan-Meier method was used to calculate survival rate and survival comparison was performed by log-rank test. Cox proportional hazard regression model was used to analyze the adverse prognostic factors. The receiver operating characteristic (ROC) curve was used to analyze the predictive value of lung V 30 for patient prognosis. Results:In 158 patients with locally advanced esophageal squamous cell carcinoma, 19 cases (12.03%) had complete remission after CCRT, 103 cases (65.19%) had partial remission, 27 cases (17.09%) had stable disease, 9 cases (5.70%) had progression disease, and the total effective rate was 77.22%. The median OS of 158 patients was 41 months (95% CI: 25-57 months), and the 1- and 3-year OS rates were 76% and 51%, respectively. The median PFS was 24 months (95% CI: 13-35 months), and the 1- and 3-year PFS rates were 60% and 39%, respectively. The 1- and 3-year OS rates in the standard-dose group were 74% and 56%, and those in the high-dose group were 77% and 49%, with no statistically significant difference ( χ2=0.300, P=0.584). The 1- and 3-year PFS rates in the standard-dose group were 62% and 37%, and those in the high-dose group were 59% and 40%, with no statistically significant difference ( χ2<0.001, P=0.990). Univariate analysis showed that the length of the lesion, N stage, clinical stage, short-term efficacy of CCRT, planning target volume (PTV) D max, gross tumor volume (GTV) D mean, V 5, V 10, V 20, V 30, D mean of the left, right and bilateral lung were all the prognostic factors for OS and PFS (all P<0.05). Multivariate analysis showed that the length of the lesion ( HR=2.226, 95% CI: 1.244-3.985, P=0.007), N stage ( HR=2.819, 95% CI: 1.137-6.991, P=0.025), clinical stage ( HR=1.897, 95% CI: 1.079-3.334, P=0.026), short-term efficacy of CCRT ( HR=1.805, 95% CI: 1.250-2.606, P=0.002), left lung V 10 ( HR=0.811, 95% CI: 0.668-0.986, P=0.035), left lung V 30 ( HR=0.617, 95% CI: 0.408-0.933, P=0.022), right lung V 20 ( HR=2.067, 95% CI: 1.010-4.231, P=0.047), bilateral lung V 10 ( HR=1.299, 95% CI: 1.016-1.662, P=0.037) and bilateral lung V 30 ( HR=2.368, 95% CI: 1.142-4.910, P=0.021) were independent prognostic factors affecting OS. N stage ( HR=2.433, 95% CI: 1.201-4.931, P=0.014), short-term efficacy of CCRT ( HR=2.067, 95% CI: 1.391-3.071, P<0.001) and bilateral lung V 30 ( HR=0.113, 95% CI: 0.018-0.719, P=0.021) were independent prognostic factors affecting PFS. The ROC curve for predicting OS and PFS showed that the optimal cut-off value of bilateral lung V 30 was 9.5%. Conclusion:Compared with the standard-dose group, increasing the dose of radiotherapy fails to improve the long-term survival of patients with locally advanced thoracic squamous cell carcinoma. Lesion length, N stage, clinical stage, short-term efficacy of CCRT, left lung V 10 and V 30, right lung V 20 , bilateral lung V 10 and V 30 are independent prognostic factors for OS in patients with locally advanced thoracic squamous cell carcinoma. N stage, short-term efficacy of CCRT and bilateral lung V 30 are independent prognostic factors for PFS. When bilateral lung V 30≤9.5%, the patients′ OS and PFS will benefit from the treatment.
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Objective:To investigate the clinical characteristics, endoscopic features and management of acquired tracheoesophageal fistula (TEF) by esophageal foreign bodies in children.Methods:The clinical data and follow-up data of 21 children with acquired TEF who were treated in Children′s Hospital Affiliated to Zhengzhou University from January 2008 to January 2019 were retrospectively analyzed.Results:A total of 21 cases with esophageal foreign bodies were button batteries, irregular iron sheets, game coins, jujube seed, animal bone sheets, and fish thorn.The statistical results suggested that the cases of button batteries were 7 cases (33.33%), 4 cases of jujube pit (19.05%), 3 cases of irregular iron (14.29%) and 3 cases of animal bone (14.29%), 2 cases of game coin (9.52%), 2 cases of fish thorn (9.52%). All foreign bodies were removed by endoscopy.Esophageal perforation with TEF was discovered in 17 cases (80.95%) during the operation.Esophageal perforation with TEF was found in 4 cases (19.05%) within 2 week after the operation, and no death occurred.A total of 13 case fistula size ≤5 mm (61.90%), 4 case fistula size>5 mm and ≤10 mm (19.05%) and 4 case fistula size >10 mm (19.05%). The treatment methods included gastrointestinal decompression and nasojejunal nutrition tube support in 10 cases (47.61%), gastrostomy and jejunostomy in 4 cases (19.05%), surgical repair in 4 cases (19.05%) and endoscopic titanium clip suture in 3 cases (14.29%). Five cases (23.81%) were healed in 3 months, 6 cases (28.57%) in 5 months, 4 cases (19.05%) in 8 months, and 2 cases (9.52%) in 12 months.Conclusions:Acquired TEF in children are mostly caused by special foreign bodies embedded in the esophagus, and endoscopic management is effective for fistulas with less trauma.Appropriate operation and intervention should be taken as early as possible.
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OBJECTIVE@#To report on the clinical features and result of genetic testing for a child featuring immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome.@*METHODS@#Clinical records, genetic testing, laboratory investigation and treatment of the child were summarized in addition with a comprehensive review of the literature.@*RESULTS@#The 3-year-old boy was administered due to intractable diarrhea, recurrent infections, liver dysfunction and failure to thrive, though no diabetes or skin disorder was observed. Laboratory testing showed elevated liver enzymes and total IgE, decreased albumin and electrolyte imbalance. Gastrointestinal endoscopy revealed erosion and granules in the duodenum, and edema in the terminal ileum and colon. Biopsies showed villous atrophy in the duodenum and terminal ileum. Genetic testing revealed that the patient has carried a missense c.1087A>G (p.I363V) variant in the exon 10 of the FOXP3 gene. He was treated with enteral and parenteral nutrition, anti infection and Sirolimus, and was waiting for hemopoietic stem cell transplantation.@*CONCLUSION@#Although IPEX syndrome usually occur during infancy, it should not be ruled out solely based on the age, and its presentation can be variable. For male children with refractory diarrhea, autoimmune disorder and growth retardation, the diagnosis should be suspected and confirmed by genetic testing.
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Child, Preschool , Humans , Male , Diabetes Mellitus, Type 1/genetics , Diarrhea/genetics , Forkhead Transcription Factors/genetics , Genetic Diseases, X-Linked/genetics , Genetic Testing , Immune System Diseases/genetics , Mutation , Polyendocrinopathies, Autoimmune/geneticsالملخص
Objective:To analyze the clinical characteristics, diagnosis, treatment and prognosis of three children with hemophagocytic syndrome (HPS) and provide diagnosis and treatment clues for reasonable treatment and prevention of serious complications and reduction of misdiagnosis and mistreatment.Methods:The clinical data of three children with HPS who received treatment in Taiyuan Maternal and Child Health Care Hospital from March 2018 to March 2020 were retrospectively analyzed. Related literature was retrieved. The clinical data of the three children were summarized to analyze the outcomes.Results:Fever (≥ 39 ℃) was the first symptom in all three cases. In the end, red blood cell line, white blood cell line, and platelet line were reduced to different degrees in all three cases. Hepatosplenomegaly was found in two cases. Transaminase highly increased in two cases, and slightly increased in one case. Ferritin greatly increased, fibrinogen decreased, and hypertriglyceridemia did not occur in all three cases. Two cases had skin rash during fever. Skin rash appeared late in one of them.All three cases had different degrees of lymphadenopathy. Bone puncture examination showed reticulocyte phagocytosis in bone marrow in one case and leishmania in bone marrow smear in another case. These two cases were cured and discharged. One case died of multiple organ failure due to rapid disease progression, and adrenal masses were found at autopsy.Conclusion:HPS has diverse clinical manifestations, complex etiology and different clinical prognoses. HPS should be considered in case of unexplained fever with hemocytopenia. The medical history and living history should be inquired in detail and relevant auxiliary examinations should be improved as soon as possible.
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Objective:To investigate the effect of polyethylene glycol combined with simethicone on boston bowel preparation scale(BBPS) score and tolerance in patients with colonoscopy.Methods:From January 2018 to January 2019, 220 patients who underwent colonoscopy in Beijing Shijitan Hospital Affiliated to Capital Medical University were selected as the research objects.According to different bowel preparation schemes, they were divided into control group and observation group, 110 cases in each group.In the control group, 4 bags of compound polyethylene glycol electrolyte powder were added with cold boiled water to prepare 4 L solution for intestinal preparation.Starting 6 hours before endoscopy, 1 L solution was taken every hour, and the interval from the last time to the beginning of colonoscopy was≤4 h. On the basis of the control group, the observation group was given 30 mL simethicone emulsion 4 hours before endoscopy on the basis of the control group.The BBPS score, intracavitary liquid volume score, total score, tolerance and adverse reactions were compared between the two groups.Results:The whole colon score (7.16±0.61), left colon score (2.89±0.62), transverse colon score (2.78±0.64) and right colon score (1.58±0.49), intestinal cavity fluid score (1.47±0.48) and total score (8.84±0.87) of the observation group were higher than those of the control group ((5.13±0.76), (2.23±0.86), (2.15±0.76), (1.14±0.16), (0.91±0.55), (7.11±1.04)), and the differences were statistically significant ( t=21.854, 6.532, 6.652, 8.957, 8.054, 13.380, all P<0.05). The tolerance of the observation group (90.00%(99/110)) was higher than that of the control group (81.81%(90/110)), but the difference was not statistically significant( χ 2=3.043, P=0.081). The incidence of abdominal distension in the observation group (1.82%(2/110)) was lower than that in the control group (8.18%(9/110)), the difference was statistically significant(χ 2=4.690, P<0.05), and there was no significant difference in the incidence of electrolyte disorder, nausea and vomiting, hypoglycemia or hunger, palpitation and chest tightness between the two groups (all P>0.05). Conclusion:The application of polyethylene glycol combined with Spanish silicone oil in intestinal preparation can improve the intestinal cleanliness of patients, but does not increase the tolerance of patients compared with polyethylene glycol alone, but significantly reduces the incidence of abdominal distension.
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Objective:To evaluate the value of shear wave elastography (SWE) and salivary gland ultrasound scoring(SGUS) system in the diagnosis of salivary glands lesions in patients with Sj?gren′s syndrome(SS) and to compare the diagnostic efficiency of the two methods.Methods:From June 2019 to November 2020, Fifty-eight patients with suspected SS were selected from the Affiliated Changzhou No.2 People′s Hospital of Nanjing Medical University. According to the 2002 American-European Consensus Group classification standard, the enrolled patients were divided into two groups: SS group (47 cases) and non-SS group(11 cases). According to symptom duration, SS group was divided into the ≤5 years group (29 cases) and the >5 years group (18 cases). Meanwhile, 40 healthy volunteers were enrolled in this study as normal control group. The diagnostic value of salivary gland ultrasound scoring system and Young′s modulus in SS were analyzed.Results:The differences in Young′s modulus of parotid gland and submandibular gland between SS group and non-SS group (or control group) were statistically significant (all P<0.05). The ultrasound score of SS group was significantly higher than that of non-SS group and control group (all P<0.05). SGUS and Young′s modulus were not significantly different between different course groups (all P>0.05). The areas under ROC curve of the mean Young′s value in parotid and submandibular gland and the SGUS were 0.801, 0.829 and 0.676, respectively. The comparison of the area under the curve between the Young′s modulus of the parotid and submandibular glands and the ultrasound score was statistically significant (all P<0.05). Conclusions:SWE and Ultrasonography scoring system have certain value in the diagnosis of salivary gland lesions in SS, and can provide important reference information for clinical diagnosis from different perspectives. The diagnostic efficiency of SWE for salivary glands lesions in patients with SS is better than that of SGUS scoring system.
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Objective:To systematically evaluate the rate of unplanned readmissions for children at 30 days after cardiac surgery, the causes and risk factors for unplanned readmissions for children at 30 days after cardiac surgery.Methods:We searched PubMed, Embase, Web of Science, CBM, CNKI, Wanfang Data, and VIP database to collect studies from inception to August 15, 2020 of unplanned readmission of children after cardiac surgery.RevMan5.3 software was used for Meta-analysis.Results:A total of 13 studies, involving 81 633 patients were included.Meta-analysis results showed that the unplanned readmission rate of children 30 days after cardiac surgery was 11.5%(95% CI 9.9%-13%). Cardiac complications[24%(95% CI 10.7%-45.7%)], infections[18.7%(95% CI 13.7%-25.3%)], respiratory complications[18.0%(95% CI 10.7%-27.5%)], pleural effusion[11.5%(95% CI 8.3%-16.7%)], and gastrointestinal complications[12.3%(95% CI 8.3%-17.4%)] were the main reasons for unplanned 30-day readmission, with statistically significant differences( P<0.001). Genetic syndrome( OR=1.25, 95% CI 1.18-1.33, P<0.05) and postoperative hospital stay >14 days( OR=1.62, 95% CI 1.05-2.50, P<0.05) were risk factors for unplanned 30-day readmission. Conclusion:The incidence rate of unplanned readmission among the children 30 days after cardiac surgery is relatively high.Cardiac complications, infection, respiratory complications, pleural effusion, and gastrointestinal complications are the main reasons for 30-day unplanned readmission.The genetic syndrome and postoperative hospital stay >14 days in children 30 days after cardiac surgery are the risk factors for unplanned readmission.
الملخص
Objective:To analyze the clinical characteristics, endoscopic manifestations, complications and related risk factors of button battery ingestion in 58 children, thus providing the basis for clinical diagnosis and treatment.Methods:The medical data of 58 children with button battery ingestion were collected and researched at Children′s Hospital Affiliated to Zhengzhou University from September 2015 to September 2020.The demographic information, battery impaction location, duration, symptoms, mucosal injury level, battery size, treatment, complications and follow-up results were analyzed.Results:The average age of the patients with button battery ingestion was (25.7±15.4)months, including 40 boys(68.9%). The average retention time of the battery in digestive tract was 13.8(2, 96) h. Vomiting, salivation, dysphagia, cough and fever were the common chief complaints.There were 29(50.0%) cases of grade I mucosal injury, as well as 14(24.1%) cases, 10(17.2%) cases and 10(17.2%) cases for grade Ⅱ, grade Ⅲ and grade Ⅳ, respectively.Additionally, common complications included esophageal stenosis, esophageal perforation and esophageal-tracheal fistula.Logistic regression analysis showed that location(esophagus) and diameter(≥15 mm) of battery incarceration were important predictors of complications.Conclusion:The degree of mucosal damage is associated with the diameter and impaction location of battery.The button battery embedded in the esophagus is prone to complications, while the ones retained in the stomach were not vulnerable to serious complications.Endoscopy and other related examinations should be performed again in 1 to 3 weeks after the button removal to determine the outcome of complications and to intervene in time.
الملخص
The procurement, preservation and transportation of the donor organs directly affect the clinical prognosis of the recipients. The establishment of process optimization and quality control standards of organ procurement, preservation and transportation contributes to improving the quality and utilization rate of donor organs and reducing the medical risk. According to Guide to the Quality and Safety of Organs for Transplantation (6th edition) proposed by European Union, the 11th chapter of organ procurement, preservation and transportation was interpreted and summarized in this article.
الملخص
Objective:To investigate the risk factors of systolic dysfunction early complicated in patients with isolated traumatic brain injury (iTBI) and to evaluate the influence of complicated systolic dysfunction on the prognosis of iTBI patients.Methods:From January 2017 to October 2018, 123 patients with moderate or severe iTBI admitted to Trauma Centre in our hospital were included in the study, and patients with previous cardiovascular diseases were excluded. Left ventricular systolic function was assessed by transthoracic echocardiography within 24 h after admission. The patients were divided into normal systolic function group ( n=100) and systolic dysfunction group ( n=23) according to the results of echocardiography. Data were collected from all patients on admission, including GCS score, systolic blood pressure, heart rate, high-sensitivity cardiac troponin T (hs-cTnT), clinical treatment variables (use of sedative drugs, vasoactive drugs, etc.), craniotomy or not and clinical outcomes (survival or death) during hospitalization. Logistic regression analysis was used to analyze the related factors for iTBI patients complicated with systolic dysfunction, and receiver operating characteristic (ROC) curve was drawn to evaluate the predictive value of each index for iTBI patients complicated with cardiac insufficiency. Results:The systolic blood pressure (147.3±23.3) mmHg, the heart rate (96.1±26.3) beats/min and the hs-cTnT level (16.48±8.17) pg/mL in the systolic dysfunction group were higher than those in the normal systolic function group on admission (all P<0.05); and the GCS score in the systolic dysfunction group was lower than that in the normal systolic function group ( P<0.05). Logistic regression analysis showed that the heart rate ( OR=1.129, 95% CI: 1.001-1.516; P=0.038), the GCS score ( OR=0.640, 95% CI: 0.445-0.920; P=0.016) and the hs-cTnT level ( OR=1.054, 95% CI: 1.009-1.101; P=0.002) on admission were independent risk factors for iTBI patients complicated with systolic dysfunction. The area under the ROC curve (AUC) of the hs-cTnT levelon admission was the largest (AUC=0.863, P<0.01). The in-hospital mortality of patients in the systolic dysfunction group was higher than that of patients in the normal systolic function group (52.5% vs 22%, P=0.004). Conclusions:The heart rate, the GCS score and the serum hs-cTnT level on admission were independent risk factors for iTBI patients complicated with systolic dysfunction. The hs-cTnT level could better predict the occurrence of cardiac systolic dysfuncion, and higher in-hospital mortality was found in iTBI patients complicated with systolic dysfunction. Therefore, early detection and timely intervention may improve the prognosis of these patients.