Objective:To understand the current status of pubertal sexual characteristics development of girls aged 6-18 years in Tongzhou District of Beijing and to compare the differences in sexual characteristics development among girls characterized as thin, normal, overweight, and obese.Methods:A cross-sectional survey was conducted among 2 844 girls aged 6-18 years in Tongzhou District of Beijing from September 2022 to July 2023. The developmental stages of breast and pubic hair were assessed on site, and menarche status was inquired. Weight and height were measured. The girls were subsequently characterized into thin, normal, overweight and obese groups. Basic information (including family and personal history) was obtained through questionnaires. Probit probability unit regression was applied to calculate the age of each Tanner stage of sexual characteristics development and the age of menarche. The χ 2 test was applied to compare the counting data between two or multiple groups. Results:A total of 2 844 girls were surveyed and 2 704 girls met the inclusion criteria, resulting in a valid response rate of 95.1%. Among these girls, 1 105 (40.9%) were aged 6-9 years, 1 053 (38.9%) were aged 10-13 years, and 546 (20.2%) were aged 14-18 years. The of height-for-age Z-score (HAZ), weight-for-age Z-score (WAZ), and body mass index-for-age Z-score (BAZ) were 0.46(-0.23,1.16), 0.69(-0.16,1.67), and 0.67(-0.27,1.73) respectively. The prevalences of thin, overweight, and obesity were respectively 1.7% (45/2 704), 17.3% (467/2 704), and 19.9% (538/2 704), respectively. There were 45 girls in the thin group, 1 654 girls in the normal weight group, 1 005 girls in the overweight and obesity group. The age of Tanner stage breast 2 (B2), Tanner stage pubic hair 2 (P2), and menarche was 9.0 (95% CI 8.9-9.1), 10.5 (95% CI 10.4-10.6), and 11.4 (95% CI 11.3-1.5) years, respectively. The current status of breast and pubic hair maturity in girls with pubertal development shows that 64.6% (1 211/1 874) of these girls had breast development preceding pubic hair development, 32.4% (607/1 874) had concurrent breast and pubic hair development, and 3.0% (56/1 874) had pubic hairs development preceding breast development. The interval age between B2 and B5 was 4.7 (95% CI 4.6-4.8) years, between P2 and P5 was 4.5 (95% CI 4.4-4.6) years, and between B2 and menarche was 2.4 (95% CI 2.3-2.5) years. The ages of sexual characteristics development in overweight and obese groups were earlier than that in normal and thin groups. The ages of B2 in thin, normal, overweight, and obese groups were 10.0 (95% CI 9.5-10.6), 9.3 (95% CI 9.2-9.4), and 8.6 (95% CI 8.4-8.7) years, respectively. The age of menarche in thin, normal, overweight, and obese groups were 13.1 (95% CI 12.4-13.7), 11.6 (95% CI 11.4-11.7), and 11.1 (95% CI 11.0-11.2) years, respectively. The interval ages between B2 and B5 and between P2 and P5 was 4.5 and 4.1 years, respectively in the overweight and obese groups, and those in normal group and thin group was 4.7 and 4.5 years, 4.6 and 4.7 years, respectively. Conclusions:The ages of sexual characteristics development and menarche tend in Tongzhou District of Beijing to be earlier than that being reported of Beijing's survey 20 years ago. Girls characterized as overweight and obese not only start puberty at an earlier age than girls of normal weight, but also have a shorter developmental process.

Purpose: The risk stratification of pediatric anaplastic large cell lymphoma (ALCL) has not been standardized. In this study, new risk factors were included to establish a new risk stratification system for ALCL, and its feasibility in clinical practice was explored. Materials and Methods: On the basis of the non-Hodgkin’s lymphoma Berlin–Frankfurt–Munster 95 (NHL-BFM-95) protocol, patients with minimal disseminated disease (MDD), high-risk tumor site (multiple bone, skin, liver, and lung involvement), and small cell/lymphohistiocytic (SC/LH) pathological subtype were enrolled in risk stratification. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL). Results: A total of 136 patients were enrolled in this study. The median age was 8.8 years. The 3-year event-free survival (EFS) and overall survival of the entire cohort were 77.7% (95% confidence interval [CI], 69.0% to 83.9%) and 92.3% (95% CI, 86.1% to 95.8%), respectively. The 3-year EFS rates of low-risk group (R1), intermediate-risk group (R2), and high-risk group (R3) patients were 100%, 89.5% (95% CI, 76.5% to 95.5%), and 67.9% (95% CI, 55.4% to 77.6%), respectively. The prognosis of patients with MDD (+), stage IV cancer, SC/LH lymphoma, and high-risk sites was poor, and the 3-year EFS rates were 45.3% (95% CI, 68.6% to 19.0%), 65.7% (95% CI, 47.6% to 78.9%), 55.7% (95% CI, 26.2% to 77.5%), and 70.7% (95% CI, 48.6% to 84.6%), respectively. At the end of follow-up, one of the five patients who received maintenance therapy with VBL relapsed, and seven patients receiving anaplastic lymphoma kinase inhibitor maintenance therapy did not experience relapse. Conclusion This study has confirmed the poor prognostic of MDD (+), high-risk site and SC/LH, but patients with SC/LH lymphoma and MDD (+) at diagnosis still need to receive better treatment (ClinicalTrials.gov number, NCT03971305).

Postzygotic mutations are acquired in normal tissues throughout an individual's lifetime and hold clues for identifying mutagenic factors.Here,we investigated postzygotic mutation spectra of healthy individuals using optimized ultra-deep exome sequencing of the time-series samples from the same volunteer as well as the samples from different individuals.In blood,sperm,and muscle cells,we resolved three common types of mutational signatures.Signatures A and B represent clock-like mutational processes,and the polymorphisms of epigenetic regulation genes influence the pro-portion of signature B in mutation profiles.Notably,signature C,characterized by C＞T transitions at GpCpN sites,tends to be a feature of diverse normal tissues.Mutations of this type are likely to occur early during embryonic development,supported by their relatively high allelic frequencies,presence in multiple tissues,and decrease in occurrence with age.Almost none of the public datasets for tumors feature this signature,except for 19.6％of samples of clear cell renal cell carcinoma with increased activation of the hypoxia-inducible factor 1(HIF-1)signaling pathway.Moreover,the accumulation of signature C in the mutation profile was accelerated in a human embryonic stem cell line with drug-induced activation of HIF-1α.Thus,embryonic hypoxia may explain this novel signature across multiple normal tissues.Our study suggests that hypoxic condition in an early stage of embryonic development is a crucial factor inducing C＞T transitions at GpCpN sites;and indi-viduals'genetic background may also influence their postzygotic mutation profiles.

Objective:To examine the concordance between bioelectrical impedance analysis (BIA) and dual energy X-ray absorptiometry (DXA) in determining body composition of children aged between 3 and 6.Methods:A total of 230 children aged 3-6 from a kindergarten in Jinnan, Tianjin were enrolled in this study from November 16 th to December 8 th, 2017.The soft lean mass (SLM) and the body fat mass (BFM) of the children were measured by using BIA and DXA.The children were divided into several groups by sex, age and body mass index (BMI). The consistency of the body composition results between BIA and DXA in each group was analyzed. Results:The SLM and BFM assessed by BIA were significantly correlated with those measured by DXA ( R2=0.951, 0.947, all P<0.001). The Bland-Altman plots suggested that the SLM measured by BIA was 0.70 kg(95% CI: -1.78-0.38) higher in that by DXA, and the BFM measured by BIA was 1.36 kg (95% CI: 0.19-2.52) lower in that by DXA.A similar trend could be seen in children stratified by age, sex and BMI.The SLM of boys and girls measured by BIA was 0.57 kg (95% CI: -1.63-0.49) and 0.84 kg (95% CI: -1.88-0.20) higher than those by DXA, respectively.The BFM of boys and girls measured by BIA was 1.23 kg (95% CI: 0.11-2.36) and 1.49 kg (95% CI: 0.34-2.64) lower than that by DXA, respectively.The SLM measured by BIA was 0.67 kg (95% CI: -1.74-0.41), 0.76 kg (95% CI: -1.65-0.13), 0.69 kg (95% CI: -1.85-0.47) and 0.67 kg (95% CI: -1.75-0.41) higher than those by DXA in the 3 to 6-year-old groups, respectively.The BFM measured BIA was 1.09 kg (95% CI: -0.12-2.30), 1.44 kg (95% CI: 0.60-2.28), 1.39 kg (95% CI: 0.15-2.64)and 1.43 kg(95% CI: 0.38-2.48)lower than that by DXA in the 3 to 6-year-old groups, respectively.Moreover, the smallest difference of the SLM and BFM were observed between BIA and DXA in the obese child.The SLM deviation between BIA and DXA was estimated within ± 0.39 kg and the BFM deviation was within ± 0.93 kg. Conclusions:There is high consistency between BIA and DXA in the assessment of the SLM and BFM of children aged from 3 to 6.

Objective:To summarize the efficiency and long-term outcomes of limited-stage Hodgkin lymphoma in children and adolescents with ABVD therapy and determined whether omitting radiotherapy for a low-risk patient enabled the achievement of complete response (CR) after chemotherapy.Methods:We retrospectively analyzed data from 13 y (2004-2016) from patients aged ≤18 y with limited-stage HL admitted to the Sun Yat-sen University Cancer Center. Patients received treatment with ABVD chemotherapy alone or ABVD chemotherapy followed by low-dose involved field radiotherapy.Results:Total 85 subjects were eligible for study inclusion; the median age was 12 (3-18) y; 66 (77.6%) were men, 80 (94.1%) had stage-II disease, 56 (65.9%) were at low-risk, and the median follow-up duration was 72 (8-196) months; 12 relapsed, 2 had secondary neoplasm, and 2 died. The 5-year event free survival (EFS) was (85.6±3.8) %, and the overall survival (OS) was 100%. The 5-year EFS and OS was (89.1±4.2) % and 100%, respectively, for the low-risk cohort and (79.3±7.5) % and 100%, respectively for the intermediate-risk cohort. Among the 39 low-risk patients who achieved CR after chemotherapy, 15 received treatment with chemotherapy followed by LD-IFRT. In the exploratory subset analysis, the low-risk cohort who achieved CR after chemotherapy, the 5-year EFS for comparing ABVD alone with chemotherapy followed by LD-IFRT was (87.0±7.0) % versus 100% ( P=0.506) , and the OS was 100% for both the groups. Conclusions:Our retrospective analysis showed excellent survival of limited-stage HL patients with ABVD therapy. For patients who achieving CR after chemotherapy with low-risk HL, received chemotherapy followed by LD-IFRT does not improve 5-year OS and EFS. The use of risk- and response-based stratification may facilitate the development of effective and less toxic protocols.

Objective:To explore the effect of preoperative visit with Child Life mode in the management of preschool children′s anxiety.Methods:A total of 80 cases of children of pre-school age who were operated from September 2018 to February 2019 were selected by cluster sampling method. The children from September to November 2018 who were given the traditional mode of preoperative visit were set as the control group. Children from December 2018 to February 2019 who were given preoperative visit with Child Life mode were set as the experimental group. The Modified Yale Perioperative Anxiety Scale (m-YPAS) scores, Induction Compliance Checklist (ICC) scores and Pediatric Anesthesia Emergence Delirium (PAED) scores were compared between the two groups at 4 different perioperative time points (6 hours after admission, waiting for surgery, induction of anesthesia, and 6 hours after surgery).Results:Totally three patients were suspended from the study due to surgery. A total of 77 children were studied, 38 in the experimental group and 39 in the control group. The score of m-YPAS in the experimental group was significantly lower than that of the control group(17.57±16.64 vs. 42.04±27.46, t value was 3.85, P<0.01) while waiting for surgery. The score of m-YPAS in the experimental group was significantly lower than control group (31.64±27.95 vs. 62.22±25.13, t value was 4.26, P<0.01) at the anesthesia induction. Compare with the control group, the score of m-YPAS in the experimental group showed no significant difference at both time of 6 hours after admission (18.19±17.83 vs.19.85±17.92, t value was 0.87, P>0.05) and 6 hours after surgery(35.61±15.70 vs. 41.81±17.97, t value was 1.37, P>0.05). Compare with the control group the score of ICC in the experimental group was significantly decreased (1.40±1.39 vs. 3.78±2.60, t value was 4.88, P<0.01) at the anesthesia induction. The score of PEAD in the experimental group and control group, there was no significant difference between the two groups (7.18±2.45 vs. 7.85±3.05, t value was 0.91, P>0.05). Conclusions:Compared with the traditional preoperative visit mode, the preoperative visit mode of Child Life can effectively relieve the anxiety of preschool children undergoing general anesthesia surgery while they are waiting for surgery and during anesthesia induction, and can improve the degree of cooperation during anesthesia induction.

OBJECTIVE: To explore the effect of small interfering RNA (siRNA)-mediated CEP55 gene silencing on the proliferation of mouse spermatogonia. METHODS: Six patients with azoospermia diagnosed to have maturation arrest (3 cases) or normal spermatogenesis (3 cases) based on testicular biopsy between January 1 and December 31, 2017 in our center were examined for differential proteins in the testicular tissue using isobaric tags for relative and absolute quantitation (iTRAQ), and CEP55 was found to differentially expressed between the two groups of patients. We constructed a CEP55 siRNA for transfection in mouse spermatogonia and examined the inhibitory effects on CEP55 expressions using Western blotting and qPCR. The effect of CEP55 gene silencing on the proliferation of mouse spermatogonia was evaluated with CCK8 assay. RESULTS: In the testicular tissues from the 6 patients with azoospermia, iTRAQ combined with LC/MS/MS analysis identified over two hundred differentially expressed proteins, among which CEP55 showed the most significant differential expression between the patients with maturation arrest and those with normal spermatogenesis. The cell transfection experiment showed that compared with the cells transfected with the vehicle or the negative control sequence, the mouse spermatogonia transfected with CEP55 siRNA showed significantly lowered expressions of CEP55 mRNA and protein ( < 0.05) and significantly decreased proliferation rate as shown by CCK8 assay ( < 0.05). CONCLUSIONS CEP55 may play a key role in spermatogenesis and may serve as a potential therapeutic target for non-obstructive azoospermia with maturation arrest.
Animals ; Azoospermia ; congenital ; genetics ; Cell Cycle Proteins ; genetics ; Gene Silencing ; Humans ; Male ; Mice ; Nuclear Proteins ; genetics ; Spermatogenesis ; Spermatogonia ; Tandem Mass Spectrometry ; Transfection

Diffuse intrinsic pontine glioma (DIPG) is the main cause of brain tumor-related death among children. Until now, there is still a lack of effective therapy with prolonged overall survival for this disease. A typical strategy for preclinical cancer research is to find out the molecular differences between tumor tissue and para-tumor normal tissue, in order to identify potential therapeutic targets. Unfortunately, it is impossible to obtain normal tissue for DIPG because of the vital functions of the pons. Here we report the human fetal hindbrain-derived neural progenitor cells (pontine progenitor cells, PPCs) as normal control cells for DIPG. The PPCs not only harbored similar cell biological and molecular signatures as DIPG glioma stem cells, but also had the potential to be immortalized by the DIPG-specific mutation H3K27M in vitro. These findings provide researchers with a candidate normal control and a potential medicine carrier for preclinical research on DIPG.
Animals ; Brain Stem Neoplasms ; genetics ; metabolism ; pathology ; Cell Line, Tumor ; Cellular Senescence ; Female ; Glioma ; genetics ; metabolism ; pathology ; Histones ; genetics ; Humans ; Mice, Inbred NOD ; Mice, SCID ; Neoplasm Transplantation ; Neoplastic Stem Cells ; drug effects ; metabolism ; pathology ; Neural Stem Cells ; drug effects ; metabolism ; pathology ; Pons ; embryology ; metabolism ; pathology ; Primary Cell Culture

OBJECTIVE： To investigate the situation and existing problems of the naming of commercially available Chinese patent medicine （CPM） in China， and to put forward the improvement suggestions. METHODS： Announced in Dec. 31， 2017 by CFDA， there were totally 169 601 kinds of national coded drugs. Total of 35 513 kinds of CPM with drug approval number “Z” and 68 kinds of imported CPM with importing registration certificates “Z” was screened by using Excel 2013 software. Based on Naming Technical Guidelines for Generic Name of Chinese Patent Medicines （hereinafter referred to as the Guidelines）， the unqualified situation of domestic CPM were summarized， and unqualified domestic Chinese patent medicine were analyzed statistically in respects of dosage form， special population medication and area. RESULTS： There were 5 091 kinds of drugs named nonconformity with the “Guidelines”， accounting for 14.34％ of domestic CPM. The problems of naming mainly focused on exaggerated naming （1 723 kinds， 33.84％）， dosage form un-located after naming （1 118 kinds， 21.96％）， naming by endangered protected animals and plants （851 kinds， 16.72％）， naming by pharmacology and other related terms （848 kinds， 16.66％）. Names emboding “traditional cultural features” （1 324 kinds， 4.35％） took the lower proportion. Top 5 dosage forms of domestic CPM with unqualified naming were tablets （1 203 kinds， 23.63％）， capsules （821 kinds， 16.13％）， mixture （802 kinds， 15.75％）， pills （706 kinds， 13.87％）， granules （448 kinds， 8.80％）. The problems of special population medication for domestic CPM with unqualified naming concentrated on children’s medication （608 kinds， 11.94％）. The main manufacturers of domestic CPM with unqualified naming came from Guangdong （613 kinds， 12.04％）， Guangxi Zhuang Autonomous Region （371 kinds， 7.29％）， Jilin （265 kinds， 5.21％）， Shaanxi （245 kinds， 4.81％） and Beijing （233 kinds， 4.58％）. CONCLUSIONS： There are many problems in naming of commercially available domestic CPM in China， mainly reflecting as naming type， dosage form， pediatric drugs， etc. There are fewer names emboding “traditional cultural features”. It is suggested that we should further standardize the naming of CPM by strengthening and perfecting the management and examination system of CPM， highlighting the naming principles of Chinese traditional culture and protecting traditional classical brands.

OBJECTIVETo evaluate the reliability and validity of Professional Quality of Life Scale (ProQOL-30, 4th version, 30 items) among government staff in the Wenchuan earthquake-stricken areas

METHODSA total of 1,175 members of government staff in the Wenchuan earthquake-stricken areas were selected by convenience sampling and required to complete the ProQOL and Self-Reporting Questionnair (SRQ). The reliability and validity of the scale was evaluated by correlation analysis, t-test, and confirmatory factor analysis.

RESULTSItem-total correlation coefficients of the three subscales were 0.590 - 0.752, 0.389 - 0.603, and 0.340 - 0.647, respectively (P<0.05), and the average coefficients were 0.672, 0.482, and 0.555 respectively (P<0.05). The Cronbach's α coefficients of the three subscales were 0.864, 0.569, and 0.742 respectively, and the split-half reliabilities were 0.829, 0.490, and 0.677, respectively. P value was 0.88 in thE chi-square test of confirmatory factor analysis model. Goodness-of-fit indices of ProQOL-30 included GFI=0.895 NFI=0.856, CFI=0.895, RMSEA=0.063, and AGFI=0.912. For the ProQOL-28 as an optimized version o ProQOL-30, the Cronbach's a coefficients for burnout and trauma/compassion fatigue increased to 0.616 and 0.757, respectively. P value was 0.91 in the chi-square test of confirmatory factor analysis model test. Goodness-of-fit indices of ProQOL-28 were GFI =0.913, AGFI =0.924, NFI =0.900, CFI =0.913, and RMSEA =0.031 CONCLUSION: ProQOL-28 has good reliability and validity among government staff in the earthquake-stricker areas in China.