Background: Lymphatic malformations are common congenital vascular lesions. Neither surgical resection nor other surgical treatments have been found to be effective for invasive cases. Recent research has suggested that sirolimus is effective in treating complex lymphatic malformations (LMs). We aimed to evaluate the effectiveness and safety of oral sirolimus for children living with LMs in our hospital. Methods: Fifty-six cases of complex LMs treated with sirolimus were collected from Shanghai Children's Medical Centre between June 2016 and March 2019. All cases were confirmed either by pathology (44) or enhanced MRI (12). Following informed consent, sirolimus 0.8 mg/m 2 bid was administered orally to participants and maintained at a trough concentration of 10-15 ng/ml. Children's ages at diagnosis were neonate to 16 years (mean 44.3 months). All children were followed up for 5 to 30 months, with a mean of 16.8 months. Results: During the follow-up period, blood, liver and kidney function as well as disseminated intravascular coagulation was regularly reviewed in all 56 children. Enhanced MRI was regularly performed to evaluate therapeutic effects. Total effective rate (complete response or partial response) of LMs was 89.3% (50/56). No serious adverse reactions were found. Conclusion: This study suggests that sirolimus is effective and tolerable for decreasing lesions in children with complex LMs, leading to fewer and more tolerable side effects. There is no need to pursue an excision rate to reduce unnecessary operative complications since adjuvant sirolimus therapy modifies the complex LMs clinical appearance and alleviates their symptoms. Type of study: Clinical research.

Background: Multidrug resistant (MDR) and extensively drug resistant (XDR) Acinetobacter baumannii presents challenges for clinical treatment and causes high mortality in children. We aimed to assess the risk factors and overall mortality for MDR/XDR Acinetobacter baumannii infected pediatric patients. Methods: This retrospective study included 102 pediatric patients who developed MDR/XDR Acinetobacter baumannii infection in the pediatric intensive care unit (PICU) of Shanghai Children's Hospital in China from December 2014 to May 2018. Acinetobacter baumannii clinical isolates were recovered from different specimens including blood, sputum, bronchoalveolar lavage fluid, cerebrospinal fluid, ascites, hydrothorax, and urine. Antibiotic susceptibility test was determined according to the Clinical and Laboratory Standards Institute interpretive criteria. Clinical and biological data were obtained from the patients' medical records.

Abstract. The present study aimed to investigate the mechanism by which Aurora kinase A (AURKA) promotes cell migration and invasion in head and neck squamous cell carcinoma (HNSCC). Transwell assays were performed to investigate the cell migration and invasion abilities of AURKA, whilst western blotting was used to analyze the protein expression in FaDu and Hep2 cells, each treated with pharmacological inhibitors. Following the inhibition of AURKA, Akt and focal adhesion kinase (FAK), the migration and invasion of the FaDu and Hep2 cells decreased. The expression of phosphorylated (p)-AURKA and p-FAK (Y397) was observed to decrease following FaDu and Hep2 cell treatment with VX-680, a small molecular inhibitor of AURKA. The expression of p-Akt and p-FAK (Y397) ceased following treatment with the Akt inhibitor triciribine. The expression of p-FAK (Y397) decreased, however, p-Akt expression did not change following treatment with the FAK inhibitor TAE226. In conclusion, AURKA activates FAK through the AURKA/Akt/FAK signaling pathway, promoting the migration and invasion of HNSCC cells, which may subsequently provide a novel approach for the treatment of HNSCC.

Adenoidectomy, surgical removal of hypertrophic adenoids, is a common operation in children worldwide. The purpose of this study was to compare the operative effectiveness, and included total operative time, blood loss and complications, between endoscopic assisted adenoidectomy and conventional curettage adenoidectomy. EMBASE, PubMed, Cochrane Library, and China National Knowledge Infrastructure and symposiums and review articles were used to choose relevant randomized controlled trials. A meta-analysis was performed to analyze the data for total operative time, blood loss and complications. Seven studies fit the inclusion criteria, and included 331 patients treated with endoscopic assisted adenoidectomy, and 251 patients treated with conventional curettage adenoidectomy. The meta-analysis demonstrated that compared with conventional curettage adenoidectomy, endoscopic assisted adenoidectomy had a shorter operative time (SMD −1.09; 95 % CI −1.29 to −0.90; p < 0.00001), less blood loss (MD −19.74; 95 % CI −22.75 to −16.73; p < 0.00001), and fewer complications (OR 0.15; 95 % CI 0.07–0.35; p < 0.0001). Endoscopic assisted adenoidectomy has advantages over conventional curettage adenoidectomy with regard to total operative time, blood loss and complications.

Background: Describe the outcome of adenovirus pneumonia in a pediatric intensive care unit (PICU) over a 3-year period, to identify the risk factors that may be associated with worse outcome. Methods: A retrospective observational study was performed in the PICU of children's hospital in Shanghai from July 2016 to June 2019. Sixty-seven children over 29 days to 14 years old with adenovirus pneumonia who were admitted to PICU with acute hypoxemic respiratory failure were included in this study. The primary outcome was hospital mortality, and secondary outcomes were hospital and PICU length of stay (LOS), and risk factors of worse outcome. Results: Of 67 children with severe adenovirus pneumonia, the hospital mortality was 16.42% (11/67) and 28-day mortality was 14.93% (10/67). Median Pediatric Risk of Mortality III (PRISM III) score at admission was 13 (interquartile range [IQR], 10-15). Median PICU LOS stay was 11 days (8-18d) and hospital LOS was 22 days (16-31d). Among children with extracorporeal membrane oxygenation (n = 9), 6 cases survived and 3 cases died. The patients who need renal replacement therapy, neuromuscular blockade, parenteral nutrition, and packed red blood cell perfusion had higher hospital mortality (p < 0.001, p = 0.041, p = < 0.001, p = 0.012, respectively). Multivariate logistic analysis indicated that liver dysfunction and nosocomial infection were associated with high risk of mortality. Conclusions: The hospital mortality of adenovirus pneumonia in our PICU was 16.42%. Patients complicated liver dysfunction and co-infection & nosocomial infection were associated with poor outcome.

Studies have shown that long non-coding RNA (lncRNA) may act as the carcinogenic factor or tumor suppressor of laryngeal squamous cell carcinoma (LSCC). This study aims to identify the prognostic value and potential target protein-coding genes (PCGs) of lncRNAs in LSCC. The LSCC datasets were collected from The Cancer Genome Atlas (TCGA). Statistical and bioinformatic methods were used to establish and evaluate the prognostic model, identify the correlation between lncRNAs and clinical characteristics, and screen for PCGs co-expressed with lncRNAs. Weighted gene coexpression network analysis (WGCNA) identified PCG modules associated with clinical characteristics. The expression of lncRNAs and PCGs was analyzed using our LSCC patients by RT-qPCR. LINC02154, LINC00528, SPRY4-AS1, TTTY14, LNCSRLR, and KLHL7-DT were selected to establish the prognostic model. The overall survival (OS) of low-risk patients forecasted by the model was significantly better than highrisk patients. Receiver operating characteristic (ROC) curve and concordance index (C-index) validated the accuracy of the prognostic model. Chi-square test showed that six lncRNAs were associated with one of the clinical characteristics, i.e., gender, clinical stage, T and N stage, respectively. WGCNA identified PCG modules associated with gender, clinical stage, T and N stage. We took the intersection of the PCG modules of WGCNA, the differentially expressed PCGs between LSCC and normal samples, and the PCGs co-expressed with six lncRNAs. The intersection PCGs survival analysis showed that four PCGs, i.e., STC2, TSPAN9, SMS, and TCEA3 affected the OS of LSCC. More importantly, the differential expression of six lncRNAs and four PCGs between LSCC and normal samples was verified by our LSCC patients. In conclusion, we successfully established a prognostic model based on six-lncRNA RiskScore and initially screened the potential target PCGs of six lncRNAs for further basic and clinical research.

Hepatocellular carcinoma is the most common liver malignancy. Salvage liver transplantation (SLT) is viewed as a feasible cure for recurrence of HCC after resectomy, but the effect is under dispute. A retrospective study examined data at Renji Hospital for 239 transplants from January 2006 to December 2015, including 211 who received primary liver transplantation (PLT) and 28 who underwent SLT. A multivariable cox regression model was employed to pick out relative factors to overall survival (OS) and recurrence free survival (RFS). Propensity score matching (PSM) was used to balance the bias. Both OS and RFS were worse in SLT group than in PLT group, especially for those patients within Milan criteria. Our study demonstrates that SLT bears higher risk of recurrence and death than PLT, indicating that SLT should be given a more careful thought at performance.

The proto-oncogene MYC can trigger the unfolded protein response (UPR). The double-stranded RNA-activated protein kinase-like endoplasmic reticulum kinase (PERK), one of three primary branches of the UPR, is a key regulator of autophagy, promoting tumorigenesis. Upon activation of PERK, there is an increase in phosphorylation of the eukaryotic initiation factor-2 alpha (eIF2α), which in turn, activates the transcription factor-4 (ATF4), responsible for an increased expression of LC3, a common autophagy marker. PERK is repressed upon GLI1 and GLI2 induction. GANT-61 is an inhibitor of GLI1 and GLI2, known to reduce autophagy in MYCN nonamplified, but not in MYCN amplified neuroblastoma (NB) cells. In our study, we tested the effect of the joint administration of a PERK inhibitor (GSK2606414) and the GLI inhibitor GANT-61 to MYCN amplified and MYCN non-amplified NB cells. Our results suggest that inhibition of PERK impairs GANT-61 induced autophagy in NB cells with MYCN amplification, but had no effect on the MYCN non-amplified NB cells. In summary, PERK seems to be a good therapeutic target for NB. Inhibition of PERK reduces autophagy in MYCN amplified NB cells, thus amplifying the efficacy of the GLI inhibitor GANT-61 in reducing proliferation of this type of cancer cells.