Objective To analyze the influencing factors for the knowledge level,cognition willingness and atti-tude of health care workers(HCWs)towards monkeypox.Methods A cross-sectional survey was conducted,from January to April 2023,700 HCWs were randomly selected from a tertiary hospital in Shanghai.Based on a self-de-signed questionnaire,the survey was conducted anonymously through the online platform"Wenjuanxing".Results A total of 612 questionnaires were collected,581 were valid,with a response rate of 87.43％and an effective rate of 94.93％.The mean score of the questionnaire was(128.80±27.70)points,with a score rate of 58.54％.Multiple linear regression analysis showed that there were statistically significant differences in the knowledge level of mon-keypox among HCWs of different ages,occupations,departments,educational levels,years of working experience,professional titles,initiative cognition on monkeypox,and participation in monkeypox-related lectures organized by the units(all P＜0.05).Conclusion HCWs'cognition on monkeypox is at a moderate level.The training of HC-Ws on monkeypox knowledge should be strengthened to improve the emergency response capabilities during mon-keypox outbreaks.

BACKGROUND: The benefits of healthy lifestyles are well recognized. However, the extent to which improving unhealthy lifestyles reduces cardiovascular disease (CVD) risk needs to be discussed. We evaluated the impact of lifestyle improvement on CVD incidence using data from the China-PAR project (Prediction for Atherosclerotic Cardiovascular Disease Risk in China). METHODS: A total of 12,588 participants free of CVD were followed up for three visits after the baseline examination. Changes in four lifestyle factors (LFs) (smoking, diet, physical activity, and alcohol consumption) were assessed through questionnaires from the baseline to the first follow-up visit. Cox proportional hazard models were used to estimate hazard ratios (HRs) and corresponding 95% confidence intervals (CIs). The risk advancement periods (RAPs: the age difference between exposed and unexposed participants reaching the same incident CVD risk) and population-attributable risk percentage (PAR%) were also calculated. RESULTS: A total of 909 incident CVD cases occurred over a median follow-up of 11.14 years. Compared with maintaining 0-1 healthy LFs, maintaining 3-4 healthy LFs was associated with a 40% risk reduction of incident CVD (HR = 0.60, 95% CI: 0.45-0.79) and delayed CVD risk by 6.31 years (RAP: -6.31 [-9.92, -2.70] years). The PAR% of maintaining 3-4 unhealthy LFs was 22.0% compared to maintaining 0-1 unhealthy LFs. Besides, compared with maintaining two healthy LFs, improving healthy LFs from 2 to 3-4 was associated with a 23% lower risk of CVD (HR = 0.77, 95% CI: 0.60-0.98). CONCLUSIONS Long-term sustenance of healthy lifestyles or improving unhealthy lifestyles can reduce and delay CVD risk.

Objective: To analyze the clinical characteristics of patients with Mucopolysaccharidosis ⅣA (MPS ⅣA). Methods: A retrospective study was conducted on 111 patients with MPS ⅣA in Xinhua Hospital of Shanghai Jiao Tong University School of Medcine from December 2008 to August 2020, confirmed by enzyme activity and genetic testing. General situation, clinical manifestations and enzyme activity test results were analyzed. According to the clinical manifestations, it can be divided into severe, intermediate and mild group. The independent sample t test was used to compare the birth body length and weight of children with that of normal boys and girls, and group comparisons of enzyme activities were evaluated by median test. Results: One hundred and eleven unrelated patients, 69 males and 42 females, were classified into 3 subtypes: severe (n=85), intermediate (n=14), and mild (n=12). The age at symptom onset were 1.6 (1.0, 3.0) years, and at diagnosis were 4.3 (2.8, 7.8) years. Skeletal manifestations were observed in all patients and consisted mainly of pectus carinatum (96/111, 86.5%), motor dysfunction (78/111, 70.3%), spinal deformity (71/111, 64.0%), growth retardation (64/111, 57.7%), joint laxity (63/111, 56.8%) and genu valgum (62/111, 55.9%). Eighty-eight patients (88/111, 79.3%) with MPS ⅣA were also along with non-skeletal manifestations, mainly including snoring (38/111, 34.2%), coarse faces (34/111, 30.6%), and visual impairment (26/111, 23.4%). The most common skeletal manifestation was pectus carinatum (79 cases), and non-skeletal manifestation was snoring (30 cases) and coarse faces (30 cases) in severe patients, pectus carinatum (13 cases) and snoring (5 cases) in intermediate type, motor dysfunction (11 cases) and snoring (3 cases) and visual impairment (3 cases) in mild patients. The height and weight of severe patients began to fall below -2 s at 2-<5 years and 5-<7 years, respectively. At the age of 10-<15 years, the standard deviation score of the height of severe patients reached (-6.2±1.6) s in males and (-6.4±1.2) s in females, and the score of weight got (-3.0±1.1) s in males and (-3.5±0.5) s in females. The height of intermediate patients began to fall below -2 s at the age of 7-<10 years, and the standard deviation score of height were -4.6 s and -3.6 s in 2 males, and -4.6 s and -3.8 s in 2 females at the age of 10-<15 years. The weight remained within -2 s in 72.0% (18/25) of intermediate patients compared to age-matched healthy children. In the mild patients with MPS ⅣA, the mean standard deviation score of height and weight was within -2 s. The enzyme activities of mild patients (2.02 (1.05, 8.20) nmol/(17 h·mg)) were both significantly higher than that of intermediate (0.57 (0.47, 0.94) nmol/(17 h·mg)) and severe (0.22 (0, 0.59) nmol/(17 h·mg)) patients (Z=9.91, 13.98, P=0.005, 0.001), and the enzyme activity of intermediate patients was significantly higher than that of severe patients (Z=8.56, P=0.010). Conclusions: The clinical manifestations of MPS ⅣA are charactered by pectus carinatum, motor function impairment, spinal deformity and growth retardation. The clinical characteristics, growth rate and enzyme activity differ among the 3 subtypes of MPS ⅣA.
Male ; Child ; Female ; Humans ; Adolescent ; Mucopolysaccharidosis IV ; Pectus Carinatum ; Retrospective Studies ; Snoring ; China ; Mucopolysaccharidoses ; Growth Disorders ; Vision Disorders

Objective: To analyze the clinical and genetic characteristics of 33 children with congenital lipoid adrenal hyperplasia (CLAH) caused by StAR gene defects. Methods: The clinical, biochemical, genetic, and follow-up (until December 2021) data of 33 children diagnosed with CLAH from 2006 to 2021 were retrospectively analyzed in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine. Results: Of the 33 children with CLAH, 17 had a karyotype of 46, XX and 16 had a karyotype of 46, XY; 31 were female and 2 were male by social gender. Classic type and non-classic type were found in 30 and 3 children respectively. The age at diagnosis was 9.0 (3.0, 34.5) months. All the 30 cases with classic CLAH presented within the first year of life with skin hyperpigmentation (28 cases, 93%), vomiting and(or) diarrhea (19 cases, 63%), no increase in body weight (8 cases, 27%), elevated adrenocorticotropic hormone levels (21cases (70%)>275 pmol/L), decreased cortisol levels (47 (31,126) nmol/L), hyponatremia ((126±13) mmol/L), hyperkalemia ((5.7±1.1) mmol/L), and normal 17α-hydroxyprogesterone levels (30 cases, 100%). All these with classic CLAH exhibited female external genitalia. Three children with non-classic CLAH (including 2 cases of 46, XY and 1 case of 46, XX) also showed signs and symptoms of adrenal insufficiency, but 2 of them had an age of onset later than 1 year of age, including 1 case of 46, XY with male external genitalia and 1 case of 46, XX with female external genitalia. The other 46, XY patient with non-classic CLAH presented with adrenal insufficiency at 2 months of age, showing micropenis and hypospadias. In the 17 females with 46, XX, 4 older than 10 years of age showed spontaneous pubertal development. A total of 25 StAR gene pathogenic variants were identified in 33 patients, with p.Q258* (18/66, 27%), p.K236Tfs*47 (8/66, 12%) and p.Q77* (6/66, 9%) being the common variantion. Six novel variants were found, including c.358T>G, c.713_714del, c.125del, c.745-1G>A, c.179-2A>C, and exon 1 deletion. Conclusions: Patients with classic CLAH typically present with signs and symptoms of primary adrenal insufficiency in the early infancy period and female external genitalia. p.Q258*, p.K236Tfs*47 and p.Q77* are common variants in CLAH patients.
Adrenal Hyperplasia, Congenital/genetics* ; Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Child, Preschool ; China ; Disorder of Sex Development, 46,XY ; Female ; Humans ; Hydrocortisone ; Hydroxyprogesterones ; Hyperplasia ; Infant ; Male ; Mutation ; Phosphoproteins/genetics* ; Retrospective Studies

This study aimed to propose an operational definition of late-onset hypogonadism (LOH) that incorporates both clinical symptoms and serum testosterone measurements to evaluate the prevalence of LOH in aging males in China. A population-based sample of 6296 men aged 40 years-79 years old was enrolled from six representative provinces in China. Serum total testosterone (TT), sex hormone-binding globulin (SHBG), and luteinizing hormone (LH) were measured and free testosterone (cFT) was calculated. The Aging Males' Symptoms (AMS) scale was used to evaluate the LOH symptoms. Finally, 5078 men were included in this analysis. The TT levels did not decrease with age (P = 0.59), and had no relationship with AMS symptoms (P = 0.87 for AMS total score, P = 0.74 for ≥ 3 sexual symptoms). The cFT levels decreased significantly with age (P < 0.01) and showed a negative association with the presence of ≥ 3 sexual symptoms (P = 0.03). The overall estimated prevalence of LOH was 7.8% (395/5078) if a cFT level <210 pmol l

BACKGROUND: The association of milk intake with cardiovascular disease (CVD) and cause-specific mortality remained controversial and evidence among the Chinese population was limited. We aimed to study the relationship between milk intake and CVDs among general Chinese adults. METHODS: A total of 104,957 participants received questionnaire survey. Results of physical examination such as anthropometric measurements and biochemical tests during 2007 to 2008, demographic data and their information on milk intake were collected through standardized questionnaires. Cox proportional hazard regression models were used to calculate hazard ratios (HRs) and their corresponding 95% confidence intervals (CIs) of CVD incidence, cause-specific mortality and all-cause mortality related to milk intake. Restricted cubic splines (RCSs) were applied to examine dose-response associations. RESULTS: Among the 91,757 participants with a median follow-up period of 5.8 years, we documented 3877 CVD cases and 4091 all-cause deaths. Compared with participants who never consumed milk, the multivariate-adjusted HRs (95% CIs) of CVD incidence for 1 to 150 g/day, 151 to 299 g/day, and ≥300 g/day were 0.94 (0.86-1.03) (P > 0.05), 0.77 (0.66-0.89) (P < 0.05), and 0.59 (0.40-0.89) (P < 0.05), respectively; each 100 g increase of daily milk intake was associated with 11% lower risk of CVD incidence (HR, 0.89; 95% CI: 0.85-0.94; P < 0.001), and 11% lower risk of CVD mortality (HR, 0.89; 95% CI: 0.82-0.97; P = 0.008) after adjustment for age, sex, residential area, geographic region, education level, family history of CVD, smoking, alcohol drinking, physical activity level, body mass index, and healthy diet status (ideal or not). RCS analyses also showed a linear dose-response relationship with CVD (P for overall significance of the curve <0.001; P for non-linearity = 0.979; P for linearity <0.001) and stroke (P for overall significance of the curve = 0.010; P for non-linearity = 0.998; P for linearity = 0.002) incidence, and CVD mortality (P for overall significance of the curve = 0.045; P for non-linearity = 0.768; P for linearity = 0.014) within the current range of daily milk intake. CONCLUSIONS Daily milk intake was associated with lower risk of CVD incidence and mortality in a linear inverse relationship. The findings provide new evidence for dietary recommendations in CVD prevention among Chinese adults and people with similar dietary pattern in other countries.

This study is a randomized controlled trial of Reyanning Mixture in the treatment of acute tonsillitis. According to the ratio of 1∶1∶1, a total of 144 patients were randomly divided into Reyanning Mixture group(RYN), Reyanning Mixture+Amoxicillin Capsules group(RYN+Amoxil) and Amoxicillin Capsules group(Amoxil), with 48 cases in each group, in order to evaluate the efficacy and safety of RYN alone or combined with Amoxil in the treatment of acute tonsillitis, and provided high-quality evidences for treatment of infectious diseases with traditional Chinese medicine and reduced use of antibiotics. The dosage of RYN was 20 mL, 3 times a day, 100 mL/bottle, oral for 7 days, and Amoxil dosage was 0.5 g, 3 times a day, 0.5 g×12 tablets/plate, oral for 7 days. A total of 144 cases were included, 3 cases were excluded(1 case was mistakenly included, 2 cases did not take drugs after inclu-ded), and a total of 141 cases were included in the full analysis set(FAS). The results showed statistical differences in the recovery time of the disease, the disappearance rate of fever on the 3 rd day and the disappearance rate of tonsillar redness and swelling between RYN and Amoxil. There were statistical differences in the cure rate of disease, recovery time of disease, body temperature recovery time, fever disappearance rate on the 3 rd day, pharynx swelling and pain disappearance rate and tonsil swelling disappearance rate between the RYN+Amoxil and Amoxil, but with no significant difference in the above aspects compared with RYN. The DDD of antibiotic use in RYN+Amoxil was significantly lower than that in Amoxil(P<0.01). According to the findings, when RYN was used alone in the treatment of acute tonsillitis, it was superior to Amoxil in time of recovery, short-term improvement of fever and redness and swelling of tonsil. Compared with RYN+Amoxil, there was no difference in cure rate of disease, recovery time of disease, body temperature recovery time, short-term improvement of fever, swelling of pharynx and swelling of tonsil, with a better efficacy than Amoxil. The clinical effect of RYN was similar to that of combined Amoxil in the treatment of acute tonsillitis, and RYN was superior to Amoxil in the time of recovery, short-term improvement of fever and redness and swelling of tonsil, with no adverse event or adverse reaction. RYN+Amoxil can significantly reduce the DDD value of antibiotics in the treatment of acute tonsillitis, with significant clinical advantages over Amoxil.
Anti-Bacterial Agents ; therapeutic use ; Double-Blind Method ; Drugs, Chinese Herbal ; Fever ; drug therapy ; Humans ; Tonsillitis ; drug therapy

OBJECTIVE: To investigate the epidemiological characteristics, phenotype, genotype, and prognosis of medium-chain acyl-CoA dehydrogenase deficiency (MCADD) in the Chinese population. METHODS: A retrospective analysis was performed for the clinical data of the neonates who underwent screening with high-performance liquid chromatography-tandem mass spectrometry from January 2009 to June 2018 and were diagnosed with MCADD by gene detection. RESULTS: A total of 2 674 835 neonates underwent neonatal screening, among whom 12 were diagnosed with MCADD. Gene detection was performed for 10 neonates with MCADD and found 13 mutation types at 16 mutation sites of the ACADM gene, among which there were 7 reported mutations (p.T150Rfs*4, p.M1V, p.R206C, p.R294T, p.G310R, p.M328V, and p.G362E), 5 novel mutations (p.N194D, p.A324P, p.N366S, c.118+3A>G, and c.387+1del G), and 1 exon 11 deletion; p.T150Rfs*4 was the most common mutation (4/16). The detection rate of mutation sites in the ACADM gene was 80%. No phenotype-genotype correlation was observed. Dietary guidance and symptomatic treatment were given after confirmed diagnosis. No acute metabolic imbalance was observed within 4-82 months of follow-up. All neonates had good prognosis except one who had brain dysplasia. CONCLUSIONS MCADD is relatively rare in southern China, and p.T150Rfs*4 is a common mutation in the Chinese population. Cases with positive screening results should be evaluated by octanoylcarnitine C8 value and gene detection.
Acyl-CoA Dehydrogenase ; deficiency ; Carnitine ; China ; Follow-Up Studies ; Humans ; Infant, Newborn ; Lipid Metabolism, Inborn Errors ; Mutation ; Neonatal Screening ; Retrospective Studies

Objective To investigate the occurrence and reporting of sharp injuries among health care workers (HCWs)at all levels of hospitals in Xuzhou City,provide evidences for formulating protective measures against sharp injuries and improving the reporting system.Methods From July to August 2016,13 hospitals in Xuzhou City were randomly selected by multi-stage stratified random sampling method,the general information,occurrence of sharp injuries,and reporting situation was performed questionnaire survey.Results A total of 2 694 valid question-naires were collected,incidence,case incidence,and reporting rate of sharp injuries were 10.32%,12.84%,and 30.64% respectively.Case incidence of sharp injuries among HCWs in primary,secondary,and tertiary hospitals were 44.83%,11.53%,and 12.52% respectively,case incidences of sharp injuries in different levels of hospitals were significantly different (χ2＝55.148,P＜0.001).The main opportunity for sharp injuries was when HCWs re-turned needle cap (79 cases,22.83%),the main device involving sharp injuries was hollow-bore needle (297 cases, 85.84%).Incidences of sharp injuries among HCWs receiving different training were significantly different (χ2＝66.760,P＜0.001).Conclusion Current situation of sharp injuries among HCWs in this region is not optimistic, there are some problems such as poor training efficacy,low reporting rate and low use rate of safety devices,effec-tive measures should be taken to establish an effective monitoring and tracking system for sharp injuries,so as to re-duce the occurrence of sharp injuries.

Objectives: To explore the trends of serum total cholesterol (TC) among Chinese adult cohorts from 1998 to 2015. Methods: The subjects of this study came from China Multicenter Collaborative Study of Cardiovascular Epidemiology and International Collaborative Study of Cardiovascular Disease in Asia. The baseline investigations were completed during 1998-2001, and the two follow-ups were conducted during 2007-2008 and 2013-2015, respectively. A total of 9 477 participants aged 35-74 years with complete serum TC data from three surveys were included in this study. Analysis on variance of data regarding repeated measurements were used to investigate the variation of serum TC in different subgroups. The serum TC levels were represented by mean (standard errors). Results: During 1998-2015, the mean level of serum TC of all participants significantly elevated from 4.81(0.01) mmol/L to 4.91(0.01) mmol/L, and to 4.98(0.01) mmol/L, growth rates of which were 2.08% and 3.53% in the first and second follow-up, respectively (Ptrend<0.001), especially for females. The fastest growth was noted in the group of young women aged 35-45 years over time, with a growth rate of 11.43%. No such a significant change in the male group during follow-up. The serum TC level and its growth rate in urban residents was significantly higher than those in rural residents. Conclusions: Longitudinal data demonstrated that the mean serum TC level among Chinese adults was continuously increased during 1998~2015. Prevention strategy for hypercholesterolemia should focus more on young women and urban residents.