简介：AbstractImportance:Extensive population-based studies have explored the prevalence of primary hypertension (HTN) in children and adolescents. However, there is little published data on the characteristics of different types of pediatric HTN and the causes of secondary HTN.Objective:To investigate the characteristics of different types of pediatric HTN and the causes of secondary HTN in a hospital setting.Methods:The study cohort comprised pediatric inpatients (<18 years of age) discharged with a diagnosis of HTN from Beijing Children’s Hospital during 2015-2020. Pediatric patients with HTN were allocated to secondary and primary HTN groups on the basis of comprehensive analyses of their diagnoses, family history of HTN, and findings on physical examination, as documented in their medical records. The Mann-Whitney U test, χ2 and Fisher’s exact test were used to assess differences in characteristics of patients with different HTN types and causes of secondary HTN.Results:Data of 1470 inpatients with HTN from 18 clinical departments were included in the analysis. Among them, 458 (31.2%) had primary HTN, and 1012 (68.8%) had secondary HTN. Compared with patients had primary HTN, children with secondary HTN were younger and had lower body mass indexes and longer lengths of stay. Moreover, children with primary HTN had mostly been managed by the Endocrinology and Cardiology Departments, 75.8% of them having obesity-related comorbidities. In contrast, most patients with secondary HTN had been managed by the Nephrology Department, renal diseases being the leading cause of their HTN (46.3%).Interpretation:Secondary HTN is more common than primary HTN in pediatric clinical settings, renal diseases being the leading cause of secondary HTN.

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简介：AbstractIntroduction:Cardiac neoplasms are particularly rare in children, and the majority of these tumors are benign. Approximately 10% of cardiac neoplasms are malignant, including soft tissue sarcomas and lymphomas. Cardiac tumors could also be metastases. Primitive EWSR1-negative round or spindle cell undifferentiated sarcoma harboring CIC gene translocation is a highly aggressive malignancy mainly occurring in soft tissues. However, it has not yet been described in the heart.Case presentation:We report a sarcoma that arose from the right ventricle in a 1-year-old girl. Histologically, it was composed of closely arranged small round or oval undifferentiated cells with fibrovascular separation, hyaline degeneration, and geographical necrosis. Immunohistochemically, the neoplastic cells exhibited focal membrane positivity for CD99 and diffuse positivity for WT1 and ETV4. Fluorescent in situ hybridization analysis showed EWSR1-negative but CIC-positive split signals. The breakpoint was also confirmed by whole genome sequencing.Conclusion:Based on morphological, immunohistochemical and molecular findings, this cardiac mass was diagnosed as CIC-rearranged sarcoma.

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简介：AbstractFamily integrated care (FICare) is a collaborative model of neonatal care which aims to address the negative impacts of the neonatal intensive care unit (NICU) environment by involving parents as equal partners, minimizing separation, and supporting parent-infant closeness. FICare incorporates psychological, educational, communication, and environmental strategies to support parents to cope with the NICU environment and to prepare them to be able to emotionally, cognitively, and physically care for their infant. FICare has been associated with improved infant feeding, growth, and parent wellbeing and self-efficacy; important mediators for long-term improved infant neurodevelopmental and behavioural outcomes. FICare implementation requires multi-disciplinary commitment, staff motivation, and sufficient time for preparation and readiness for change as professionals relinquish power and control to instead develop collaborative partnerships with parents. Successful FICare implementation and culture change have been applied by neonatal teams internationally, using practical approaches suited to their local environments. Strategies such as parent and staff meetings and relational communication help to break down barriers to change by providing space for the co-creation of knowledge, the negotiation of caregiving roles and the development of trusting relationships. The COVID-19 pandemic highlighted the vulnerability within programs supporting parental presence in neonatal units and the profound impacts of parent-infant separation. New technologies and digital innovations can help to mitigate these challenges, and support renewed efforts to embed FICare philosophy and practice in neonatal care during the COVID-19 recovery and beyond.

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简介：AbstractBackground:Epigenetics, especially DNA methylation, plays an important role in the pathogenesis of primary Sjogren syndrome (pSS). Our study aimed to reveal the role of DNA methylation in peripheral monocytes of pSS patients.Methods:A total of 11 pSS patients and five age-matched healthy controls (HCs) were included in this study. Monocytes were isolated from peripheral blood mononuclear cells using magnetic microbeads. DNA methylation profiles were generated using Human Methylation 850K BeadChips.Results:In monocytes from pSS patients, we identified 2819 differentially methylated positions (DMPs), comprising 1977 hypomethylated- and 842 hypermethylated-DMPs, corresponding to 1313 unique genes when compared with HCs. IFI44L, MX1, PAARP9, and IFITM1, which influence the interferon (IFN) signaling pathway, were among the genes hypomethylated in pSS. Functional analysis of genes with a minimum of two DMPs showed involvement in antigen binding, transcriptional regulation, cell adhesion, IFN-γ pathway, type I IFN pathway, antigen presentation, Epstein-Barr virus infection, human T-lymphotropic virus type 1 virus infection, and metabolic disease-related pathways. In addition, patients with higher serum IgG levels exhibited enrichment in Notch signaling and metabolic-related pathways. Upon comparing monocytes with salivary gland epithelial cells, an important overlap was observed in the cell cycle, cell senescence, and interleukin-17 signaling pathways. The differentially methylated genes were more enriched in the ribosome- and AMP-activated protein kinase signaling pathway in anti-Ro/SSA and anti-La/SSB autoantibodies double-positive patients.Conclusion:Genome-wide DNA methylation profiling revealed significant differences in DNA methylation in monocytes isolated from patients with pSS.

简介：AbstractBackground:Normal tension glaucoma (NTG) is a less pressure-dependent type of glaucoma with characteristic optic neuropathy. Recently, the biomechanical mechanism has been thought to account for glaucomatous optic neuropathy to some degree. We intended to compare dynamic corneal response parameters (DCRs) among patients with primary open-angle glaucoma with normal tension or hypertension and controls. The correlations between DCRs and known risk factors for glaucoma were also analyzed.Methods:In this cross-sectional study, 49 NTG subjects, 45 hypertension glaucoma (HTG) subjects, and 50 control subjects were enrolled. We compared the differences in DCRs using corneal visualization Scheimpflug technology among the NTG, HTG, and control groups. We also analyzed the correlations between DCRs and known risk factors for glaucoma (eg, central corneal thickness [CCT], intraocular pressure [IOP], etc).Results:The maximum inverse concave radius (NTG: 0.18 [0.17, 0.20] mm-1; control: 0.17 [0.16, 0.18] mm-1; P= 0.033), deformation amplitude ratio of 2 mm (DAR 2 mm, NTG: 4.87 [4.33, 5.39]; control: 4.37 [4.07, 4.88]; P < 0.001), and DAR 1 mm (NTG: 1.62 [1.58, 1.65]; control: 1.58 [1.54, 1.61]; P < 0.001) were significantly higher in NTG than in the controls. The integrated radius (IR, NTG: 8.40 ± 1.07 mm-1; HTG: 7.64 ± 1.31 mm-1; P = 0.026) and DAR 2 mm (NTG: 4.87 [4.33, 5.39]; HTG: 4.44 [4.12, 5.02]; P < 0.007) were significantly higher, whereas the stiffness parameter at the first applanation (SP-A1, NTG: 91.23 [77.45, 107.45]; HTG: 102.36 [85.77, 125.12]; P = 0.007) was lower in NTG than in HTG. There were no significant differences in the DCRs between HTG and control groups (P > 0.05). In the univariate and multivariate analyses, some of the DCRs, such as IR, were negatively correlated with CCT and IOP, whereas SP-A1 was positively correlated with CCT and IOP.Conclusions:The cornea was more deformable in NTG than in HTG or controls. There were no significant differences in corneal deformability between HTG and controls. The cornea was more deformable with the thinner cornea and lower IOP.

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简介：AbstractPurpose:It is a challenge for the primary hospitals to manage multiple trauma patients. In this article, we explored the advantage of establishing a surgical intensive care unit (SICU) predominant by cardiothoracic surgeons in the early management of multiple trauma.Methods:This was a retrospective study and patients with multiple trauma in our hospital were collected and divided into two groups, based on time period and treat modes: group A (retrospective observation group) where patients were treated with the traditional treatment mode from January 2017 to December 2017 and group B (study group) where patients were treated in the SICU predominant by cardiothoracic surgeons from January 2018 to December 2018. Clinical data including demographics, injury severity score (ISS), causes of injury, time intervals from reception to entering SICU or operating room and mortality three days after injuries were collected. Data were analyzed by SPSS 20.0 software. Categorical variables were presented as number and/or frequency and continuous variables as mean ± SD.Results:Altogether 406 patients were included in this study, including 217 patients in group A and 189 patients in group B. General data between the two groups revealed no significant difference: mean age (years) (35.51 ± 12.97 vs. 33.62 ± 13.61, p = 0.631), gender distribution (mean/female, 130/87 vs. 116/73, p = 0.589) and ISS (15.92 ± 7.95 vs. 16.16 ± 6.89, p = 0.698). Fall from height were the dominant mechanism of injury, with 135 cases in group A (71.4%) and 121 cases in group B (55.8%), followed by traffic accidents. Injury mechanism showed no significant differences between two groups (p = 1.256). Introduction of the SICU significantly improved the care of trauma patients, regarding speed and mortality. Time intervals between reception and entering SICU or operating room was (108.23 ± 6.72) min and (45.67 ± 7.96) min in group A and B, respectively (p = 0.001). Mortality three days after injuries was 13.89% and 5.53% in group A and B, respectively (p = 0.005).Conclusion:Establishing a SICU predominant by cardiothoracic surgeons can reduce the early mortality rates in multiple trauma patients.

简介：AbstractImportance:Pediatric hypervascular primary airway tumors are progressive, fatal lesions with a low incidence, and the disease is often more serious than that in adults.Objective:To evaluate the clinical efficacy and safety of interventional therapeutic bronchoscopy combined with conservative treatment and bronchial arterial embolization in children with primary airway tumors.Methods:We retrospectively analyzed the clinical data of four pediatric patients with hypervascular primary airway tumor between 2017 and 2019 at Beijing Children’s Hospital.Results:Two patients were low-grade bronchial mucoepidermoid carcinoma, one patient was pleomorphic adenoma, and one was bronchial leiomyoma. Interventional therapeutic bronchoscopy combined with bronchial arterial embolization was used for treatment (all four patients received general anesthesia). The tumors were safely resected in all patients via interventional bronchoscopy. There were no severe complications related to the procedures. All patients were followed up for 5-12 months, and one low-grade bronchial mucoepidermoid carcinoma recurred.Interpretation:Interventional therapeutic bronchoscopy combined with bronchial arterial embolization appears to be a safe and efficient therapeutic method associated with less trauma and fewer complications, including no serious adverse events, in children with hypervascular primary airway tumors without bronchus wall infiltration.

简介：AbstractIntroduction:Primary central nervous system lymphoma (PCNSL) is extremely rare in pediatric population. We reported a case of PCNSL in a 3-year-old girl and reviewed the literature in the past three decades.Case presentation:A 3-year-old girl presented with gait disturbance. A contrast-enhanced magnetic resonance image of the brain showed a solitary bulky mass in the left cerebellar hemisphere, hydrocephalus and cerebellar tonsillar hernia. Surgical resection was performed and the patient was diagnosed with primary central nervous system lymphoblastic B cell lymphoma. Then the patient received regular chemotherapy, including 6 cycles of chemotherapy containing high-dose methotrexate (HD-MTX). The patient remains alive 15 months after the diagnosis with no evidence of active disease, but suffered twice chronic subdural hematoma, which was treated by burr hole drainage.Conclusion:Lymphoblastic B cell lymphoma is a rare histologic subtype of pediatric PCNSL. Chemotherapy containing HD-MTX remains the most effective treatment. The patient should avoid head impact after surgical resection of the tumor to prevent chronic subdural hematoma.

简介：AbstractTotal elbow arthroplasty as a treatment option for open elbow fracture is relatively rare described. We reported a 39 years old polytrauma patient with complex open elbow fracture (Gustilo-Anderson type IIIB). The patient presented with large soft tissues defect on dorsal part of the left elbow, ulnar palsy due to the irreparable loss of the ulnar nerve, distal triceps loss due to the complete loss of the olecranon, loss of both humeral condyles with collateral ligaments and complex elbow instability. Only few similar cases have been published. Reconstructive surgery included repetitive radical debridement, irrigation, vacuum assisted closure system therapy, external fixation, coverage of the soft tissue defect with fascia-cutaneous flap from the forearm. Four months after the injury, total elbow arthroplasty with autologous bone graft (from the proximal radius) inserted in the ulnar component, was performed. At 3 years postoperatively, the patient is able to perform an active flexion from 0° to 110° with full pronosupination. Only passive extension is allowed. The ulnar neuropathy is persistent. Patient has no signs of infection or loosening of the prosthesis.

简介：AbstractObjective:To identify potential early diagnostic markers for hepatitis B progression to primary liver carcinoma using routine immunological tests based on 6 cytokine combinations.Methods:Eight hundred and ninety-nine patients with hepatitis B progressing to early primary liver carcinoma admitted to and treated at Changhai Hospital, Naval Military Medical University, Shanghai, China between March 2015 and June 2017 were included in this observational study, including 666 patients with HBsAg+, HBeAb+, HBcAb+ liver carcinoma and 233 patients with HBsAg+, HBeAg+, HBcAb+ liver carcinoma. Receiver operating characteristic (ROC) curves were used to evaluate the efficiency of the different cytokine in the diagnosis of hepatocellular carcinoma in patients with hepatitis B. This study was approved by the Institutional Review Board of Changhai Hospital, Naval Military Medical University, China (approval No. CHEC2020-080) on June 6, 2020.Results:Changed levels of interleukin (IL)-1β, IL-2R, IL-8, and tumor necrosis factor (TNF)-α were statistically significant (P < 0.05). The area under the ROC curve, sensitivity, specificity, positive predictive value, negative predictive value, and Youden index for the diagnosis of primary liver carcinoma using the combination of IL-1β, IL-2R, IL-8, and TNF-α were 0.938, 79.2%, 96.7%, 96%, 82.0%, 0.759, respectively. The serum alpha-fetoprotein level in patients with primary liver carcinoma was positively correlated with IL-2R (r=0.3502, P < 0.001), IL-8 (r=0.1558, P=0.0273), and TNF-α (r=0.2544, P < 0.001) levels. The equation fitted to the results was logit(P)=0.086+ 0.01 × IL-2R-0.001 × IL-8-0.033 × TNF-α-0.041 × IL-1β.Conclusion:Our study establishes a novel, potentially valuable diagnostic model based on four cytokines related to the early stages of liver carcinoma.

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简介：AbstractBackground:Although endovascular therapy has been widely used for focal aortoiliac occlusive disease (AIOD), its performance for extensive AIOD (EAIOD) is not fully evaluated. We aimed to demonstrate the long-term results of EAIOD treated by endovascular therapy and to identify the potential risk factors for the loss of primary patency.Methods:Between January 2008 and June 2018, patients with a clinical diagnosis of the 2007 TransAtlantic Inter-Society Consensus II (TASC II) C and D AIOD lesions who underwent endovascular treatment in our institution were enrolled. Demographic, diagnosis, procedure characteristics, and follow-up information were reviewed. Univariate analysis was used to identify the correlation between the variables and the primary patency. A multivariate logistic regression model was used to identify the independent risk factors associated with primary patency. Five- and 10-year primary and secondary patency, as well as survival rates, were calculated by Kaplan-Meier analysis.Results:A total of 148 patients underwent endovascular treatment in our center. Of these, 39.2% were classified as having TASC II C lesions and 60.8% as having TASC II D lesions. The technical success rate was 88.5%. The mean follow-up time was 79.2 ± 29.2 months. Primary and secondary patency was 82.1% and 89.4% at 5 years, and 74.8% and 83.1% at 10 years, respectively. The 5-year survival rate was 84.2%. Compared with patients without loss of primary patency, patients with this condition showed significant differences in age, TASC II classification, infrainguinal lesions, critical limb ischemia (CLI), and smoking. Multivariate logistic regression analysis showed age <61 years (adjusted odds ratio [aOR]: 6.47; 95% CI: 1.47-28.36; P = 0.01), CLI (aOR: 7.81; 95% CI: 1.92-31.89; P = 0.04), and smoking (aOR: 10.15; 95% CI: 2.79-36.90; P < 0.01) were independent risk factors for the loss of primary patency.Conclusions:Endovascular therapy was an effective treatment for EAIOD with encouraging patency and survival rate. Age <61 years, CLI, and smoking were independent risk factors for the loss of primary patency.

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