OBJECTIVESince glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary hemolytic erythrocyte enzyme deficiency, most cases have single nucleotide mutations in the coding region, and current test methods for gene mutation have some missed detections, this study aimed to investigate the feasibility of RT-PCR sequencing in the detection of gene mutation in G6PD deficiency.

METHODSAccording to the G6PD/6GPD ratio, 195 children with anemia of unknown cause or who underwent physical examination between August 2013 and July 2014 were classified into G6PD-deficiency group with 130 children (G6PD/6GPD ratio <1.00) and control group with 65 children (G6PD/6GPD ratio≥1.00). The primer design and PCR amplification conditions were optimized, and RT-PCR sequencing was used to analyze the complete coding sequence and verify the genomic DNA sequence in the two groups.

RESULTSIn the G6PD-deficiency group, the detection rate of gene mutation was 100% and 13 missense mutations were detected, including one new mutation. In the control group, no missense mutation was detected in 28 boys; 13 heterozygous missense mutations, 1 homozygous same-sense mutation (C1191T) which had not been reported in China and abroad, and 14 single nucleotide polymorphisms of C1311T were detected in 37 girls. The control group showed a high rate of missed detection of G6PD deficiency (carriers) in the specimens from girls (35%, 13/37).

CONCLUSIONSRT-PCR sequencing has a high detection rate of G6PD gene mutation and a certain value in clinical diagnosis of G6PD deficiency.

Adolescent ; Child ; Child, Preschool ; Female ; Glucosephosphate Dehydrogenase ; genetics ; Glucosephosphate Dehydrogenase Deficiency ; diagnosis ; genetics ; Humans ; Infant ; Male ; Mutation ; Reverse Transcriptase Polymerase Chain Reaction ; methods ; Sequence Analysis, DNA

Objective To evaluate efficacy and safety of ursodeoxycholic acid(UDCA)for preven-tion of obstruction of biliary plastic stent.Methods A total of 88 patients with plastic biliary stents at our hospital were divided into UDCA group and the control group.Stents were collected 3 months after the opera-tion for argile biliare in the stent.The contents of APF,calcium bilirubin and calcium carbonate were ana-lyzed by biochemistry,and bacterium culture was conducted.Results The obstruction rate of UDCA group (n=43)and control group(n=45)were 8. 9% and 58. 1% respectively with significant differences(P<0. 05).There were significant differences in the protein level of APF between the UDCA group(17. 29 ± 9. 52)μm/L and the control group(10. 39 ±2. 17)μm/L(P<0. 05).Calcium bilirubin[(13. 90 ±3. 80) vs.(30. 92 ±7. 07)]and calcium carbonate[(12. 60 ±5. 69)vs.(16. 52 ±4. 11)]were less than those of the control group (P<0. 05 ).Only one type of bacterium was found,and Escherichia coli were cultured in 40. 0% samples,a lower incidence compared with the control(72. 1%).Conclusion UDCA would lessen biofilm and smooth stent surface by promoting secretion of endogenous bile acid and increasing APF,and keep the biliary patency.

Influenza caused by influenza virus,seriously threaten human life and health.Drug treatment is one of the effective measurement. However, there are only two classes of drugs, one class is M2 blockers and another is neuraminidase (NA)inhibitors. The recent antiviral surveillance studies reported a global significant increase in M2 blocker resistance among influenza viruses, and the resistant virus strains against NA inhibitor are also reported in clinical treatment.Therefore thediscovery of new medicines with low resistance has become very urgent.As all known,traditional medicines with multi-target features and network mechanism often possess low resistance. Compound Yizhihao, which consists of radix isatidis,folium isatidis,Artemisia rupestris,is one of the famous traditional medicine for influenza treatment in China, however its mechanism of action against influenza is unclear. In this study, the multiple targets related with influenza disease and the known chemical constituents from Compound Yizhihao were collected, and multi-target QSAR (mt-QSAR) classification models were developed by Na?ve Bayesian algorithm and verified by various datasets. Then the classification models were applied to predict the effective constituents and their drug targets.Finally,the constituent-target-pathway network was constructed,which revealed the effective constituents and their network mechanism in Compound Yizhihao. This study will lay important basis for the clinical uses for influenza treatment and for the further research and development of the effective constituents.

China/epidemiology* ; Coronary Angiography ; Coronary Artery Disease/epidemiology* ; Cross-Sectional Studies ; Humans ; Risk Assessment ; Risk Factors

Objective To evaluate the effects of different target plasma concentrations of propofol on ventricular repolarization in elderly patients.Methods Forty-five patients,aged 65-80 yr,weighing 43-85 kg,of American Society of Anesthesiologists physical status Ⅰ or Ⅱ,scheduled for elective surgery under general anesthesia,were divided into 3 groups (n=15 each) using a random number table:propofol at target plasma concentration of 2 μg/ml group (group P1),propofol at target plasma concentration of 3 μg/ml group (group P2) and propofol at target plasma concentration of 4 μg/ml group (group P3).Before induction of anesthesia (T1) and at 5 min after propofol reached the target plasma concentration (T2),12-lead electrocardiogram was recorded,the QT and Tp-e intervals were measured,and the corrected QT (QTc) interval and Tp-e/QT ratio were calculated.Results There were no significant differences in QTc interval,Tp-e interval or Tp-e/QT ratio at T1,2 between the three groups (P＞0.05).Compared with those at T1,the QTc and Tp-e intervals were significantly shortened and the Tp-e/QT ratio was decreased at T2 in P1 and P2 groups,and the Tp-e interval was shorten and the Tp-e/QT ratio was decreased at T2 (P＜ 0.05),and no significant change was found in the QTc interval at T2 in group P3 (P＞0.05).Conclusion Propofol at clinically relevant concentrations can shorten the ventricular repolarization in elderly patients.

BACKGROUND: Topoisomerase II alpha (TOP2A) has been reported to play a crucial role in the tumorigenesis of various cancer types. However, the biological role of TOP2A in gallbladder cancer (GBC) remains unknown. The current study aimed to explore the function and potential mechanism of TOP2A in GBC. METHODS: Based on Gene Expression Profiling Interactive Analysis data, we found TOP2A was significantly up-regulated in GBC tissues and resulting in shorter overall survival. Quantitative real-time polymerase chain reaction and immunohistochemistry were conducted to detect the expression of TOP2A in 45 pairs of GBC tissues and adjacent non-tumor tissues. In vitro, cell proliferation, migration, and invasion ability were examined by cell counting kit-8 and transwell assay, respectively. Epithelial-mesenchymal transition (EMT) related and phosphatidylinositol 3-kinase/protein kinase B/mammalian target of rapamycin (PI3K/Akt/mTOR) pathway-related markers were measured by Western blotting. Xenograft model assay was performed to evaluate the effect of TOP2A in vivo. RESULTS: TOP2A was found up-regulated in GBC (tumor vs. normal, 12.62 vs. 0.34) and correlated with the late tumor node metastasis stage (P = 0.0032), present of lymph node metastasis (P = 0.0273), and poor prognosis in GBC patients (log-rank P = 0.028). In vitro and in vivo assays showed that knockdown of TOP2A notably inhibited cell proliferation, migration, invasion, EMT process, and tumor growth in GBC. In addition, TOP2A down-regulation significantly decreased the protein levels of phosphor (p)-PI3K, p-Akt, and p-mTOR. CONCLUSION Our study demonstrates that TOP2A was overexpressed in GBC and associated with poor prognosis in GBC patients. TOP2A promotes GBC cell proliferation, migration, invasion, EMT process, and tumor growth through activating PI3K/Akt/mTOR signaling pathway, and may serve as a novel prognostic biomarker and therapeutic target for GBC.

BACKGROUNDThalidomide is an immunomodulatory and anti-angiogenic drug that has shown promise in patients with myeloma. Trials comparing efficacy of standard melphalan and prednisone (MP) therapy with MP plus thalidomide (MPT) in transplant-ineligible or elderly patients with multiple myeloma (MM) have provided conflicting evidence. This meta-analysis aimed to determine the efficacy and toxicity of thalidomide in previously untreated elderly patients with myeloma.

METHODSMedline, the Cochrane Controlled Trials register, conference proceedings of the American Society of Hematology (1995-2014), the American Society of Clinical Oncology (1995-2014), and CBM, VIP, and CNKI databases were searched for randomized control trials with the use of the medical subject headings "MM " and "thalidomide ". Trials were assessed by two reviewers for eligibility. Meta-analysis was conducted using a fixed effects model. Sensitivity analysis was performed to test the robustness of the findings.

RESULTSOverall, seven trials were identified, covering a total of 1821 subjects. The summary hazard ratio (thalidomide vs. control) was 0.82 (95% confidence interval [CI]: 0.72-0.94) for overall survival (OS), and 0.65 (95% CI: 0.58-0.73) for progression-free survival, in favor of thalidomide treated group. The risk ratio of complete response with induction thalidomide was 3.48 (95% CI: 2.24-5.41). A higher rate of III/IV adverse events were observed in MPT arm compared with the MP arm. However, analysis of sub-groups administering anticoagulation as venous thromboembolism prophylaxis suggested no difference in relative risk of thrombotic events between two arms (RR = 1.47, 95% CI: 0.43-5.07, P = 0.54). Further analysis of trials on the treatment effects of MPT versus MP on adverse events-related mortality showed no statistical difference between two arms (RR = 1.24, 95% CI: [0.95-1.63], P = 0.120).

CONCLUSIONThalidomide appears to improve the OS of elderly and/or transplant-ineligible patients with MM when it is added to standard MP therapy.

Disease-Free Survival ; Humans ; Immunosuppressive Agents ; therapeutic use ; Melphalan ; therapeutic use ; Multiple Myeloma ; drug therapy ; mortality ; Prednisone ; therapeutic use ; Thalidomide ; therapeutic use

Objective To compare the clinical effect of endoscopic thyroidectomy and open thyroidectomy in the treat-ment of benign thyroid diseases. Methods A total of 120 patients with benign thyroid diseases were selected from March 2015 to June 2017 in the Third Affiliated Hospital of Xinxiang Medical University. The patients were divided into endoscopic opera-tion group(n = 62)and open operation group(n = 58)according to the treatment methods. The patients in the open operation group were treated with conventional open thyroidectomy,and the patients in the endoscopic operation group were treated with endoscopic thyroidectomy. The operation time,intraoperative bleeding volume,postoperative drainage volume and hospitalization time were recorded in the two groups;and the intraoperative and postoperative complications were observed. The pain of the pa-tients in the two groups was evaluated by visual analogue scale(VAS)at the time points of 6,12 and 24 hours after operation. The cosmetic satisfaction of the patients in the two groups was evaluated by numerical scoring system(NSS)at the time points of 1 and 3 months after operation. Results The operation time and hospitalization time in the endoscopic operation group were significantly shorter than those in the open operation group(P < 0. 05),and the intraoperative bleeding volume and postopera-tive drainage volume in the endoscopic operation group were significantly lower than those in the open operation group(P <0. 05). The VAS score of pain in the endoscopic operation group was significantly lower than that in the open operation group at the time points of 6,12 and 24 hours after operation(P < 0. 05). The NSS score in the endoscopic operation group was sig-nificantly higher than that in the open operation group at the time points of 1 and 3 months after operation(P < 0. 05). The in-cidence of complications in the endoscopic operation group and the open operation group was 12. 90％(8 / 62)and 39. 66％(23 / 58)respectively,the incidence of complications in the endoscopic operation group was significantly lower than that in the open operation group(χ2 = 11. 210,P < 0. 05). Conclusion Endoscopic thyroidectomy is reliable in the treatment of benign thyroid diseases. It has the advantages of small trauma,short operation time,quick recovery,light postoperative pain,small sur-gical scar and fewer complications and so on.

OBJECTIVE: To perform a review and data analysis of the pediatric projects funded by National Natural Science Foundation of China from 2009 to 2018, and to investigate the changes in key support areas, research interest, and research hotspots in pediatrics. METHODS: The database of National Natural Science Foundation of China was searched to screen out pediatric research projects in 2009-2018, and the changes in funding intensity and research direction were analyzed. RESULTS: From 2009 to 2018, a total of 1 017 pediatric projects were funded by National Natural Science Foundation of China, with 485 (47.69%) General Projects, 426 (41.89%) Youth Fund Projects, 73 (7.18%) Regional Research Programs, 16 (1.57%) Key Programs, 6 (0.59%) Outstanding Youth Fund Projects, 7 (0.69%) Overseas Programs, and 4 (0.39%) other programs. There was a seven-fold increase in the total amount of subsidies, which increased from 8.42 million yuan in 2009 to 66.25 million yuan in 2018. The projects with the Primary Discipline Code of reproductive system/perinatology/neonatology, nervous system and mental illness, or circulatory system received the highest amount of fund. CONCLUSIONS The support of pediatric projects by National Natural Science Foundation of China continues to increase in the past ten years, and the main types of projects are General Projects and Youth Fund Projects. Neonatology, nervous system/mental illness, and circulatory diseases are the main directions of funded projects.
Adolescent ; Child ; China ; Financial Management ; Foundations ; Humans ; Natural Science Disciplines ; Neonatology

BACKGROUNDSurgical resection is generally considered the main curative treatment for intrahepatic biliary cystadenocarcinoma (IBCA) or suspected IBCAs, but controversy exists regarding the prognosis for IBCAs. This study aimed to describe the clinicopathological characteristics of IBCA and identify prognostic factors that may influence the survival of patients treated with surgical procedures.

METHODSThirty-four patients with histologically confirmed IBCA treated between January 2000 and June 2014 were included. The clinical characteristics of patients with IBCA were compared with those of 41 patients with intrahepatic biliary cystadenoma (IBC); factors that significant difference were analyzed for prognosis analysis of IBCA using multivariate/univariate Cox proportional hazards regression models. Survival curves were constructed using the Kaplan-Meier method and compared using the log-rank test.

RESULTSIBCAs had a strong female predominance, and the most common presenting symptoms were abdominal pain or discomfort. Compared with IBCs, IBCAs occurred in older patients, in more male patients, and were associated statistically significant abnormal increase in alanine aminotransferase (P = 0.01) and total bilirubin (P = 0.04). Mural nodules were more frequently seen with IBCAs and may associate with malignancy. It was difficult to differentiate between IBC and IBCA based on laboratory examination and imaging findings. Although complete resection is recommended, enucleation with negative margins also achieved good outcomes. Median overall patient survival was 76.2 months; survival at 1, 3, and 5 years was 88.0%, 68.7%, and 45.8%, respectively. Radical resection and noninvasive tumor type were independent prognostic factors for overall survival.

CONCLUSIONSIt remains difficult to distinguish between cystadenomas and cystadenocarcinomas based on laboratory examination and image findings. Complete resection is recommended for curative treatment, and patients should be closely followed postoperatively, particularly those with invasive tumors.

Aged ; Bile Duct Neoplasms ; pathology ; Bile Ducts, Intrahepatic ; pathology ; Cystadenocarcinoma ; pathology ; Female ; Humans ; Liver Neoplasms ; pathology ; Male ; Middle Aged ; Prognosis ; Proportional Hazards Models