Objective The aim of the present study is to evaluate the reliability and validity of the Mandarin version of the PP (MPP) .Methods The first step in the establishment was to translate the original English version into mandarin version with the method of cross -culture translation .The reliability was performed with the internal consistency analysis and test -retest reliability .The validity was performed for the content validity and structure va-lidity .The samples were from 80 Chinese CI children ,and 43 parents answered this questionnaire again 1 month lat-er to evaluate the test -retest reliability .The average age at cochlear implantation were 26 ± 14 months ,ranging from 7 months to 68 months ,the average duration of CI use were 10 ± 7 months ,ranging from 0 month to 24 months .Results The reliability analysis indicates that the Cronbach'sαcoefficient was 0 .797 ,except for the well-being and happiness ,education ,whose coefficients are respectively 0 .303 ,and 0 .341 ,all of the other sundomainscoefficient were greater than 0 .5 ,indicating the internal consistency was good .Test -retest reliability of the scale Cronbach'sαwas satisfactory .All subdomains and total score of the scale coefficients were greater than 0 .70(P<0 .01) .The validity analysis indicated that the pearson correlation coefficients among the total scale and the 8 subdo-mains were 0 .395~0 .992 ,the correlation coefficients among each subdomains were 0 .09~0 .654 ,which confirmed with the psychological characteristics ,proving its good structure validity .Conclusion The Chinese version of the PP show s good reliability and validity and can be used to evaluation the quality of life in mandarin CI children.

Objective:To analyze the application for and funding for individual key clinical specialties under the Youth Program at the approved hospital groups, for suggestions on future development of hospitals.Methods:Based on the Youth Program data of the applications and approval as summarized by Beijing Hospitals Authority from year 2015 through 2018, 22 municipal hospitals were classified into three groups with more, less and none key clinical specialties respectively(the more group, the less group and the non group for short). The categories were made according to the number of state key clinical specialties approved by National Health Commission and key specialties approved by the National Administration of Traditional Chinese Medicine. χ2 test was used to compare the baseline situation, the approval rate of project applications, and the categories of approved project among the three hospital groups. Results:During 2015—2018 period, the number of applicants for Youth Program was 1 128 persons/cases. The groups with more, less, and none key clinical specialties were 7, 10, 5 respectively. In the more specialties group, the applicants of seniority less than five years accounted for 40.93%, who had higher than those of the other two groups( χ2=13.794, P=0.032), and higher doctoral degree applicants(80.31%) as well. The difference was statistically significant( χ2=116.611, P<0.001). The approval rate of the Youth Program in 2015—2018 in the group with key clinical specialties vs. the group with none key clinical specialties was 36.09%(166/460) rersus. 29.04%(194/668) with significant difference( χ2=6.222, P=0.013). The approval rate of the departments with more key clinical specialties was 37.82%, higher than those of less and none key clinical specialties(approval rate at 31.82% and 21.50%, respectively), with significant difference( χ2=16.897, P<0.001). However, there was no significant difference between the approval rates of key specialties group and non-key specialties in each hospital group( P>0.05). Of the three groups, the proportion of clinical medical projects in each group was all higher than such other types as scientific research, medical technology, hospital administration and nursing. Conclusions:The applications and approval rate of key specialties under the Youth Program were higher than the rest, reflecting the discipline advantage. At the same time, it can play an important leading role in the construction of research-oriented hospitals. It is necessary to further strengthen the showcase role of specialties, for encouraging innovative talents cultivation of other specialties, in the hope for promoting the construction of research-oriented hospitals in their clinical, scientific research, medical technology, management and nursing comprehensively.

Objective:To analyze the public demands for information about congenital birth defects in “Baidu zhidao” based on word frequency retrieval.Methods:Based on discussion between obstetrics and gynecology experts and epidemiological experts, the key words related to congenital birth defects were determined and the search strategy was formulated. Python 2.7 was used for web crawler search. Questions related to congenital birth defects were obtained on the “Baidu zhidao” platform, and then the R 4.0.2 software was used to process the data, complete the semantic analysis of keywords and statistical analysis of word frequency, and draw word cloud graph and polar chart to describe the key results.Results:A total of 16668 non-repetitive questions were retrieved from “Baidu zhidao” platform, and the frequency of semantic words was 15 371. Among them, 35.02% were the names and symptoms of congenital birth defects. In addition, the frequency of congenital heart disease was the highest (26.09%). The results of subject analysis of key words of birth defects showed that the average word frequency of diagnosis and treatment semantic words (49.55) was significantly higher than that of etiology and prevention semantic words (12.47). In addition, the key words of examination, cause, treatment, development and heredity were more frequently used in the semantic words related to the seven types of systemic malformations.Conclusion:The public in China has a high demand for information on congenital birth defect related diseases, and their causes, prevention and treatment, especially congenital heart disease.

OBJECTIVETo study the anti-inflammatory mechanism of total saponins from Semen Nigellae (TSSN).

METHODIFN-gamma plus LPS stimulated RAW 264. 7 macrophage has been used as inflammatory experimental model. Griess reaction for nitric oxide production, FRAP assay for total antioxidant capacity, RT-PCR for mRNA expression and Western blot for protein expression examination were performed.

RESULTTSSN inhibited NO production in a dose-dependent manner. The gene and protein expression of iNOS were also suppressed by the herb extract. TSSN treatment significantly attenuated mRNA of inflammatory mediators such as COX-2, IL-1beta, IL-6 while increased PPAR-gamma gene and protein expression. Furthermore, phosphorylation of ERK (p-ERK) was markedly inhibited by TSSN.

CONCLUSIONTSSN suppressed pro-inflammatory mediators such as COX-2, IL-1beta, IL-6 and increased anti-inflammatory mediator PPAR-gamma expression. Meanwhile, TSSN inhibited over production of NO and iNOS expression through ERK/MAPK pathway.

Animals ; Anti-Inflammatory Agents ; pharmacology ; Cells, Cultured ; Cyclooxygenase 2 ; metabolism ; Humans ; Inflammation ; chemically induced ; Inflammation Mediators ; metabolism ; Interleukin-6 ; metabolism ; Lipopolysaccharides ; pharmacology ; Macrophages ; drug effects ; enzymology ; metabolism ; Mice ; Nitric Oxide ; metabolism ; Nitric Oxide Synthase Type II ; metabolism ; Phosphorylation ; drug effects ; Saponins ; pharmacology ; Signal Transduction

Objective To evaluate the effect of two-site blood cultures on detection rate in pediatric patients.Methods The data were retrospectively analyzed of 1 985 hospitalized children with blood cultures from January 2013 to February 2015 in Department of Infectious Diseases,Beijing Children's Hospital,Capital Medical University,including blood culture collection,the administration of antibiotics prior to obtaining blood cultures and positive condition of blood culture.It was divided into 3 stages according to blood culture collection.Blood culture of a single bottle referred to the blood culture in an aerobic bottle.Double bottles transition stage referred to two blood samples taken from the same skin puncture point and the aerobic bottle culture was carried out at the same time.Two-site blood cultures referred to two blood samples taken from the different skin puncture point and the aerobic bottle culture was carried out at the same time.The interval time between the two blood cultures should be less than 5 minutes.The positive rates of three stages were analyzed by Pearson x2 test.The change tendency of positive rates in three stages were analyzed by Cochran-Armitage test.Bilateral P ＜ 0.05 was considered as statistically significant difference in all test analysis.Results More than 80％ of the children in the three stages were given antibiotics.There was no significant difference in the true positive rate (x2 =1.343,P ＞ 0.05).There was no significant difference in the change tendency of positive rates (P ＞ 0.05).False-positive strains were common for coagulase-negative staphylococci.In terms of false positive rate,blood culture of single bottle was higher than two-site blood culture (x2 =6.051,P ＜ 0.05).Conclusion For children (non-neonates),two-site blood cultures can reduce the false positive rate of blood culture and play a role in distinguishing between true positives and false positives in blood culture.

Objective To explore the significance of the initial clinical symptoms and clinical manifestations of neuroblastoma(NB)to achive early identification of NB. Methods A retrospective study was performed on patients diagnosed with NB,who attended the Hematology Oncology Center,Beijing Childrenˊs Hospital from March 31st ,2007 to March 31st,2015. The clinical characteristics were compared between the children 〈1_year_old and≥1_year_old. The result was analyzed to compare the difference in clinical symptoms and tumor biologic characteristics of patients with different initial clinical symptoms between 2 groups. Results A total of 330 patients were included in the study,43 of them were younger than 1 year old,and their most common symptoms were cough and fever( each 17 cases,and accounted for 39. 5%,respectively);while the most common symptoms in patients≥1_year_old(287 cases)was fe_ver(177 cases,61. 7%),followed by lymphadenopathy(107 cases,37. 3%),bone pain(97 cases,33. 8%)and anemia (48 cases,16. 7%). The frequency of symptoms differed significantly between 2 groups(all P〈0. 05),such as fever (39. 5% vs. 61. 7%,χ2 ﹦6. 68),anemia(4. 7% vs. 16. 7%,χ2 ﹦6. 00),bone pain(0 vs. 33. 8%,χ2 ﹦18. 99),abdo_minal pain(0 vs. 25. 3%,χ2 ﹦10. 19),diarrhea(16. 3% vs. 3. 0%,χ2 ﹦12. 73),lymphadenopathy(7. 0% vs. 37. 3%, χ2 ﹦14. 12)and anorexia(9. 3% vs. 33. 4%,χ2 ﹦9. 21). Datients had fever,anemia,lymphadenopathy,cutaneous hemorrhagic spot or periorbital ecchymosis,bone pain,abdominal pain,exophthalmos,and anorexia early in the initial course of the disease,whose serum lactate dehydrogenase values were significantly increased(P〈0. 05). Datients suf_fered from fever,anemia,lymphadenopathy,bone pain,limbs dysfunctions,abdominal pain at the beginning of the disease,whose urine vanillymandelic acid values were higher than normal(P〈0. 05). Conclusions The study of sympto_mology suggests the most common symptoms in patients with NB 〈1_year_old are cough and fever,while those ≥1_year_old are fever,lymphadenopathy,bone pain,and anemia. Por patients with symptoms mentioned,carrying out the necessary NB_diagnose_related laboratory and imaging studies was statistically relevant to patientsˊ ages(〈1_year_old and≥1_year_old),which may contribute to earlier identification and diagnosis of NB.

Objective: To investigate the clinical data of children with inflammatory bowel disease (IBD) retrospectively, including Crohn′s disease (CD) and ulcerative colitis (UC) and identify the clinical characteristics and trends of change. Method: Clinical data of hospitalized patients diagnosed as IBD in Beijing Children′s Hospital from January 2000 to December 2014 were collected and retrospectively analyzed. Patients were divided into six groups based on type of disease and year of admission: Group A1(CD, 2000-2004) included 12 patients, Group B1(CD, 2005-2009) included 11, Group C1(CD, 2010-2014) included 51; Group A2(UC, 2000-2004) included 17, Group B2(UC, 2005-2009) included 25, Group C2(UC, 2010-2014) included 68. Result: A total of 184 IBD patients were included in the study, 74 had CD and 110 had UC. The hospitalization constituent ratio of CD increased from 0.6/10 000 in Year 2000 to 2.9/10 000 in Year 2014. The hospitalization constituent ratio of UC increased from 0.5/10 000 in Year 2001 to 3.9/10 000 in Year 2014. The hospitalization constituent ratios of CD and UC both increased gradually(P<0.05). Up to 61.4%(113/184) of IBD patients belong to early onset IBD, furthermore the very early onset IBD and infantile IBD accounted for 41.8%(77/184) and 26.6%(49/184) respectively. For CD, ileocolonic type(47.3%, 35/74) and non-structuring, non-penetrating type (67.6%, 50/74) were more common. Perianal disease occured in 31.1%(23/74) of CD patients; 81.1%(60/74) of CD patients had moderate/severe activity. For UC, pancolitis type(59.1%, 65/110) was more common. There were no significant changes for location of pathological change, disease behavior, activity degree of CD, extent of UC lesion and incidence of surgery, intestinal perforation and hemorrhage of gastrointestinal tract for IBD in the past 15 years(P>0.05). Severe UC(S3) was more common in Group A2(64.7%, 11/17), but moderate UC(S2) was more common in Group C2(48.5%, 33/68), the difference was statistically significant (P=0.001 7). Conclusion During the past 15 years, the hospitalization constituent ratio for IBD in our hospital showed a growing trend. The ratio of infantile IBD and very early onset IBD was high. For CD, perianal disease was commonly seen and most patients had moderate/severe activity. The surgery rate and incidence of intestinal obstruction and perforation were higher in the CD patients than UC patients. For UC, the lesions were more extensively combined with higher disease activity.

Objective To evaluate the effect of an emollient containing Prinsepia utilis Royle oil extracts and other extracts on clinical symptoms and disease recurrence in children aged 2-12 years with atopic dermatitis (AD) in the remission period.Methods A multicenter,randomized,parallel-group,controlled clinical trial was conducted from December 2017 to September 2018.A total of 297 children aged 2-12 years with moderate AD were enrolled from 5 hospitals in China,and randomly divided into the test group (148 cases) and control group (149 cases).In the acute stage,the two groups were both topically treated with mometasone furoate cream once a day on the skin lesions,and with an emollient containing Prinsepia utilis Royle oil extracts and other extracts twice a day throughout the whole body for 2-4 weeks.The children would be enrolled into the remission stage if their Investigator's Global Assessment (IGA) score was ≤ 1 at following visits.In the remission stage,the test group was only topically treated with the emollient twice a day throughout the whole body,while mometasone furoate cream and the emollient were both withdrawn in the control group.At weeks 4,8 and 12 in the remission stage,the recurrence of AD,eczema area and severity index (EASI),children's dermatology life quality index (CDQOL) and adverse events were evaluated.Statistical analysis was carried out with SAS 9.4 software by using t test for comparison of normally distributed continuous data between two groups,chi-square test for comparison of unordered categorical data,Kaplan-Meier method for analysis of survival rates,Cox regression analysis for evaluating the effect of different therapies on AD recurrence in children in the remission stage,and Logistic regression analysis for analysis of odds ratio (OR) of EASI or CDQOL at week 4 in the remission stage between the test group and control group.Results Of the 297 children with AD,31 breached the clinical trial protocol,and 266 were included in the per protocol set (PPS),including 132 in the test group and 134 in the control group.In the PPS,114 and 106 patients completed the follow-up in the test group and control group respectively,and the recurrence rate was significantly lower in the test group (47,41.23％) than in the control group (84,79.25％;x2 =32.96,P ＜ 0.001).The time to recurrence was significantly longer in the test group(61.99 d ± 2.80 d)than in the control group(39.17 d ± 2.54 d,t =6.03,P ＜ 0.001),and the recurrence risk was significantly lower in the test group than in the control group (Log rank test,x2 =32.02,P ＜ 0.001).After adjustment for age and gender,Cox regression analysis showed that the recurrence risk in the test group was 0.35 times that in the control group (HR =0.35,95％ CI:0.24-0.51,P ＜ 0.01).At week 4 in the remission stage,the EASI score at P50-P75 and P75-P100 in the test group were 0.42,0.25 times that in the control group respectively (95％ CI:0.20-0.86,0.12-0.54 respectively;P =0.02,＜ 0.01respectively).Moreover,the CDQOL score at P75-P100 in the test group was 0.33 times that in the control group (95％ CI:0.17-0.65,P ＜ 0.01).No significant difference in the incidence of adverse events was observed between the two groups (P ＞ 0.05).Conclusion Maintenance treatment with the emollient containing Prinsepia utilis Royle oil extracts and other extracts can markedly reduce the recurrence risk in AD children,improve clinical symptoms,and enhance the quality of life.

Objective:To investigate the clinical phenotype and prognosis among different genotypes of progressive familial intrahepatic cholestasis(PFIC) by cases analysis.Methods:The PFIC cases diagnosed at Beijing Children′s Hospital from 2015 to 2022 were collected, and the clinical phenotypic characteristics, treatment and prognosis were compared and analyzed.Results:A total of 628 cases of cholestatic liver disease were diagnosed, and 26 cases of PFIC were found, accounting for 4.1%.The number of PFIC 2 were the most, 14(53.8%)cases; three(11.5%) cases were PFIC 1; five(19.2%)cases were PFIC 3; while two(7.7%) cases were PFIC 4 and PFIC 6, respectively, and there was no case of PFIC 5.Type 1, 2, 4, and 6 had early onset ages(2 days to 21 months), while type 3 had a wide range of onset ages(8 to 145 months). The symptoms included jaundice(96.2%), pruritus(42.3%), and mucosal bleeding(15.4%). All three cases of type 1 had extrahepatic manifestations of diarrhea and malnutrition.Two cases of type 3 were found to have end-stage liver disease.Cases of PFIC 3 had increased serum γ-glutamyltransferase(97.2-439.5 U/L), while those of other types were normal.The bile acids were all increased(10.1-599.6 μmol/L). Abdominal ultrasound mainly showed liver enlargement(80.8%)and enhanced echogenicity of liver parenchyma(73.1%), enlargement of the spleen(61.5%). Ultrasound liver elastography ranged from 6.3 kPa to 23.1 kPa, there were 21(80.8%) cases ≥9 kPa.Among 26 cases, one case was lost to follow-up, and 11 cases were effective by oral medication alone.Fourteen children were still suffering from relapse or progress after drug treatment: four cases received liver transplantation (three cases had a good prognosis and one case died), two cases received biliary drainage, six cases were still taking drugs orally, and two cases died without active intervention in disease progress.Conclusion:Type 2 is the most common type in PFIC.The onset of most cases is in infancy.Jaundice, pruritus and hepatosplenomegaly are common clinical manifestations, and extrahepatic manifestations can be seen in type 1 cases.Type 3 cases can start with end-stage liver disease.Bile acid of all cases are increased.Except for type 3, the serum γ-glutamyltransferase of cases are normal.Oral medication has certain effects on some cases, but more than half progress, and some need biliary diversion or liver transplantation.

Objective:To explore the differences of risk stratification of very high-risk or extreme high-risk atherosclerotic cardiovascular diseases (ASCVD) and the attainment rates of low-density lipoprotein cholesterol (LDL-C) management targets evaluated by three different criteria, and the causal attributions of these differences.Methods:Patients with ASCVD were consecutively enrolled from January 1 to December 31 in 2019, and were evaluated for very high-risk or extreme high-risk and LDL-C goal attainment rates with 2018 American guideline on the management of blood cholesterol (2018AG), 2019 China Cholesterol Education Program (CCEP) Expert Advice for the management of dyslipidemias (2019EA) and 2020 Chinese expert consensus on lipid management of very high-risk ASCVD patients(2020EC), respectively. The causal attributions of the differences in attainment rates were analyzed as well.Results:A total of 1 864 ASCVD patients were included in this study. According to 2018AG, 2019EA and 2020EC, the proportions of the patients with very high-risk or extreme high-risk were 59.4%, 90.7%, and 65.6%, respectively. The absolute LDL-C target attainment rates were 37.2%, 15.7%, and 13.7%, respectively, the differences between each two rates were statistically significant (all P<0.001). As to the differences in attainment rates between 2020EC and 2018AG, 61.5% were due to the different LDL-C goal attainment values and 38.5% were caused by the different risk stratifications, while for the differences between 2020EC and 2019EA attainment rates, different LDL-C goal attainment values were responsible for 13.2%, and different risk stratifications were responsible for 86.8% of the differences. Conclusions:There are significant differences in the proportions and LDL-C attainment rates among the three different criteria for very high-risk or extreme high-risk ASCVD. 2020EC showed a moderate proportion of patients with extreme high-risk, and had the lowest LDL-C attainment rate. The differences between 2020EC and 2018AG are mainly due to the LDL-C target values, and the differences between 2020EC and 2019EA are mainly caused by the risk stratifications.