Objective:To analyze and summarize the clinical characteristics of mpox patients, thereby to rise clinicians′ awareness of severe mpox and provide a foundation for clinical diagnosis and treatment.Methods:The clinical data were retrospectively analyzed in 41 mpox patients treated at the Beijing You′an Hospital, Capital Medical University, from June to November 2023. Patients were categorized into mild and severe cases based on clinical manifestations, and laboratory results of the two groups were compared. Statistical analysis was performed using the Mann-Whitney U test. Results:The clinical manifestations of 41 mpox patients mainly included fever, rash and lymphadenopathy. Five patients with severe mpox might develop serious complications, including bacterial pneumonia, type Ⅰ respiratory failure, fungal infections, penile or perianal dry gangrene, penile soft tissue edema, intestinal obstruction, septic shock, perianal abscess, and necrotizing fasciitis. Patients with severe mpox had significantly higher white blood cell count (WBC), neutrophil count and C-reactive protein (CRP) level compare to those with mild cases (14.60(9.92, 24.08)×10 9/L vs 6.41(5.64, 8.37)×10 9/L, 12.43(7.02, 21.15)×10 9/L vs 3.35(2.46, 5.03)×10 9/L, 108.20(56.20, 124.10) mg/L vs 16.6(6.25, 49.98) mg/L), while the albumin level and CD4 + T lymphocyte count in the severe group were significantly lower compared to the mild cases (31.80(22.90, 35.15) g/L vs 44.70(42.90, 47.40) g/L, 24.00(12.00, 81.50)/μL vs 606.00(414.50, 767.50)/μL)). All these differences were statistically significant ( U=2.81, 3.02, 2.98, 3.56 and 3.26, respectively, all P<0.01). Conclusions:In clinical practice, clinicians should be vigilant for the possibility of severe mpox if patients exhibit a significant increase of WBC and CRP, a significant decrease in CD4 + T lymphocyte count, or if they develop severe complications.

Background::Total human immunodeficiency virus (HIV) DNA and integrated HIV DNA are widely used markers of HIV persistence. Droplet digital polymerase chain reaction (ddPCR) can be used for absolute quantification without needing a standard curve. Here, we developed duplex ddPCR assays to detect and quantify total HIV DNA and integrated HIV DNA.Methods::The limit of detection, dynamic ranges, sensitivity, and reproducibility were evaluated by plasmid constructs containing both the HIV long terminal repeat (LTR) and human CD3 gene (for total HIV DNA) and ACH-2 cells (for integrated HIV DNA). Forty-two cases on stable suppressive antiretroviral therapy (ART) were assayed in total HIV DNA and integrated HIV DNA. Correlation coefficient analysis was performed on the data related to DNA copies and cluster of differentiation 4 positive (CD4 +) T-cell counts, CD8 + T-cell counts and CD4/CD8 T-cell ratio, respectively. The assay linear dynamic range and lower limit of detection (LLOD) were also assessed. Results::The assay could detect the presence of HIV-1 copies 100% at concentrations of 6.3 copies/reaction, and the estimated LLOD of the ddPCR assay was 4.4 HIV DNA copies/reaction (95% confidence intervals [CI]: 3.6-6.5 copies/reaction) with linearity over a 5-log 10-unit range in total HIV DNA assay. For the integrated HIV DNA assay, the LLOD was 8.0 copies/reaction (95% CI: 5.8-16.6 copies/reaction) with linearity over a 3-log 10-unit range. Total HIV DNA in CD4 + T cells was positively associated with integrated HIV DNA ( r = 0.76, P <0.0001). Meanwhile, both total HIV DNA and integrated HIV DNA in CD4 + T cells were inversely correlated with the ratio of CD4/CD8 but positively correlated with the CD8 + T-cell counts. Conclusions::This ddPCR assay can quantify total HIV DNA and integrated HIV DNA efficiently with robustness and sensitivity. It can be readily adapted for measuring HIV DNA with non-B clades, and it could be beneficial for testing in clinical trials.

Objective: Cognition impairments are considered as a fundamental characteristic of severe mental disorders (SMD). Recent studies suggest that hyperprolactinemia may exert a detrimental influence on cognitive performance in patients with SMD. The objective of this study was to investigate the correlation between serum prolactin levels and cognitive function in female individuals diagnosed with SMD. Methods: We conducted a study on 294 patients with SMD and 195 healthy controls, aged between 14 to 55 years old. Cognitive function was assessed using the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), while prolactin levels were measured in serum. Descriptive analysis and comparative analysis were performed to compare cognitive function and prolactin levels between groups, and linear regression models were used to explore the relationship between prolactin and cognitive function. Results: Compared to the healthy control, individuals with SMD exhibited significantly higher levels of prolactin, while scoring lower on RBANS total and every index scores. Furthermore, a negative association between prolactin levels and cognitive function (RBANS total index score, attention, and delayed memory) was observed in SMD patients. Importantly, this inverse correlation between prolactin and cognition function (RBANS total index score, total scale score, and attention) persisted in patients who were not taking medications that could potentially influence serum prolactin levels. Conclusion Our study reveals a significant correlation between elevated prolactin levels and cognitive impairment in female patients with SMD, underscoring the importance of monitoring prolactin levels in order to prevent cognitive deterioration among female SMD patients.

OBJECTIVE: To explore the clinical characteristics and genetic basis of a child with Teebi hypertelorism syndrome 1 (TBHS1). METHODS: A child with TBHS1 who was admitted to the Children's Medical Center Affiliated to Shanghai Jiao Tong University School of Medicine on July 13, 2021 was selected as the study subject. Clinical data of the child was collected. Peripheral blood samples of the child and his parents were collected and subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a 13-year-old male, had manifested delayed growth and development. WES results revealed that he has harbored a heterozygous c.1244A>G variant of the SPECC1L gene, which was verified to be de novo in origin. The variant has not been included in the HGMD and gnomAD databases. As predicted by online software including PolyPhen-2, SIFT, and Mutation Taster, the variant may affect the function of protein domain. And PyMOL software has predicted that the structural stability of SPECC1L protein (p.Gln415Arg) might be reduced. Based on the guidelines of the American College of Medical Genetics and Genomics (ACMG), the variant was classified as pathogenic (PM6+PM1+PP4+PM2_Supporting+PP3). CONCLUSION The heterozygous c.1244A>G variant of the SPECC1L gene probably underlay the TBHS1 in this child. Above finding has expanded the genotypic and phenotypic spectrum of the SPECC1L gene and provided a basis for the clinical diagnosis of this child.
Adolescent ; Humans ; Male ; China ; Computational Biology ; Genomics ; Genotype ; Mutation

From February 1 to April 30, 2021, 48 general practice resident physicians in the First Affiliated Hospital of Naval Medical University were randomly divided into study group and control group with 24 in each group. The common comorbidities of the community-dwelling elderly, namely diabetes, diabetic retinopathy and osteoporosis were selected as teaching cases. The residents in control group received conventional teaching, while the scenario simulation teaching model of multicomorbity co-treatment was applied for the study group. The teaching effect, satisfaction and self-efficacy scores were compared between two groups. After training, the knowledge levels, the mastery of referral indicators and the performance of fundus ophthalmoscopy in the study group were significantly better than those in the control group ( t=2.27, 6.34, 4.09; P<0.05). They were (80.96±11.27) vs. (73.96±10.09), (10.33±2.41) vs. (6.38±1.88), (70.27±10.44) vs. (63.50±7.98), and students′ satisfaction and self-efficacy evaluation were higher than those of the observation group (all P<0.05). It is suggested that the simulation teaching of multi-disease co-treatment scenario is more beneficial than the traditional teaching to improve the comprehensive care ability of standardized training physicians in general practice for patients with chronic disease.

Objective    To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD). Methods    A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed. Results    There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001). Conclusion    All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

Background::Cluster of differentiation 8 (CD8 T) cells play critical roles in eradicating human immunodeficiency virus (HIV)-1 infection, but little is known about the effects of T cells expressing CD8 at low levels (CD8 low) or high levels (CD8 high) on HIV-1 replication inhibition after HIV-1 invasion into individual. Methods::Nineteen patients who had been acutely infected with HIV-1 (AHI) and 20 patients with chronic infection (CHI) for ≥2 years were enrolled in this study to investigate the dynamics of the quantity, activation, and immune responses of CD3 +CD8 low T cells and their counterpart CD3 +CD8 high T cells at different stages of HIV-1 infection. Results::Compared with healthy donors, CD3 +CD8 low T cells expanded in HIV-1-infected individuals at different stages of infection. As HIV-1 infection progressed, CD3 +CD8 low T cells gradually decreased. Simultaneously, CD3 +CD8 high T cells was significantly reduced in the first month of AHI and then increased gradually as HIV-1 infection progressed. The classical activation of CD3 +CD8 low T cells was highest in the first month of AHI and then reduced as HIV-1 infection progressed and entered the chronic stage. Meanwhile, activated CD38 -HLA-DR +CD8 low T cells did not increase in the first month of AHI, and the number of these cells was inversely associated with viral load ( r = -0.664, P = 0.004) but positively associated with the CD4 T-cell count ( r = 0.586, P = 0.014). Increased programmed cell death protein 1 (PD-1) abundance on CD3 +CD8 low T cells was observed from the 1st month of AHI but did not continue to be enhanced, while a significant T cell immunoreceptor with immunoglobulin and immunoreceptor tyrosine-based inhibition motif (ITIM) domains (TIGIT) abundance increase was observed in the 12th month of infection. Furthermore, increased PD-1 and TIGIT abundance on CD3 +CD8 low T cells was associated with a low CD4 T-cell count (PD-1: r = -0.456, P = 0.043; TIGIT: r = -0.488, P = 0.029) in CHI. Nonetheless, the nonincrease in PD-1 expression on classically activated CD3 +CD8 low T cells was inversely associated with HIV-1 viremia in the first month of AHI ( r = -0.578, P = 0.015). Notably, in the first month of AHI, few CD3 +CD8 low T cells, but comparable amounts of CD3 +CD8 high T cells, responded to Gag peptides. Then, weaker HIV-1-specific T-cell responses were induced in CD3 +CD8 low T cells than CD3 +CD8 high T cells at the 3rd and 12th months of AHI and in CHI. Conclusions::Our findings suggest that CD3 +CD8 low T cells play an anti-HIV role in the first month of infection due to their abundance but induce a weak HIV-1-specific immune response. Subsequently, CD3 +CD8 low T-cell number decreased gradually as infection persisted, and their anti-HIV functions were inferior to those of CD3 +CD8 high T cells.

ObjectiveTo investigate the value of remote consultation of heart sound acquisition in screening and referral of neonates with congenital heart diseases （CHD） in primary hospitals. MethodsA total of 4 030 neonates with non-critical diseases were selected. They were born in Shanghai Pudong New Area Maternal and Child Health Hospital from November 5， 2019 to March 31， 2021. After birth， routine cardiac auscultation was performed and remote consultation of heart sound collection were performed at the same time in combination with percutaneous oxygen saturation measurement to screen CHD. The children with any positive screening index were advised to verify the diagnosis by cardiac ultrasound examination in Shanghai Children's Medical Center. The diagnostic value of different screening methods was compared. ResultsA total of 110 cases were detected positive by routine screening. Among them， 16 cases were lost to follow-up， and 46 cases were confirmed by cardiac ultrasound examination， with a positive diagnosis rate of 48.94% （46/94）. A total of 51 cases were detected positive by routine screening and remote consultation of heart sound collection simultaneously. Among them， 42 cases were confirmed by cardiac ultrasound examination， with a positive diagnosis rate of 82.35% （42/51）. The difference between the two positive diagnosis rates was statistically significant （P<0.001）. ConclusionRemote consultation of heart sound acquisition on the basis of routine neonatal CHD screening can effectively improve the positive diagnosis rate of CHD screening in primary hospitals， and reduce unnecessary referrals. This method is simple and feasible. It has practical value in primary hospitals that lack professional technicians for the diagnosis and treatment of CHD.

Objective:To investigate the etiology of necrotizing pneumonia (NP) in children and the clinical characteristics of NP caused by different pathogens in China.Methods:A retrospective, case-control study was performed in children with NP who were admitted to 13 hospitals in China from January 2008 to December 2019. The demographic and clinical information, laboratory data, etiological and radiological findings were analyzed. The data were divided into three groups based on the following years: 2008-2011, 2012-2015 and 2016-2019, and the distribution characteristics of the pathogens in different period were compared. Meanwhile, the pathogens of pediatric NP in the southern and northern China were compared. And the clinical characteristics of the Mycoplasma pneumoniae (MP) NP and the bacterial NP were also compared. T-test or Mann-Whitney nonparametric test was used for comparison of numerical variables, and χ 2 test was used for categorical variables. Results:A total of 494 children with NP were enrolled, the median ages were 4.7 (0.1-15.3) years, including 272 boys and 222 girls. Among these patients, pathogens were identified in 347 cases and the pathogen was unclear in the remaining 147 cases. The main pathogens were MP (238 cases), Streptococcus pneumoniae (SP) (61 cases), Staphylococcus aureus (SA) (51 cases), Pseudomonas aeruginosa (13 cases), Haemophilus influenzae (10 cases), adenovirus (10 cases), and influenza virus A (7 cases), respectively. MP was the most common pathogen in all three periods and the proportion increased yearly. The proportion of MP in 2016-2019 was significantly higher than that in 2012-2015 (52.1% (197/378) vs. 36.8% (32/87), χ 2= 6.654, P=0.010), while there was no significant difference in the proportion of MP in 2012-2015 and that in 2008-2011 (36.8% (32/87) vs. 31.0% (9/29), χ2=0.314, P=0.575).Regarding the regional distribution, 342 cases were in the southern China and 152 in the northern China. Also, MP was the most common pathogen in both regions, but the proportion of MP was higher and the proportion of SP was lower in the north than those in the south (60.5% (92/152) vs. 42.7% (146/342), χ 2=13.409, P<0.010; 7.9% (12/152) vs. 14.3% (49/342), χ 2= 4.023, P=0.045). Comparing the clinical characteristics of different pathogens, we found that fever and cough were the common symptoms in both single MP and single bacterial groups, but chest pain was more common (17.0% (34/200) vs. 6.1% (6/98), χ 2=6.697, P=0.010) while shortness of breath and wheezing were less common in MP group (16.0% (32/200) vs. 60.2% (59/98), χ 2=60.688, P<0.01; 4.5% (9/200) vs. 21.4% (21/98), χ 2=20.819, P<0.01, respectively). The white blood cell count, C-reactive protein and procalcitonin in the bacterial group were significantly higher than those in the MP group (14.7 (1.0-67.1)×10 9/L vs. 10.5 (2.5-32.2)×10 9/L, 122.5 (0.5-277.3) mg/L vs. 51.4 (0.5-200.0) g/L, 2.13 (0.05-100.00) μg/L vs. 0.24 (0.01-18.85) μg/L, Z=-3.719, -5.901 and -7.765, all P<0.01). Conclusions:The prevalence of pediatric NP in China shows an increasing trend during the past years. MP, SP and SA are the main pathogens of NP, and the most common clinical symptoms are fever and cough. The WBC count, C-reactive protein and procalcitonin in bacterial NP are significantly higher than those caused by MP.

Objective:To use the methods of Health Care Failure Mode and Effects Analysis to reduce the paediatric wards noise pollution, Ensure the physical and mental health of medical staff and children, and maintain a good medical environment.Methods:The sampling method is adopted, noise investigation questionnaire was used in our study and we use the noise instrument to monitor the sound, and the focus source, the key time period and the key section of the room noise are obtained, then the HFMEA is carried out, we listed the work flow of the pediatric ward, and the flow is analyzed and discussed, calculate the Risk Priority Number (RPN). Then, some improvement measures were carried out aimed at the failure mode ,the noise survey questionnaire were used for evaluation.Results:After the implemented the HFMEA , The RPN value was significantly decreased ( P < 0.05). the degree of noise interference of children and their families decreased from 38 to 29, the difference was statistically significant ( χ2 value was 21.82, P<0.05) , and the satisfaction with control noise was improved from 18 to 30, the difference was statistically significant ( χ2 value was 36.28, P<0.05) .The intensity of noise key areas, The intensity of noise source and intensity in each period of the day were significantly improved and the difference was statistically significant ( P < 0.05), and the critical value was significantly decreased. Conclusion:Applying HFMEA can reduce ward noise pollution, improve patient satisfaction, ensure the physical and mental health of medical staff and children, and create a good medical environment.