Reduced peroxisome proliferator-activated receptor-α and bile acid nuclear receptor NR1H4/FXR may affect the hepatic immune microenvironment of biliary atresia.

Background: Biliary atresia (BA) is a childhood liver disease characterized by fibrous obstruction and obstruction of the extrahepatic biliary system and is one of the most common and serious biliary disorders in infants. Significant inflammation and fibrosis of the liver and biliary tract are the most prominent features, regardless of the initial damage to the BA. Abnormalities in innate or adaptive immunity have been found in human patients and mouse models of BA. We previously reported that children with BA had abnormal lipid metabolism, including free serum carnitine. Objective: To study gene and protein expression levels of the hepatic peroxisome proliferator-activated receptor-α (PPARα) signaling pathway and farnesoid X receptor (FXR) in BA and BA fibrosis, and assess their clinical values. Methods: Low expression of PPARα and NR1H4 (FXR) in BA were validated in the Gene Expression Omnibus database. Functional differences were determined by gene set enrichment analysis based on of PPARα and NR1H4 expression. BA patients from GSE46960 were divided into two clusters by using consensus clustering according to PPARα, NR1H4, and SMAD3 expression levels, and immunoinfiltration analysis was performed. Finally, 58 cases treated in our hospital were used for experimental verification. (IHC: 10 Biliary atresia, 10 choledochal cysts; PCR: 10 Biliary atresia, 14 choledochal cysts; WB: 10 Biliary atresia, 4 choledochal cysts). Results: Bioinformatics analysis showed that the expression of PPARα, CYP7A1 and NR1H4 (FXR) in the biliary atresia group was significantly lower than in the control group. More BA-specific pathways, including TGFß signaling pathway, P53 signaling pathway, PI3K-AKT-mTOR signaling pathway, etc., are enriched in BA patients with low PPARα and NR1H4 expression. In addition, low NR1H4 expression is abundant in inflammatory responses, IL6/STAT3 signaling pathways, early estrogen responses, IL2 STAT5 signaling pathways, and TGFß signaling pathways. The TGFß signaling pathway was significant in both groups. According to the expression of PPARα, NR1H4 and SMAD3, a key node in TGFß pathway, BA patients were divided into two clusters using consensus clustering. In cluster 2, SMAD3 expression was high, and PPARα and NR1H4 expression were low. In contrast to cluster 1, immune cell infiltration was higher in cluster 2, which was confirmed by immunohistochemistry. The mRNA and protein levels of PPARα and NR1H4 in BA patients were lower than in the control group by immunohistochemistry, Western blot analysis and real-time PCR. Conclusions: The downregulation of PPARα and NR1H4 (FXR) signaling pathway may be closely related to biliary atresia.

Risk factors for pathological fractures of simple bone cysts of the humerus in children: a retrospective cohort study.

Background: Simple bone cysts (SBCs) are common osteolytic lesions in children, which often lead to pathological fractures of the non-weight-bearing bone (humerus) in patients. Cyst index is a classic index to evaluate the risk of pathological fracture, but the reliability of this index is reduced due to the deviation of cyst area in the frontal and lateral position and the subjectivity of the determination of cyst area. MRI can clearly show the cyst boundary, the appointment cycle is long, and children need to be examined under sedation, which cannot be judged by clinicians in time. Therefore, it is necessary to find a basic and simple imaging evaluation index in clinic. The purpose of this study is to retrospectively analyze the risk factors of pathological fracture of simple bone cyst of humerus in children. Methods: The medical records and plain films of patients with SBCs from January 2014 to December 2021 were retrospectively analyzed. Patients were divided into a fracture group (35 cases) and a non-fracture group (10 cases). The relationship between pathological fractures and the children's age, gender, and cyst index was analyzed. The presence of the pectoralis major and the link to the tendon insertion site of ectopectoralis and anconeus lateralis (TEAL) of the lesion were also examined. Perform binary logistic regression analysis. Results: Binary logistic regression analysis was performed on the age, gender, and cyst index, and whether the lesion was located in the TEAL. The results demonstrated that the TEAL was an independent risk factor for fractures [odds ratio (OR) =21.423, 95% confidence interval (CI): 2.409-190.539; P=0.006]. Conclusions: SBCs of humerus in children is often accompanied by pathological fracture. The lesion is located at TEAL, so we should be alert to the risk of pathological fracture.

Prognostic impact of tumor size on patients with neuroblastoma in a SEER-based study.

OBJECTIVE: The prognostic value of tumor size in neuroblastoma (NB) patients has not been fully evaluated. Our purpose is to elucidate the prognostic significance of tumor size in surgery performed on neuroblastoma patients. METHODS: Neuroblastoma patients diagnosed from 2004 to 2015 were selected from the Surveillance, Epidemiology, and End Results Program (SEER) for the study. Univariate and multivariate Cox proportional hazard regression models were used to identify risk factors and the independent prognostic influences of tumor size on NB patients. Overall survival (OS) was analyzed through univariate Cox regression analysis. To determine the optimal cutoff value of tumor size, we first divided the cohort into three groups (≤5 cm, 5-10 cm, >10 cm). Subsequently, the patients were divided into two groups repeatedly, with tumor size at 1 cm intervals. The cutoff value that maximized prognostic outcome difference was selected. Furthermore, we performed the Kaplan-Meier methods to visually present differences in prognosis between the optimal tumor size cutoff value in different subgroups. RESULTS: A total of 591 NB patients who met the inclusion criteria were selected from the SEER database in this study. Cox analysis showed that age >1 year (HR = 2.42, p < 0.0001), originate from adrenal site (HR = 1.7, p = 0.014), distant stage (HR = 6.4, p < 0.0001), undifferentiated grade (HR = 1.94, p = 0.002), and large tumor size (HR = 1.5, p < 0.0001) independently predicted poor prognosis. For tumor size, there were significant differences in tumor size distribution in different ages, tumor grade, disease stage, and primary site subgroup but not in sex, race, and histology subgroup. Furthermore, both univariate (HR = 4.96, 95% CI 2.31-10.63, p < 0.0001) and multivariable analysis (HR = 2.8, 95% CI 1.29-6.08, p < 0.0001) indicated the optimal cutoff value of tumor size was 4 cm for overall survival of NB patients. Using a 4 cm of tumor size cutoff in subgroups, we found that it can identify poor prognosis patients whatever their age or primary site. Interestingly, tumor size of 4 cm cutoff can only identify unfavorable NB patients with diagnosis at distant-stage disease, or differentiated grade tumor, but not with regional and local or undifferentiated tumor. CONCLUSIONS: Tumor size is first to be recognized as a key prognostic factor of neuroblastoma patients and a cutoff value >4 cm might predict poor prognosis, which should be included in the evaluation of prognostic factors for NB.

Primary omental torsion in children: single-center experience of 17 cases.

OBJECTIVE: Primary omental torsion (POT) is a rare condition in children often misdiagnosed because of a lack of clinical manifestations. Here we present insights gained from the treatment of pediatric POT cases at a single center over 8 years and summarize some influencing factors found in diagnosis and treatment, in order to reduce the misdiagnosis of POT in children in the future. METHODS: Demographic and clinical data of 17 children with POT treated in Shanghai Children's Hospital, Shanghai Jiaotong University from June 2012 to December 2020 were reviewed. RESULTS: The mean age of the 17 pediatric patients was 7.65 ± 2.12 years (range: 4-12 years), sixteen were male. The average time from the pain onset to operation was 73.82 ± 47.21 h (range: 21-144 h). In 5 cases, the ultrasound scan revealed a low-intensity inflammatory mass in the abdominal cavity. Only 1 of the 17 patients had normal body mass index, while others were overweight or obese. The mean computed tomography (CT) value of the mass in the lower right abdomen was - 58.74 ± 10.32 HU (range: - 70 to - 46 HU), which is close to that of its own abdominal wall fat [- 46.29 ± 9.45 HU (range: - 62 to - 32 HU)]. The location of the mass was located in front of the right colon in ten cases, except for two cases near the ligamentum teres. Five patients had whirl sign on CT images and 8 patients had pelvic fluid. Five cases were diagnosed as acute appendicitis and 12 were diagnosed as POT, the preoperative diagnosis was correct in 70.59% of cases. All 17 patients were treated with laparoscopic omentectomy. The average duration of hospitalization was 5.53 ± 1.12 days (range: 4-8 days). All cases were followed up. 1 case relapsed 8 months post operation, while the remaining cases had no complications. CONCLUSION: POT is rare in children with acute abdominal pain, which is more common in obese boys. The fatty mass in front of colon and between rectus abdominis sheath in CT image is specific, which is helpful for diagnosis of POT. Laparoscopy is an effective method for the treatment of POT in children.

Self-Reference Analysis Based on Temperature Difference Absorption Spectra.

The direct qualitative identification of pure liquids in laboratories and in security checks is generally performed by the detection of the refractive index or the permittivity. However, refractive indices are strongly influenced by temperature, while the permittivities of some organics are difficult to differentiate. On the other hand, the quantitative monitoring of samples with high concentration in plating baths and in chemical production lines are generally performed via a "Sampling-Dilution-Analysis" approach because of significant deviations from the linear range at high concentration, which makes the real-time monitoring of concentrated samples difficult. Here, we propose a self-reference analysis (SRA) method to directly analyze pure liquids and concentrated samples based on temperature difference absorption spectra (TDAS) without the need for dilution. This method was performed by simultaneously scanning the spectra of the reference and the sample, which are both obtained from the same analyte for detection but are at different temperatures. Compared to conventional absorption spectra with a blank reference, the red-shifted peak wavelengths of TDAS enable the detection of many far UV absorptive compounds in the near-ultraviolet region (λ > 190 nm). More importantly, organic compounds with similar structures can be easily distinguished. In addition, TDAS can also be used for the quantitative detection of concentrated analytes. The proposed SRA-TDAS method is a rapid and effective method; this approach does not require dilution and utilizes a self-reference, implying the wide potential applicability in security checks, and the real-time monitoring of concentrated compounds in chemical production lines.

Construction of an integrated map of Haliotis diversicolor using microsatellite markers.

Small abalone, Haliotis diversicolor, is naturally distributed along the coastal waters of East Asia from Japan to the Philippines. It is an economically important maricultured species in southern China and Taiwan. Genetic linkage maps for small abalone were constructed using a total of 308 simple sequence repeat markers including 297 novel markers. Segregation data on 96 progeny were genotyped using a pseudo-testcross strategy. Sixteen linkage groups were identified in both female and male maps, consistent with the haploid chromosome number. The female linkage map covered 758.3 cM, with an average interval of 5.2 cM. The male linkage map spanned a total genetic distance of 676.2 cM, with an average interval of 4.5 cM. An integrated map was constructed by incorporating the homologous parental linkage groups, resulting in 16 linkage groups with a total of 762.1 cM. Genome coverage of the integrated linkage map was approximately 80.7%. The genetic linkage maps of small abalone may facilitate marker-assisted selection and quantitative trait loci mapping.

Epidemiology and vector efficiency during a dengue fever outbreak in Cixi, Zhejiang Province, China.

An emigrant worker returning from Southeast Asia triggered the outbreak of a DF epidemic in Zhejiang province, China, in October, 2004. Eighty-three cases, mainly young and middle-aged people between 20 and 50 (78.3%), were reported in the area of Cixi. There were no obvious occupational patterns. The majority of cases were female, with a sex ratio of 1:1.86 (m:f). The dengue virus (DENV) strains from the epidemic area were isolated and identified as DENV-1, which belongs to Asian strain 1. According to the epidemiological investigation, the incidence of DF had no relationship to temperature, humidity, or precipitation, and the Breteau index of larvae showed a clear relationship only with the House Index and Container Index. Recent dengue problems in the town have been associated with the complex social factors and hygienic conditions for endemic villagers and immigrant workers. Some hygienic measures should be taken by the local government to reduce the risk of mosquito-borne disease. These measures should aim to eliminate the breeding sites of the vector Aedes albopictus in indoor and outdoor containers filled with rainwater and thus reducing the risk of DF transmission.