Primary immunodeficiency disease (PID) are rare diseases caused by inherent defects of the immune system,the main clinical manifestations are increased susceptibility to infections,autoimmune diseases and malignancies.For such rare diseases,it's essential to establish national patient online database for collaborative studies.By using hypertext preprocessor (PHP) and MySQL,the database and registration system are successfully set up,and itcould complete inquiries for the information saving,export,query,retrieval,backup etc.This database includes patient's information on the diagnosis,clinical manifestations,laboratory examinations,treatments.Through communication and information-sharing between multiple centers,it creates a basis for nationwide research on PID.

DNA End-Joining Repair ; Humans ; Immunologic Deficiency Syndromes ; genetics

BACKGROUND: Hip arthroplasty is now an effective method of effectively solving the problem of femoral neck fracture in the elderly, but there is a risk of big trauma, high joint dislocation and postoperative complications. OBJECTIVE: To explore the application value of 3D printing guide plate in hip arthroplasty in the elderly by retrospective study and literature retrieval. METHODS: (1) Clinical data of 25 elderly patients with femoral neck fracture undergoing hip arthroplasty from February 2017 to October 2018 in Liaoning Jinqiu Hospital were retrospectively analyzed. In the guide plate group, 13 patients received hip arthroplasty using 3D printing guide plate. In the non-guide plate group, printing guide plate was not used in 12 cases. The application value of SuperPATH technique with 3D printing guide plate in hip arthroplasty in the elderly was observed. (2) Wanfang database was retrieved by the first author for studies published from 2000 to 2019. The key words were “3D, hip”. According to the inclusion criteria, 31 articles were selected to analyze the application value of 3D printing guide plate in hip arthroplasty in the elderly. RESULTS AND CONCLUSION: (1) The results of retrospective analysis showed that the operation time and the times of X-ray fluoroscopy were less in the guide plate group than those in the non-guide plate group (P < 0.05). Intraoperative blood loss and Harris score of hip function at the final follow-up were not significantly different between the two groups (P > 0.05). (2) Literature search results showed that 3D guide plates could be used to compensate for some defects of surgery in hip arthroplasty. There are many approaches to implement joint arthroplasty. Both conventional and minimally invasive approaches have direct anterior approach and posterolateral approach. According to several literature analysis, direct anterolateral approach can reduce trauma, and minimally invasive approach can reduce the risk of surgery. The above results show that the use of 3D printing guide plate in elderly hip arthroplasty can reduce the operation time and the times of intraoperative X-ray fluoroscopy, and the effect is better. 3D guide plate has application value in hip arthroplasty, and it has advantages in combination with minimally invasive techniques.

Purpose To study of predictive value for detection of high-grade cervical intraepithelial neoplasia (CIN2 +) by p16/Ki-67 dual-stained liquid-based cytology.Methods Random collection of 123 women including 103 samples of atypical squamous cell of undetermined significance (ASC-US) and above with results of high-risk human papillomavirus (HR-HPV)testing and cervical biopsy,20 samples of negative for intraepithelial lesion or malignancy (NILM) by using immunocytochemical p16/Ki-67 dual-stained and the morphology assessment.Results In normal control group,the expression of p16/Ki-67 dual-stained in squamous epithelial cells were negative.Sensitivity of p16/Ki-67 dual-staind cytology for biopsy-confirmed CIN2 + was 66.67％ (ASC-US),91.67％ (LSIL) and 92.86％ (HSIL),specificity rates were 95.92％ (ASC-US),95.00％ (LSIL) and 0 (HSIL),positive predictive value were 50.00％ (ASC-US),91.67％ (LSIL) and 92.86％ (HSIL),negative predictive value were 97.92％ (ASC-US),95.00％(LSIL) and 0 (HSIL),respectively.Condusion p16/Ki-67 dual-stained cytology are improved obviously the predictive value for detection of CIN2 +,p16/Ki-67 dual-stained cytology may efficiently complement HPV-based screening programs to prevent cervical cancer.

Objective To investigate the diagnostic value of DNA ploidy analysis combined with immunocytochemistry p16/ki-67 double staining in cervical high grade squamous intraepithelial neoplasia(HSIL) and cervical squamous cell carcinoma(SCC).Methods A total of 73 cases of cytological tests were randomly collected.Among them,53 cases were small DNA ploidy abnormal cells and 20 cases were DNA ploidy negative.The p16/Ki-67 results were detected by immunocytochemistry double staining.With the pathological results as the golden standard,the diagnostic values of DNA ploidy analysis and DNA ploidy analysis combined with p16/Ki-67 double staining in HSIL + was contrastively analyzed by pathologic results.Results Among 20 samples of DNA ploidy negative,the p16/Ki-67 double staining results all were negative.The positive predictive value of DNA ploidy analysis for HSIL + was 34.62％.The sensitivity of DNA ploidy analysis combined with p16/Ki-67 double staining for HSIL + was 84.62％,and its specificity was 92.31％,the positive predictive value was 78.57％ and the negative predictive value was 94.74％,which were significantly higher than those of DNA ploidy analysis(P＜0.05).Conclusion p16/Ki-67 double staining can significantly im prove the prediction value of HSIL.The DNA ploidy analysis combined with p16/Ki-67 double staining is an effective method for predicting HSIL +,which is suitable for the implementation in the areas with lack of medical resources.

Background Atrial electrical remodeling(AER)plays an important role in the pathogenesis and maintenance of atrialfibrillation.However,little is known about modulation of vagal activilty to AER.This study aimed to investigate the relationshipbetween vagal moduation and AER. Methods Twenty four adult mongrel dogs under general anesthesia were randomized into 3groups.Sympathetic activity was blocked by administration of metoprolol in 3 groups.The changes in vagal modulation to atria afterAER were observed in 10 dogs without vagal interruption in group A.The effects of vagal intervention on AER were investigated in 8dogs with administration of atropine in group B.The impact of aggressively vagal activity on AER was studied in 6 dogs with bilateralcervical vag sympathetic trunLks stimulation during AER in group C.Bilateral cervicall vagosympathetic trunks were decentralized.Multipolar catheters wereplaced into high right atria(RA),coronary sinus(CS)and rightventricle(RV).AER was induced by 600 bpmpacing through RA catheter for 30 minutes.Attial effective refractory period(ERP)and vulnerability window (VW)of atrial fibrillationwere measured with and without vagal stimulation before and after AER.Results In group A,ERP decreased significantly at baselineand during vagal stimulation after AER compared with that beforeAER(all P＜0.05).In group B,ERP remaind unchanged at baselineand vagal stimulation after AER compared with tbat before AER (all P＞0.05).In group C,ERP shortened significantly at baseline andvagal stimulation after AER compared with that before AER(all P＜0.05).ERP shortening after AER in Groups A and C increasedsignificantly than that in group B (all P＜0.05).Atrial fibrillation could not be induced at baseline(VW close to 0) before and after AERin three groups.VW became widen significantly during vagal stimulation after AER compared with that before AER in Groups A and C(all P＜0.05),while VW remained unchanged in group B (VW close to 0).Conclusions Short-term AER results in the decrease inERP.AER is accompanied by the increases in atrial vagal modulation.The increased vagal activity and vagal stimulation promote AER,thereby increase the susceptibility to atrial fibrillation.The interrupted vagal activity attenuates AER.thereby suppresses the atriaIfibrillation mediated by vagal stimutlation.

Objective To evaluate the satisfaction and current situation of advanced health assessment course for postgraduates. So that it can provide the evidence for the development of teaching reform. Methods Facilitating the convenient sampling of 69 nursing master′s degree authorized schools in China. And a further survey using self-designed questionnaires was conducted in the teacher who in charge of this course and 73 postgraduates in 17 schools offering Advanced Health Assessment. The data were analyzed using reliability, validity, frequency and percentage. Results There were 45 schools responded effectively, of which only 17 schools set up this course. There were no uniform norms of the teaching materials, hours and credits. 73 postgraduates were satisfied with the teaching methods, training practical abilities, meeting academic development needs, practicality, and commonality of the advanced health assessment course. However, 54.8％ of the students said the course is too difficult to handle. Conclusion All the schools should pay more attention to Advanced Health Assessment Course and improve the professional level of postgraduates.

The design principle and application of an adjustable spine prone frame are introduced. Adjustable spine prone frame is used in posterior lumbar surgery, during operation, X-ray can shoot fast and internal fixation implants can fix accurately, the vacant abdominal ensure normal respiratory and circulatory function, and the lumbar curvature can adjust conveniently. It meets the surgical position and care requirements, can prevent the complications effectively.
Equipment Design ; Humans ; Lumbar Vertebrae ; surgery ; Orthopedic Procedures ; instrumentation ; Prone Position

OBJECTIVETo observe the effect of Rgl treatment on prognosis of alcoholic hepatitis using a rat model.

METHODSFemale Sprague-Dawley rats were radomly divided into four groups:unmodeled control, untreated model, Rgl-treated model, and dexamethasone (DXM)-treated model. The model groups were generated by intragastric injection of alcohol. The unmodeled control group was given an equal dosage of normal saline by the same route. After model establishment, the Rg1 treatment group and the DXM treatment group were administered a 120-hour treatment of Rgl or DXM; the unmodeled controls were administered normal saline on the same schedule. All rats were then fasted for 120 hours and venous blood samples were collected for detection of serum aspartate aminotransferase (AST), alanine transaminase (ALT), total bilirubin (TBil), albumin (Alb), tumor necrosis factor-alpha (TNFat) and interleukin 6 (IL-6). Markers of liver inflammation were measured by immunohistochemistry, western blotting, and real-time quantitative reverse transcription PCR. Fat and apoptosis indices were assessed by hematoxylin-eosin staining and TUNEL assay, respectively. The t-test and F test were used for statistical analyses.

RESULTSThe model group showed remarkably more liver steatosis (over one-third of the tissue) than the unmodeled control group, indicating proper establishment of alcoholic liver disease in the modeled rats. The AST, ALT, TBil, and IL-6 levels were significantly higher in the untreated model group than in the Rgl-treated group and the DXM-treated group. The values were significantly different between the Rg1-treated group and the DXM-treated group:ALT, 69.19+/-8.00 U/L vs.102.88+/-5.16 U/L; TBil, 0.36+/-0.07 µmol/L vs.1.20+/-0.18 µmol/L; IL-6, 126.50+/-6.50 U/ml vs.169.19+/-7.68 U/ml; TNFa, 268.31+/-13.19 µg/L vs.318.94+/-7.87 µg/L (all P less than 0.05). Expression of caspase3 and caspase8 was significantly higher in the model group than in the Rgltreated group and the DXM-treated group (both P<0.05). The apoptosis index was significantly lower in the Rgltreated group and the DXM-treated group than in the model group (both P<0.05). The mRNA and protein expression of caspase3, caspase8 and NF-kB were significantly lower in the Rgl-treated group and the DXM-treated group than in the model group (allP less than 0.05), and the levels of all were significantly lower in the Rgl-treated group cornered to the DXM-treated group (all P<0.05). Conehision In rats with alcoholic hepatitis, Rg1 can significantly relieve pathological injury, improve liver function by blocking the apoptotic pathway, and inhibit release of inflammatory cytokines.

Alanine Transaminase ; Animals ; Aspartate Aminotransferases ; Bilirubin ; Cytokines ; Disease Models, Animal ; Ethanol ; Female ; Ginsenosides ; Hepatitis, Alcoholic ; NF-kappa B ; Rats ; Rats, Sprague-Dawley

Objective: To examine the regulatory effect of ginsenoside Rg1 (G-Rg1) on endoplasmic reticulum stress and its effect on hepatocellular apoptosis in carbon tetrachloride (CCl₄)-induced acute liver failure (ALF). Methods: Forty healthy, adult male C57/BL mice were randomly divided into normal saline control (NS) group, G-Rg1 blank control (G-Rg1) group, CCl₄ model (CCl₄) group, and G-Rg1 preventive treatment (CCl₄+G-Rg1) group, and an ALF mouse model was established by CCl₄ induction. Blood and liver specimens were collected from all mice upon sacrifice at 12 hours post-intraperitoneal injection. Serum alanine aminotransferase (ALT), serum aspartate aminotransferase (AST) and total bilirubin (TBil) levels were determined using commercial test kits. The mRNA expression of glucose-regulated protein 78 (GRP78) and C/EBP homologous protein (CHOP) was measured using real-time PCR. The protein expression of GRP78, CHOP, caspase12, and caspase3 were measured by Western blot. Histological changes in the liver were assessed by hematoxylin-eosin staining, and the expression of GRP78 and caspase3 was detected by immunohistochemistry. Hepatocyte apoptosis was determined using terminal transferase dUTP nick end labeling. Quantitative data were analyzed using one-way ANOVA, and subsequent pairwise comparisons were performed using the LSD-t method. Results: Serum ALT, AST, and TBil levels in the CCl₄+G-Rg1 group were significantly reduced compared with those in the CCl₄ group (ALT: 691.30 ± 108.06 U/L vs 980.66 ± 110.29 U/L, F = 365.07, P < 0.05; AST: 195.40 ± 15.41 U/L vs 319.44 ± 89.32 U/L, F = 115.64, P < 0.05; TBil: 1.09 ± 0.11 mg/dl vs 1.56 ± 0.12 mg/dl, F = 211.29, P < 0.05). The relative mRNA expression of GRP78 and CHOP was significantly lower in the CCl₄ + G-Rg1 group than in the CCl₄ group (P < 0.05). The relative protein expression of caspase3, GRP78, caspase12, and CHOP was significantly reduced to different extents in the CCl₄+G-Rg1 group compared with those in the CCl4 group (P < 0.05). The CCl₄ + G-Rg1 group showed reduced liver tissue degeneration and necrosis compared with the CCl₄ group. Furthermore, the CCl₄+G-Rg1 group showed significantly fewer brown granules in the liver than the CCl4 group (P < 0.05), indicating that G-Rg1 preventive treatment reduced CCl₄-induced hepatocyte apoptosis. Conclusion G-Rg1 prophylaxis can inhibit inflammation and reduce hepatocyte necrosis and apoptosis during CCl₄-induced ALF. Its mechanism may involve the suppression of endoplasmic reticulum stress-related signaling molecules to alleviate hepatocyte endoplasmic reticulum stress and apoptosis. The results of this study suggest that G-Rg1 may inhibit liver inflammation and hepatocyte apoptosis through multiple targets to protect liver function.