Objective:To summarize the clinical manifestations, experiences in diagnosis and treatment of arrhythmogenic cardiomyopathy (ACM) in children.Methods:A retrospective analysis of the clinical manifestations, laboratory tests, radiological features, treatment and follow-up results was conducted in 11 children diagnosed with ACM at the center of congenital heart disease, Beijing anzhen hospital from May 2010 to March 2022.Results:A total of 11 patients aged 2 to 16 years, including 5 males and 6 females were diagnosed with ACM. The clinical manifestations included decreased activity tolerance (7 patients), heart failure (4 patients), syncope or sudden death (3 patients), palpitation (3 patients), and chest tightness and pain (3 patients). Electrocardiogram showed right bundle branch block in 9 cases, paroxysmal ventricular tachycardia in 4 cases, frequent premature ventricular contraction in 4 cases, ventricular pre-excitation in 1 case, left bundle branch block in 1 case, and first degree atrioventricular block in 2 cases. Echocardiography showed enlargement of the right heart, widening of the right ventricular outflow tract, and thinning and bulging of the local wall of the right ventricle with reduced pulsation. Ventricular thrombosis was found in 2 cases. Six children underwent cardiac magnetic resonance imaging, which mainly showed severe enlargement of the right heart, thin free wall of the right ventricle, decreased right heart function, enhanced right ventricular myocardium, and formation of right ventricular aneurysm. Two children underwent myocardial biopsy examination and presented with typical pathological changes of ACM. Genetic tests in five patients revealed DSG2 gene mutation in 2 cases, PKP2 gene mutation in 2 cases, and MYH6 gene mutation in 1 case. All patients received anti heart failure treatment and antiarrhythmic drugs. Two children received anticoagulant treatment due to ventricular thrombosis. Radiofrequency ablation was performed in 2 patients. Glenn procedure was performed in 4 patients, and heart transplantation was performed in 1 patient due to progressive heart failure. The follow-up period ranged from 6 months to 12 years. Two cases died of right heart failure, 6 cases had different degrees of heart failure, 1 case had intermittent chest tightness and pain, and 2 cases were stable.Conclusions:ACM is a progressive genetic cardiomyopathy characterized by decreased activity tolerance, cardiac failure and arrhythmia in pediatric patients. The diagnosis is mainly based on clinical manifestations, electrocardiogram, cardiac imaging changes, and genetic testing. Early detection, diagnosis, and personalized treatment can improve the prognosis.

Objective:To evaluate the impact of bladder volume on dosimetric parameters of clinical target volume (CTV) and organs at risk (OAR) of intensity modulated proton therapy (IMPT) for localized prostate cancer during the treatment planning and daily treatment.Methods:Clinical data of 25 patients with localized prostate cancer admitted to Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine from November 2021 to June 2022 and enrolled in the "Proton Therapy System" (SAPT-PS-01) registered clinical trial were retrospectively analyzed. All patients were male and the median age was 72 years old. A total of 30 sets of IMPT plans were obtained. Based on the planning CT (30 sets) and weekly verification CT during treatment (172 sets), bladder volume, CTV and OAR dose parameters were collected. Spearman correlation analysis was used to evaluate the correlation between bladder volume in CT and the dosimetric parameters of CTV and OAR during IMPT plans, and Wilcoxon-Mann-Whitney test was adopted to compare the dosimetric parameters of CTV and OAR among different bladder volume change groups.Results:The V 95% of CTV1 and CTV2 were both 100.0%±0.0% in IMPT plans. Bladder volume was significantly negatively correlated with D mean, V 70 Gy(RBE), V 60 Gy(RBE), V 50 Gy(RBE), V 40 Gy(RBE) of the bladder ( P<0.001, 0.003, <0.001, <0.001,<0.001), and D mean, V 50 Gy(RBE) of the small intestine (both P<0.001). During treatment, bladder D mean, V 70 Gy(RBE), V 60 Gy(RBE), V 50 Gy(RBE), V 40 Gy(RBE)( P<0.001, 0.001, <0.001, <0.001, <0.001), rectal D mean, V 50 Gy(RBE), V 40 Gy(RBE) (all P<0.001), small intestine D mean, V 50 Gy (RBE) (both P<0.001) of patients with bladder volume increase >20% compared to baseline were significantly decreased compared to those in IMPT plans. But CTV1 V 100%, and CTV2 V 95% were significantly decreased too( P=0.029, 0.020). In the bladder volume decreased>20% patients, the D mean, V 70 Gy(RBE), V 60 Gy(RBE), V 50 Gy(RBE), V 40 Gy(RBE) of the bladder were significantly increased compared to those in IMPT plans (all P<0.001). However, a bladder volume reduction of ≤20% and increase of ≤20% from baseline had no significant impact on CTV and OAR dosimetric parameters during treatment. Conclusions:For patients with localized prostate cancer undergoing proton therapy, a certain bladder volume should be ensured during planning CT scans. During the daily treatment, the bladder volume should be maintained between 80%-120% of the baseline level to ensure CTV coverage and good dose sparing to OAR.

ObjectiveTo investigate a suspected outbreak of carbapenem-resistant Acinetobacter baumannii （CRAB） nosocomial infection in an intensive care unit （ICU） and provide scientific evidence for prevention and control of multi-drug resistant nosocomial infection. MethodsClinical and epidemiological data of 4 patients with CRAB infection were retrospectively investigated in the ICU of Renji Hospital in November 2021. Environmental hygiene monitoring and multilocus sequence typing （MLST） were conducted and intervention measures were taken. ResultsA total of 4 cases with CRAB infection were identified， among which 1 case was determined to be community-acquired and3 cases were hospital-acquired. The isolated strains shared the same drug resistance， and were all classified into ST368 type. In the surface and hand samples （n=40）， 2 CRAB strains were detected in the air filter beside the bed of the first case， with a detection rate of 5%. After adopting comprehensive prevention and control strategies， including environmental cleaning and disinfection， hand hygiene， staff management and training， and supervision， no similar case with CRAB infection was found. ConclusionThis suspected outbreak of CRAB nosocomial infection may be induced by inadequate environmental cleaning and disinfection， and inadequate implementation of hand hygiene. Timely identification， investigation， and targeted measures remain crucial to effective control of possible nosocomial infection.

ObjectiveTo understand the use of antibiotics in inpatients and the incidence and trend of hospital infections， to explore the implementation effect of comprehensive management measures， and to provide reference for hospitals to use antibiotics reasonably. MethodsBased on the hospital infection monitoring and management system， a retrospective analysis and comparison were conducted on the use of antibiotics， submission of microbial test samples， and incidence of hospital infections among inpatients in a tertiary hospital from 2012 to 2021. ResultsFrom 2012 to 2021， the use of antibiotics showed a downward trend， from 50.82% in 2012 to 41.29% in 2021. At the same time， the use rate of restricted and special antibiotics had also decreased， and the use rate of restricted and special antibiotics in patients without hospital infection was significantly lower than that in patients with hospital infection， and the microbial testing rate was also on the rise. The annual incidence rate of hospital infection was 0.69%‒1.92%， and the annual case-time prevalence rate was 0.79%‒2.17%. The annual average rate of the above two in 10 years was 1.18% and 1.34%， respectively. The results of the exponential smoothing model also showed that the utilization rate of antibiotics was decreasing and the incidence of nosocomial infection was stable. ConclusionLarge general hospitals took comprehensive management measures to strengthen the management of rational use of antibiotics， which led to a decline in the use rate of antibacterial drugs for inpatients and an increase in the rate of microbial examination. At the same time， the overall incidence of hospital infection was relatively stable， suggesting that the comprehensive management measures of antibacterial drugs in hospitals had achieved certain results. The current measures need to be optimized in the future to continuously improve the management level of rational use of antibacterial drugs.

Objective:To study the current status of longitudinal extrauterine growth restriction (EUGR) in extremely preterm infants (EPIs) and to develop a prediction model based on clinical data from multiple NICUs.Methods:From January 2017 to December 2018, EPIs admitted to 32 NICUs in North China were retrospectively studied. Their general conditions, nutritional support, complications during hospitalization and weight changes were reviewed. Weight loss between birth and discharge > 1SD was defined as longitudinal EUGR. The EPIs were assigned into longitudinal EUGR group and non-EUGR group and their nutritional support and weight changes were compared. The EPIs were randomly assigned into the training dataset and the validation dataset with a ratio of 7∶3. Univariate Cox regression analysis and multiple regression analysis were used in the training dataset to select the independent predictive factors. The best-fitting Nomogram model predicting longitudinal EUGR was established based on Akaike Information Criterion. The model was evaluated for discrimination efficacy, calibration and clinical decision curve analysis.Results:A total of 436 EPIs were included in this study, with a mean gestational age of (26.9±0.9) weeks and a birth weight of (989±171) g. The incidence of longitudinal EUGR was 82.3%(359/436). Seven variables (birth weight Z-score, weight loss, weight growth velocity, the proportion of breast milk ≥75% within 3 d before discharge, invasive mechanical ventilation ≥7 d, maternal antenatal corticosteroids use and bronchopulmonary dysplasia) were selected to establish the prediction model. The area under the receiver operating characteristic curve of the training dataset and the validation dataset were 0.870 (95% CI 0.820-0.920) and 0.879 (95% CI 0.815-0.942), suggesting good discrimination efficacy. The calibration curve indicated a good fit of the model ( P>0.05). The decision curve analysis showed positive net benefits at all thresholds. Conclusions:Currently, EPIs have a high incidence of longitudinal EUGR. The prediction model is helpful for early identification and intervention for EPIs with higher risks of longitudinal EUGR. It is necessary to expand the sample size and conduct prospective studies to optimize and validate the prediction model in the future.

Objective:To recheck the Histoplasma capsulatum and Coccidioides sp. strains of the past by molecular identification and matrix-assisted laser desorption/ionization time-of-flight mass spectrometry(MALDI-TOF MS). Methods:The phylogenetic relationships among the ten Histoplasma capsulatum isolates and reference strains were analyzed by multilocus sequence typing (MLST). Based on the Coi region, Coccidioides posadasii was distinguished from Coccidioides immitis accurately. MALDI-TOF MS was used to set up the MALDI-TOF MS database of Histoplasma capsulatum and Coccidioides sp. for rapid identification. In addition, hierarchical clustering of spectra was compared with MLST. Results:An unrooted dendrogram constructed with MLST showed that ten individuals of Histoplasma capsulatum were divided into three clades: Eurasia clade, Australia clade and North American class 2 clade, in agreement with the establishment by MALDI-TOF MS cluster analysis. All individuals of Coccidioides sp. were identified as Coccidioides posadasii with Coi region primers. The in-house MALDI-TOF MS database of Histoplasma capsulatum and Coccidioides posadasii was expanded and reached an identified accuracy of 100%. Conclusions:We improve the recognition of Histoplasma capsulatum and Coccidioides posadasii by molecular pathways which shows the major species or clades in Chinese mainland. The in-house MALDI-TOF MS database of Histoplasma capsulatum and Coccidioides posadasii can provide a new efficient way to identify those pathogens rapidly.

Objective:To investigate the correlation between specific expression of serum micro ribonucleic acid (miRNA) and dilated cardiomyopathy (DCM) in children.Methods:Sixteen children diagnosed with DCM in Pediatric Heart Center of Beijing Anzhen Hospital from November 2013 to March 2016 were enrolled in the DCM group.Meanwhile, 12 age- and gender-matched healthy children who underwent medical examinations at the same time in the same hospital were selected as the healthy control group.Their serum was collected and miRNA sequencing was performed.The sample size was expanded at the later stage (the DCM group included 30 cases, and the healthy control group included 16 cases). The real-time fluorescence quantitative polymerase chain reaction (qRT-PCR) verification experiment was conducted on 11 miRNAs with statistically significant sequencing results.Results:Serum miRNA sequencing showed that 172 miRNAs were up-regulated but no miRNAs were down-regulated in the DCM group, compared with the healthy control group (fold change>2, P<0.001). Top 11 significantly up-regulated miRNAs were verified by qRT-PCR, and it was found that 8 of the 11 miRNAs (let-7f, let-7g, miR142-5p, miR143-3p, miR26a, miR27a-3p, miR27b-3p, and miR126-3p) in the DCM group were significantly up-regulated (all P<0.05). In the receiver operating characteristic (ROC) curves of DCM patients, the area under the curves of serum miR142-5p, miR143-3p, miR27b-3p, and miR126-3p were 0.983, 0.992, 0.915 and 0.950, respectively, which were statistically significantly different from those of the healthy control group (all P<0.05). Conclusions:Four serum miRNAs (miR-142-5p, miR-126-3p, miR-143-3p and miR-27b-3p) can distinguish children with DCM from healthy children.Circulating miRNAs are effective in screening DCM children.

Objective: To study the antipyretic, anti-inflammatory and analgesic effects of Yinhaojiere mixture and the antipyretic effect of Ilex asprella. Methods: The experiments of mice ear edma induced by xylene, the increase of capillary permeability by acetic acid and the sterilized cotton caused rat groin granulation tissue hyperplasia were used to study the anti-inflammatory effect of Yinhao-jiere mixture; the hot-plate tests and the acetic acid writhing test were applied to research the analgesic effect of Yinhaojiere mixture. In addition, the dry yeast induced fever in rats was employed to explore the efficacy of antipyretic agents in Yinhaojiere mixture and Ilex asprella water extract. Results: Yinhaojiere mixture could obviously inhibit the inflammation of the peritoneal cavity of mice ( P <0. 05). The inhibition of Yinhaojiere mixture against ear swelling induced by p-xylene was significant. Yinhaojiere mixture could sig-nificantly increase the pain threshold. Especially, Yinhaojiere mixture had significant antipyretic effect (P<0. 05). In the anti-inflam-matory, analgesic and antipyretic experimetns, there were no significant differences between the middle and high dose groups of Yinha-ojiere mixture and indomethacin group (P>0. 05). Conclusion: Yinhaojiere mixture with the Ilex asprella as the main ingredient has notable anti-inflammatory, analgesic and antipyretic effects, and Ilex asprella exhibits anti-inflammatory, analgesic effects, and its anti-pyretic effect is more significant.

Objective To observe the clinical efficacy and safety of high-frequency diathermic therapy and intra-thoracic chemotherapy with recombinant endostar combined with cisplatin in treatment of malignant pleural effusion. Methods A total of 48 patients with malignant pleural effusion diagnosed in the First People ' s Hospital of Guiyang from September 2014 to September 2016 were randomly divided into observation group and control group with envelope method. Twenty-four patients in observation group were received high-frequency diathermic therapy and cisplation 60 mg/m2 combined with endostar 25-30 mg/m2 intra-thoracic chemotherapy. Twenty-four patients in control group were treated with high-frequency diathermic therapy and single cisplation 60 mg/m2 intra-thoracic chemotherapy. The patients were karnofsky performance scores ≥70, cooperate with intravenous systemic chemotherapy, every 21 days for a total of two or three cycles. High frequency diathermic therapy was administered twice a week for 3 weeks 30 minutes after intra-thoracic chemotherapy,treatment time of 60 minutes every time. Chi-squared test were selected to evaluate the clinical efficacy and quality of life and adverse and toxic responses, respectively. The paired samplest test were selected to evaluate the variation of tumor markers in hydrothorax of the two groups pretherapy and post-treatment. Results In 24 patients of observation group, 2 patients were complete remission (CR), 17 patients were partial remission (PR), 4 patients were disease stable (SD), 1 patient was disease progression (PD), and the objective response rate (ORR) (CR+PR) was 79.2 ％. In 24 patients of control group, 0 patient was CR, 12 patients were PR, 7 patients were SD, 5 patients were PD, and the ORR was 50.0％, there was significant difference in ORR between the two groups (χ 2 = 4.463, P = 0.035). Karnofsky performance scores in observation group was higher than that in control group after treatment, patients with clinical benefit rate was 79.2 ％ vs. 54.2 ％, but the difference in life quality between the two groups was no statistically significant (χ2=3.375, P=0.066). There was no significant difference in adverse reactions between the two groups (P>0.05). The tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 125 (CA-125), carbohydrate antigen 199 (CA-199), cytokeratin 19 fragments (CYFRA21-1) and neuron-specific enolase (NSE) in hydrothorax of the two groups were reduced by the treatment, except for the difference of CYFRA21-1 between the two group was not statistically significant (P= 0.161), the changes of the other 3 indicators in the observation group before and after treatment were greater than those in the control group (all P< 0.05). Conclusions High-frequency diathermic therapy and intra-thoracic chemotherapy with single cisplatin are effective in treating patients with malignant pleural effusion, and is more superior when combined with endostar. Additionally, the combination of the above two drugs has synergistic action and better safety, deserves to be further promoted in clinic.

Objective To investigate the value of 99Tcm-3PRGD2 SPECT/CT imaging in the diagnosis of benign and malignant lung lesions.Methods A retrospective analysis of 99Tcm-3PRGD2 planar imaging and chest SPECT/CT imaging with lung lesions before treatment was performed.Totally 37 cases including 27 malignant and 10 benign lesions confirmed pathologiclly were enrolled.Compared with hepar in planar imaging,the intake gray scale of 99Tcm-3PRGD2 in lung lesions was divided into low,moderate,and high elevations.The ratio of the highest intake value of the lesion (L) to the contralateral normal lung (N),the hepar (H),the mediastinum (Me) and the contralateral deltoid muscle (Mu) was calculated,and statistical analysis was performed to evaluate the diagnostic efficacy.The expression of immunohistochemistry integrin αvβ3 was performed through selecting 1 case of benign and malignant lung lesions,respectively.Results The intake of 99Tcm-3PRGD2 was higher in 37 patients with lung lesions.High elevation of malignant lesion was 66.67％ (18/27),low and moderate elevation of benign lesion was 90.00％ (9/10).The intake ratio of malignant lung lesions was higher than that of benign lesions (all P＜0.05).When L/N was greater than 5.45 or L/Mu was greater than 4.65,the sensitivity,specificity and accuracy of diagnosis of lung malignant lesions was 77.80％,80.00％ and 78.40％,respectively.Immunohistochemical results showed that malignant lung lesions highly expressed integrin αvβ3,and only a small number of benign lesions expressed it.Conclusion 99 Tcm-3PRGD2 SPECT/CT is valuable in the differential diagnosis of benign and malignant lung lesions.