Objective: To analyze the clinical characteristics of children with Burkitt lymphoma (BL) and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen. Methods: Clinical features of 436 BL patients who were ≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively. Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared. Survival analysis was performed by Kaplan-Meier method, and Cox regression was used to identify the prognostic factors. Results: Among 436 patients, there were 368 (84.4%) males and 68 (15.6%) females, the age of disease onset was 6.0 (4.0, 9.0) years old. According to the St. Jude staging system, there were 4 patients (0.9%) with stage Ⅰ, 30 patients (6.9%) with stage Ⅱ, 217 patients (49.8%) with stage Ⅲ, and 185 patients (42.4%) with stage Ⅳ. All patients were stratified into following risk groups: group A (n=1, 0.2%), group B1 (n=46, 10.6%), group B2 (n=19, 4.4%), group C1 (n=285, 65.4%), group C2 (n=85, 19.5%). Sixty-three patients (14.4%) were treated with chemotherapy only and 373 patients (85.6%) were treated with chemotherapy combined with rituximab. Twenty-one patients (4.8%) suffered from progressive disease, 3 patients (0.7%) relapsed, and 13 patients (3.0%) died of treatment-related complications. The follow-up time of all patients was 24.0 (13.0, 35.0) months, the 2-year event free survival (EFS) rate of all patients was (90.9±1.4) %. The 2-year EFS rates of group A, B1, B2, C1 and C2 were 100.0%, 100.0%, (94.7±5.1) %, (90.7±1.7) % and (85.9±4.0) %, respectively. The 2-year EFS rates was higher in group A, B1, and B2 than those in group C1 (χ²=4.16, P=0.041) and group C2 (χ²=7.21, P=0.007). The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were (79.3±5.1)% and (92.9±1.4)% (χ²=14.23, P<0.001) respectively. Multivariate analysis showed that stage Ⅳ (including leukemia stage), serum lactate dehydrogenase (LDH)>4-fold normal value, and with residual tumor in the mid-term evaluation were risk factors for poor prognosis (HR=1.38,1.23,8.52,95%CI 1.05-1.82,1.05-1.43,3.96-18.30). Conclusions: The CNCL-B-NHL-2017 regimen show significant effect in the treatment of pediatric BL. The combination of rituximab improve the efficacy further.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Burkitt Lymphoma/drug therapy* ; Child ; Disease-Free Survival ; Female ; Humans ; Lactate Dehydrogenases ; Lymphoma, B-Cell/drug therapy* ; Male ; Prognosis ; Retrospective Studies ; Rituximab/therapeutic use* ; Treatment Outcome

A total of 15 batches of the substance reference of Guizhi Jia Gegen Decoction(GZGGD) were prepared and the characteristic fingerprints of them were established. Furthermore, the similarity of the fingerprints and peak attributes were explored. The extraction rate, and the content and the transfer rate ranges of the index components, puerarin, paeoniflorin, liquiritin, and ammonium glycyrrhizate were determined for the analysis of the quality value transfer. The result demonstrated that the fingerprints of the 15 batches of the samples showed high similarity(>0.99). A total of 15 characteristic peaks were identified from the fingerprints, with 10 for Puerariae Lobatae Radix, 1 for Cinnamomi Ramulus, 2 for Paeoniae Radix Alba, and 2 for Glycyrrhizae Radix et Rhizoma. The content of puerarin was 11.05-18.35 mg·g~(-1) and the average transfer rate was 21.27%-39.49%. The corresponding figures were 7.95-10.90 mg·g~(-1) and 23.28%-43.23% for paeoniflorin, 3.25-4.95 mg·g~(-1) and 32.31%-61.27% for ammonium glycyrrhizate, and 3.65-5.80 mg·g~(-1) and 14.57%-27.05% for liquiritin. The extraction rate of the 15 batches of samples was in the range of 16.85%-21.78%. In this paper, the quality value transfer of the substance reference of GZGGD was analyzed based on characteristic fingerprint, content of index components, and the extraction rate. This study is expected to lay a basis for the quality control and further development of GZGGD.
Ammonium Compounds ; Benchmarking ; Chromatography, High Pressure Liquid ; Drugs, Chinese Herbal ; Paeonia

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/genetics* ; Child ; China/epidemiology* ; Cryptorchidism/genetics* ; Disorders of Sex Development/genetics* ; Female ; Genital Diseases, Male ; Genotype ; Humans ; Hypospadias/genetics* ; Male ; Membrane Proteins/genetics* ; Penis/abnormalities* ; Phenotype ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics*

Objective: To explore the association between self-reported gingival bleeding and prevalent hypertension among adults in Zhejiang with a cross-sectional study. Methods: After excluding participants with self-reported, physician-diagnosed heart diseases, stroke, diabetes, cancer, and those who never or rarely brush their teeth at baseline study, 48 625 participants aged 30-79 in the China Kadoorie Biobank (CKB) study from Tongxiang, Zhejiang were included for the final analysis. Three multivariable logistic regression models were used to estimate the odds ratios (ORs) for the associations of self-reported gingival bleeding with prevalent hypertension. Results: The mean age of women was (51.2±9.2) years, and 42.9% of participants had prevalent hypertension. The percentage of self-reported frequent gingival bleeding was 6.56% (95%CI: 6.38%-6.75%), significantly higher among women (8.08%, 95%CI: 7.82%-8.35%) than among men (4.36%, 95%CI: 4.12%-4.60%) (P<0.001). After adjusting for socio-demographic factors, behavioral lifestyle, sleep duration, BMI, waist circumference, snoring, in comparison with men whose gingivae never or rarely bleed while brushing teeth, the odds ratio (95%CI) of hypertension for those with occasional, and frequent gingival bleeding were 1.04 (0.96-1.12) and 1.18 (1.02-1.37), respectively (trend P =0.038). The corresponding figures for women were 0.96 (0.91-1.02) and 0.95 (0.86-1.05), respectively (trend P=0.344). Conclusion: Frequent gingival bleeding was positively associated with prevalent hypertension among men.
Adult ; China/epidemiology* ; Cross-Sectional Studies ; Female ; Gingival Hemorrhage ; Humans ; Hypertension/epidemiology* ; Male ; Middle Aged ; Risk Factors ; Self Report

Objective To investigate the relationship between changes in protein tyrosine kinase 7 (PTK7) and receptor tyrosine kinase-like orphan receptor 2 ( ROR2) expression in the brainstem and the pathogenesis of amyotrophic lateral sclerosis (ALS). Methods Forty-four human superoxide dismutase 1( hSODl)-G93A mutant ALS transgenic mice were selected, and an equal number of wild-type littermates was used as control. The brainstems were isolated at da)' 70, day 95, day 108 and da)' 122 after birth, and the morphology of frypoglossal nucleus (12N) and nucleus of facial nerve(7N) neurons in the brainstem of the model mice were observed by Nissl staining. The mRNA and protein expression of PTK7 and ROR2 were detected by RT-PCR and Western blotting respectively, and the cellular localization and distribution of PTK7 and ROR2 in 12N and 7N were observed by immunofluorescence double-labeling technique. Results The result of Nissl staining showed that Nissl bodies in the neurons reduced distinctly with vacuolar degeneration of neurons, cell body atrophy and nuclear volume reduction in the 12N and 7N brainstems of ALS transgenic mice. RT-PCR result indicated that ROR2 and PTK7 mRNA level in the brainstem of ALS transgenic mice were up-regulated at da)' 70, da)' 95, day 108 and day 122 compared with wild-type littermates. Western blotting result showed that PTK7 protein was up-regulated at day 70, day 95, day 108 and day 122, ROR2 protein was up-regulated at day 70, day 95, day 108, and down-regulated at day 122 in the brainstem of ALS transgenic mice compared with wild-type littermates. Immunofluorescence result showed that ROR2/neuronal nuclei (NeuN)and PTK7/NeuN double positive cells, ROR2/glial fibrillary acidic protein (GFAP) and PTK7/GFAP double positive cells were observed in the 12N and 7N of the brainstem of ALS transgenic mice and wild-type mice, suggesting that ROR2 and PTK7 were expressed both in neurons and astrocytes. Conclusion PTK7 and ROR2 are abnormally expressed in the brainstem of ALS transgenic mice, which is closely related to the pathogenesis of ALS.

Objective To investigate the distribution and density of Culex mosquito populations and the resistance of Culex pipiens pallens to insecticides in Jiangsu Province in 2018 and 2019. Methods During the period from June to October in 2018 and 2019, six counties (districts, cities) were sampled in southern, northern and central Jiangsu Province as surveillance sites. The density of Culex mosquitoes was measured overnight using the light trapping technique. In addition, Culex pipiens pallens mosquitoes were collected from Hai’an of Nantong City and Yandu District of Yancheng City, central Jiangsu Province, and the sensitivity of female first filial generations to dichlorodiphenyltrichloroethane (DDT), malation, proposur, beta cypermethrin and deltamethrin was tested using the standard WHO insecticide susceptibility test assay. Results A total of 104 423 Culex mosquitoes were captured in six surveillance sites of Jiangsu Province in 2018 and 2019, and Culex quinquefasciatus (49.11%), Culex pipiens pallens (28.38%), and Culex tritaeniorhynchus (21.04%) were predominant species. The density of Culex mosquitoes started to increase since early June, peaked in July and tended to be low in late October. Culex pipiens pallens mosquitoes captured from Hai’an was susceptible to malation, while those from Yandu District were moderately resistant to malation. Culex pipiens pallens mosquitoes from both Yandu and Hai’an were moderately resistant to proposur, and were resistant to DDT, beta cypermethrin and deltamethrin. Conclusions Culex quinquefasciatus, Culex pipiens pallens and Culex tritaeniorhynchus are predominant Culex species in Jiangsu Province. Culex pipiens pallens is resistant to DT, beta cypermethrin and deltamethrin in central Jiangsu Province.

OBJECTIVE: To understand the relationship between health literacy and patient experience of outpatients in China, and to explore its mechanism. METHODS: The conceptual framework was developed based on Andersen's behavioral model of health services use and health literacy skills framework. An online cross-sectional survey was conducted with snowball sampling method, while the health literacy was measured by self-designed patient health literacy scale, and the patient experience was measured by the Chinese patient experience questionnaire for ambulatory care developed by Peking Union Medical College. And a structural equation model was built to explore the relationship between them and test the mechanism of health literacy influencing patient experience. RESULTS: A total of 2 773 subjects were investigated. The average score of health literacy was (90.72±12.90) points, accounting for 78.89% of the full score, and the dimension of seeking social support had the lowest score. The average score of overall rating of patient experience was (3.71±0.74) points, and the scores of each dimension of patient experience were between 3.56 and 3.80. The model fit indices of structural equation model for overall rating of patient experience among the outpatients were χ²/df=9.29 (χ²=4 107.27, df=442), root mean square error of approximation (RMSEA)=0.055 (< 0.06), comparative fit index (CFI)=0.926 (>0.90), Tucker-Lewis index (TLI)=0.918 (>0.90), standardized root mean square residual (SRMR)=0.061 (< 0.08), the model was acceptable. The variance in patient experience explained by the model was 0.108. The structural equation model analysis results showed that the overall rating of outpatient experience was directly affected by health literacy (β=0.263, P < 0.001), also indirectly affected by health literacy (β=0.012, P < 0.001). In other words, the overall rating increased by 0.275 units for each standard deviation increase of health literacy. Self-evaluated health status mediated the relationship between health literacy and the overall rating of outpatient experience. In terms of diffe-rent dimensions of patient experience, the standardized path coefficient of the total effect of health literacy on patient experience was as follows: Information guidance 0.337, humanistic care 0.319, communication with doctors 0.294, service efficiency 0.240, and hospital environment 0.173. CONCLUSION The patients with higher level of health literacy were more likely to have a better outpatient experience in China, and the information guidance experience and humanistic care experience were most affected by health literacy. And the communication and information utilization ability had the greatest influence on patient experience.
China ; Cross-Sectional Studies ; Health Literacy ; Humans ; Outpatients ; Patient Outcome Assessment ; Surveys and Questionnaires

Objective:To investigate constituents containing phenolic hydroxyl or carboxylic acid （excluding diarylheptanoids） from Curcumae Rhizoma and Sparganii Rhizoma herbal pair and single herb. Method:Multiple chromatographic separation techniques， including silica gel，MCI gel and Sephadex LH-20 gel， were employed to isolate and purify the compounds. Their structures were identified by means of the nuclear magnetic resonance （NMR），mass spectrometry （MS） and physicochemical properties. Constituents were quickly analyzed by UPLC-LTQ-Orbitrap-MSⁿ，and scanned in positive and negative ion modes. Result:These compounds were determined as trans-p-hydroxycinnamic acid（1），vanillic acid（2），protocatechuic acid（3），fumalic acid（4），succinic acid（5），succinic acid monomethyl ester（6），docosanoic acid（7），azelaic acid（8） and p-hydroxybenzaldehyde（9）. Forty compounds were speculated by comparing mass spectrometry data，retention times of some compounds and reference materials，including 14 phenols and 26 organic acids. Among the compounds of herbal pair，8 phenols and 22 organic acids in Sparganii Rhizoma as well as 11 phenols and 13 organic acids in Curcumae Rhizoma were identified. Cleavage pathways of main compounds were described. Conclusion:There are abundant phenols and organic acids in Curcumae Rhizoma and Sparganii Rhizoma herbal pair and single herb. The results enrich pharmacodynamic material basis of Curcumae Rhizoma and Sparganii Rhizoma herbal pair.