3D Aligned Nanofiber Scaffold Fabrication with Trench-Guided Electrospinning for Cardiac Tissue Engineering.

Constructing three-dimensional (3D) aligned nanofiber scaffolds is significant for the development of cardiac tissue engineering, which is promising in the field of drug discovery and disease mechanism study. However, the current nanofiber scaffold preparation strategy, which mainly includes manual assembly and hybrid 3D printing, faces the challenge of integrated fabrication of morphology-controllable nanofibers due to its cross-scale structural feature. In this research, a trench-guided electrospinning (ES) strategy was proposed to directly fabricate 3D aligned nanofiber scaffolds with alternative ES and a direct ink writing (DIW) process. The electric field effect of DIW poly(dimethylsiloxane) (PDMS) side walls on guiding whipping ES nanofibers was investigated to construct trench design rules. It was found that the width/height ratio of trenches greatly affected the nanofiber alignment, and the trench width/height ratio of 1.5 provided the nanofiber alignment degree over 60%. As a proof of principle, 3D nanofiber scaffolds with controllable porosity (60-80%) and alignment (30-60%) were fabricated. The effect of the scaffolds was verified by culturing human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs), which resulted in the uniform 3D distribution of aligned hiPSC-CMs with ∼1000 µm thickness. Therefore, this printing strategy shows great potential for the efficient engineered tissue construction.

Qingjie Huagong decoction inhibits pancreatic acinar cell pyroptosis by regulating circHipk3/miR-193a-5p/NLRP3 pathway.

BACKGROUND: Safer and more effective drugs are needed for the treatment of acute pancreatitis (AP). Qingjie Huagong decoction (QJHGD) has been applied to treat AP for many years and has shown good clinical effects. However, the potential mechanism has not yet been determined. PURPOSE: To investigate the role and underlying mechanism of the effects of QJHGD on AP both in vitro and in vivo. METHODS: QJHGD was characterized by UHPLC-Q-Orbitrap-MS. The protective effect of QJHDG and the underlying mechanism were investigated in MPC-83 cells in vitro. A caerulein-induced AP model was established to evaluate the protective effect of QJHGD in mice. CCK-8 assays were used to detect cell viability. The contents of inflammatory mediators were determined by ELISA. Expression levels of circRNA, miRNA and mRNA were determined by qRT-PCR. Protein expression was determined using Western blot. Pancreatic tissues were assessed by hematoxylin and eosin staining as well as immunohistochemical and immunofluorescence analyses. Pull-down and luciferase activity assays were performed to determine the regulatory relationships of circHipk3, miR-193a-5p and NLRP3. RESULTS: Our results confirmed that mmu-miR-193a-5p was sponged by mmu-circHipk3, and NLRP3 was a target of miR-193a-5p. In vitro experiments showed that QJHGD enhanced MPC-83 cell viability by regulating circHipk3 sponging mir-193a-5 targeting NLRP3 and inhibiting pyroptosis-related factors. Finally, we showed that QJHGD ameliorated pancreatic tissue injury in AP mice via this pathway. CONCLUSION: This study demonstrate that QJHDG exerted its anti-AP effects via the circHipk3/miR-193a-5p/NLRP3 pathway, revealing a novel mechanism for the therapeutic effect of QJHDG on AP.

Exploring the mechanism of LncRNA CASC15 affecting hepatocellular carcinoma through miRNA.

This study aimed to determine the potential mechanisms through which long noncoding (Lnc) RNA cancer susceptibility candidate 15 (CASC15) affects hepatocellular carcinoma (HCC). We retrieved HCC RNA-seq and clinical information from the UCSC Xena database. The differential expression (DE) of CASC15 was detected. Overall survival was analyzed using Kaplan-Meier (K-M) curves. Molecular function and signaling pathways affected by CASC15 were determined using Gene Set Enrichment Analysis. Associations between CASC15 and the HCC microenvironment were investigated using immuno-infiltration assays. A differential CASC15-miRNA-mRNA network and HCC-specific CASC15-miRNA-mRNA ceRNA network were constructed. The overexpression of CASC15 in HCC tissues was associated with histological grade, clinical stage, pathological T stage, poor survival, more complex immune cell components, and 12 immune checkpoints. We identified 27 DE miRNAs and 270 DE mRNAs in the differential CASC15-miRNA-mRNA network, and 10 key genes that were enriched in 12 cancer-related signaling pathways. Extraction of the HCC-specific CASC15-miRNA-mRNA network revealed that IGF1R, MET, and KRAS were associated with HCC progression and occurrence. Our bioinformatic findings confirmed that CASC15 is a promising prognostic biomarker for HCC, and elevated levels in HCC are associated with the tumor microenvironment. We also constructed a disease-specific CASC15-miRNA-mRNA regulatory ceRNA network that provides a new perspective for the precise indexing of patients with elevated levels of CASC15.

Mechanical behavior of an FGM-type frozen soil wall: Theory and numerical analysis.

With a laminate model foundation, we have used the complex variable function method to calculate the boundary displacement and stress of a frozen soil wall in a horizontal connecting passage. Using an actual engineering case, the effects of the number of divided layers of a functionally graded material-type frozen soil wall, the position of the freezing pipe and the section shape of the connecting passage on the displacements and tangential stresses of the frozen soil wall are discussed. The results indicate that the frozen soil wall as a temporary support structure exhibits a good supporting effect. With the increase of layers, the material strength of the frozen soil wall weakens, and the displacements and tangential stresses of the inner boundary increase. When the midline of the freezing pipe moves toward the inner boundary, the tensile area in the frozen soil wall begins to shift, and the displacements and tangential stresses of the inner boundary decrease differently. Thedistributions of internal boundary displacements and tangential stresses are significantly affected by the section shape of the frozen soil wall, and the internal boundary displacements and tangential stresses of the frozen soil wall of the small section are more uniform than those of the frozen soil wall of the large section.

Prognostic significance of LRRC1 in hepatocellular carcinoma and construction of relevant prognostic model.

This study aimed to elucidate the prognostic value of the leucine rich repeat containing 1 (LRRC1) gene in hepatocellular carcinoma (HCC) and to determine the effects of high and low LRRC1 expression on mutation and immune cell infiltration. We downloaded HCC mRNA-seq expression and clinical data from University of California Santa Cruz Xena. The expression of LRRC1 was compared between HCC tumor and normal samples. Tumor samples were divided according to high and low LRRC1 expression. Differentially expressed genes between the 2 groups were identified, and function, mutation, and immune cell infiltration were analyzed. Genes associated with immune cells were identified using weighted gene co-expression network analysis, and transcription factors of these genes were predicted. Moreover, a prognostic model was developed and its performance was evaluated. The expression of LRRC1 was upregulated in HCC tissues, and this indicated a poor prognosis for patients with HCC. Differentially expressed genes between high and low LRRC1 expression were significantly enriched in pathways associated with cancer, amino acid metabolism, carbohydrate metabolism, and the immune system. We identified 15 differentially infiltrated immune cells between tumors with high and low LRRC1 expression and 14 of them correlated with LRRC1 gene expression. Weighted gene co-expression network analysis identified 83 immune cell-related genes, 27 of which had prognostic value. Cyclic AMP-response element binding protein regulated annexin A5, matrix metallopeptidase 9, and LRRC1 in the transcription factor regulatory network. Finally, a prognostic model composed of 7 genes were generated, which could accurately predict the prognosis of HCC patients. The LRRC1 gene might serve as a potential immune-associated prognostic biomarker for HCC.

Hydrothermal carbonization of food waste for sustainable biofuel production: Advancements, challenges, and future prospects.

With the improvement of living standards, food waste (FW) has become one of the most important organic solid wastes worldwide. Owing to the high moisture content of FW, hydrothermal carbonization (HTC) technology that can directly utilize the moisture in FW as the reaction medium, is widely used. Under mild reaction conditions and short treatment cycle, this technology can effectively and stably convert high-moisture FW into environmentally friendly hydrochar fuel. In view of the importance of this topic, this study comprehensively reviews the research progress of HTC of FW for biofuel synthesis, and critically summarizes the process parameters, carbonization mechanism, and clean applications. Physicochemical properties and micromorphological evolution of hydrochar, hydrothermal chemical reactions of each model component, and potential risks of hydrochar as a fuel are highlighted. Furthermore, carbonization mechanism of the HTC treatment process of FW and the granulation mechanism of hydrochar are systematically reviewed. Finally, potential risks and knowledge gaps in the synthesis of hydrochar from FW are presented and new coupling technologies are pointed out, highlighting the challenges and prospects of this study.

Machine learning modeling identifies hypertrophic cardiomyopathy subtypes with genetic signature.

Previous studies have revealed that patients with hypertrophic cardiomyopathy (HCM) exhibit differences in symptom severity and prognosis, indicating potential HCM subtypes among these patients. Here, 793 patients with HCM were recruited at an average follow-up of 32.78 ± 27.58 months to identify potential HCM subtypes by performing consensus clustering on the basis of their echocardiography features. Furthermore, we proposed a systematic method for illustrating the relationship between the phenotype and genotype of each HCM subtype by using machine learning modeling and interactome network detection techniques based on whole-exome sequencing data. Another independent cohort that consisted of 414 patients with HCM was recruited to replicate the findings. Consequently, two subtypes characterized by different clinical outcomes were identified in HCM. Patients with subtype 2 presented asymmetric septal hypertrophy associated with a stable course, while those with subtype 1 displayed left ventricular systolic dysfunction and aggressive progression. Machine learning modeling based on personal whole-exome data identified 46 genes with mutation burden that could accurately predict subtype propensities. Furthermore, the patients in another cohort predicted as subtype 1 by the 46-gene model presented increased left ventricular end-diastolic diameter and reduced left ventricular ejection fraction. By employing echocardiography and genetic screening for the 46 genes, HCM can be classified into two subtypes with distinct clinical outcomes.

Identification of molecular subtypes and prognostic signatures based on transient receptor potential channel-related genes to predict the prognostic risk of hepatocellular carcinoma: A review.

Abnormal transient receptor potential (TRP) channel function interferes with intracellular calcium-based signaling and causes malignant phenotypes. However, the effects of TRP channel-related genes on hepatocellular carcinoma (HCC) remain unclear. This study aimed to identify HCC molecular subtypes and prognostic signatures based on TRP channel-related genes to predict prognostic risks. Unsupervised hierarchical clustering was applied to identify HCC molecular subtypes using the expression data of TRP channel-related genes. This was followed by a comparison of the clinical and immune microenvironment characteristics between the resulting subtypes. After screening for differentially expressed genes among subtypes, prognostic signatures were identified to construct risk score-based prognostic and nomogram models and predict HCC survival. Finally, tumor drug sensitivities were predicted and compared between the risk groups. Sixteen TRP channel-related genes that were differentially expressed between HCC and non-tumorous tissues were used to identify 2 subtypes. Cluster 1 had higher TRP scores, better survival status, and lower levels of clinical malignancy. Immune-related analyses also revealed higher infiltration of M1 macrophages and higher immune and stromal scores in Cluster 1 than in Cluster 2. After screening differentially expressed genes between subtypes, 6 prognostic signatures were identified to construct prognostic and nomogram models. The potential of these models to assess the prognostic risk of HCC was further validated. Furthermore, Cluster 1 was more distributed in the low-risk group, with higher drug sensitivities. Two HCC subtypes were identified, of which Cluster 1 was associated with a favorable prognosis. Prognostic signatures related to TRP channel genes and molecular subtypes can be used to predict HCC risk.

Characteristics and treatment of congenital perineal groove in male patients.

Background: Congenital perineal groove (CPG) of male patients has rarely been reported before. The purpose of this study was to review our cases and describe their characteristics and treatment. Methods: Four male patients diagnosed with CPG were included in this study. Medical records were retrospectively reviewed. Type of CPG and anal position index (API) of the patients were recorded. Follow-up was through outpatient visits. Results: Their age ranged from 4 years and 2 months to 10 years and 9 months. Among the four patients, two complained of intermittent CPG mucosal hemorrhage and the other two had mucous secreting and soiling. The API was 0.24, 0.35, 0.36, and 0.40 for each patient, respectively, all represented anterior displacement. Type of CPG for the four patients were all partial, and the sulcus was from the posterior perineum to the edge of anus. Two patients were associated with hydrocele, imperforated anus, and rectoperineal fistula; one patient had left varicocele; the remaining patient had sacrum split. All the patients had no postoperative complication, and during the follow-up period of 5-8 months, no symptoms recurred in the four patients; they all had normal defecation. Conclusions: Both genders share the common three characteristics. In addition, shortened perineum with anterior anus, association of perineal malformations, and partial type occurrence are the extra morphological features in male patients. Furthermore, CPG in males are rarely accompanied by urinary tract infection. Favorable prognosis could be reached after operation.

Selection of operative approach in children with Currarino syndrome.

PURPOSE: To summarize the experience of surgical treatment of children diagnosed with Currarino syndrome, with an emphasis on the selection of an optimal operative approach. METHODS: The clinical materials of patients diagnosed with Currarino syndrome were recorded. Special attention was given to the operative management, particularly the different routes for operation. The type of ARM was the critical point. The Rintala score was used for the evaluation of bowel function. RESULTS: The medical records of 26 patients were reviewed. Seven were male, and 19 were female, with a mean age of 19.38 ± 13.80 months. The standard posterior sagittal approach (SPS) group included three perineal fistulae, one anal stenosis, one retraction of the rectum after anoplasty for vestibular fistula, one ARM with no fistula, one rectourethral fistula, and one cloaca. In the limited posterior sagittal approach (LPS) group, there were 13 perineal fistulae, one displacement of the rectum, and one retraction of the rectum after anoplasty for the vestibular fistula. In addition, the transanal approach (TA) and anterior sagittal approach (AS) were also used. The mean follow-up time was 39.48 ± 26.84 m. The Rintala score was 16.74 ± 2.93. CONCLUSION: For a perineal fistula, SPS or LPS should be used to reach anoplasty and remove the presacral mass. For a vestibular fistula, the AS or LPS should be chosen. For anal stenosis, SPS or LPS should be used.

Nanofiber self-consistent additive manufacturing process for 3D microfluidics.

3D microfluidic devices have emerged as powerful platforms for analytical chemistry, biomedical sensors, and microscale fluid manipulation. 3D printing technology, owing to its structural fabrication flexibility, has drawn extensive attention in the field of 3D microfluidics fabrication. However, the collapse of suspended structures and residues of sacrificial materials greatly restrict the application of this technology, especially for extremely narrow channel fabrication. In this paper, a 3D printing strategy named nanofiber self-consistent additive manufacturing (NSCAM) is proposed for integrated 3D microfluidic chip fabrication with porous nanofibers as supporting structures, which avoids the sacrificial layer release process. In the NSCAM process, electrospinning and electrohydrodynamic jet (E-jet) writing are alternately employed. The porous polyimide nanofiber mats formed by electrospinning are ingeniously applied as both supporting structures for the suspended layer and percolating media for liquid flow, while the polydimethylsiloxane E-jet writing ink printed on the nanofiber mats (named construction fluid in this paper) controllably permeates through the porous mats. After curing, the resultant construction fluid-nanofiber composites are formed as 3D channel walls. As a proof of concept, a microfluidic pressure-gain valve, which contains typical features of narrow channels and movable membranes, was fabricated, and the printed valve was totally closed under a control pressure of 45 kPa with a fast dynamic response of 52.6 ms, indicating the feasibility of NSCAM. Therefore, we believe NSCAM is a promising technique for manufacturing microdevices that include movable membrane cavities, pillar cavities, and porous scaffolds, showing broad applications in 3D microfluidics, soft robot drivers or sensors, and organ-on-a-chip systems.

Co-hydrothermal carbonization of organic solid wastes to hydrochar as potential fuel: A review.

The organic solid waste (OSW) is a potential resource that loses its original value in people's daily production process. It can be used for secondary energy utilization through hydrothermal technology, which is similar to artificially simulating the natural coalification process. Co-hydrothermal carbonization (co-HTC) is a promising thermochemical conversion pathway, and advanced mechanisms can eliminate the drawbacks of single-feedstock hydrothermal carbonization (HTC). The preparation and production process of hydrochar can solve the problems of energy crisis and environmental pollution. This paper comprehensively reviews the key mechanisms of co-HTC to prepare solid fuels, and reviews the development process and practical application of hydrothermal technology. To begin with, the physical and chemical properties and combustion performance of co-hydrochar depend on the production method, process parameters, and selection of raw materials. The co-hydrochar usually has a higher HHV and a low atomic ratio of H/C and O/C, which improves combustion performance. Subsequently, the transformation path of the hydrothermal process of lignocellulosic and protein OSW was comprehensively expounded, and the reaction mechanism of the co-HTC of the two OSWs was effectively proposed. The effect of the ratio of different raw materials on the synergistic effect of co-HTC was also analyzed. Furthermore, the typical advantages and disadvantages of environmental safety, technical economy, and practical application in the co-HTC process are expounded. All in all, this review provides some foundations and new directions for the co-HTC of OSWs to prepare potential fuel. In addition, several prospects for the development and integrated application of co-HTC are presented in the future.

Epifriedelinol Ameliorates the Neuropathic Pain and Recovers the Function in Spinal Cord Injury by Downregulation of Neuronal Apoptosis and NMDA Receptor.

Spinal cord injury (SCI) is commonly associated with neuropathic pain, which affects large population. Thus, the presented investigation evaluates the beneficial effect of epifriedelinol against SCI-associated neuropathic pain. SCI injury was induced in rats by clip-compression and rats were treated with epifriedelinol 100 and 200 mg/kg, i.p. for 21 days after the induction of SCI. The effect of epifriedelinol was assessed on neuropathic pain by mechanical allodynia and locomotor function. Level of inflammatory cytokines were assessed in the neuronal tissue using enzyme linked immunosorbent assay (ELISA) and expression of caspase-3 and Bcl2 protein were assessed by western blot assay. Data of investigation reveals that epifriedelinol reduces mechanical allodynia in SCI injured rats. Moreover, it also improves locomotor function in SCI injured rats. There was significant decrease in level of interleukin (IL)-1ß, IL-6 and tumor necrosis factor (TNF)-α in the neuronal tissues of epifriedelinol-treated group than negative control group. Moreover, treatment with epifriedelinol ameliorates the altered expression of caspase 3, Bcl2 and GluN1 and level of glutamate in neuronal tissue of SCI-injured rats. In conclusion, data reveal that epifriedelinol treatment protects neuropathic pain associated with spinal cord injury by downregulating the N-methyl-D-aspartate (NMDA) receptor function.

Caudal duplication syndrome: 10-year experiences with a comprehensive literature review.

PURPOSE: Caudal duplication syndrome (CDS) has rarely been reported. The purpose was to describe the characteristics and discuss possible pathogenesis of CDS by reviewing our experience along with a comprehensive literature review. METHODS: A total of 51 patients including 3 from our team and 48 from literature were selected in this study. General condition, clinical manifestations, type of anomalies, treatment and prognosis was analyzed and summarized. RESULTS: Among the 51 patients were 30 females and 21 males, and age at first clinical visit was from birth to 39 years old. Except 12 patients, most of the patients had no troubling clinical manifestation. Physical examination showed that 30 patients had 1 perineum, 21 patients had 2 completely independent perineums. Degree of duplication varied; colon-rectum tubular, bladders and urethras, vaginas in females and penis shafts and glans in males were found to be the most common type of alimentary system and urogenital system duplication in this study with 24/51, 41/51, 10/30 and 16/21 patients, respectively. Anorectal malformation was calculated: 18 had 2 ARMs, 14 had 1 normal anus and 1 ARM on the other side, 12 had a normal anus, 5 had 2 normal anus, the remaining 2 patients had only 1 ARM. Spinal cord anomalies were showed as meningomyeloceles and lipomas in 13 and 3 patients. Vertebral anomalies of bifid, dysplasias, scoliosis, and hemivertebra were noticed in 28 patients and accessory dysplasia lower limbs were found in 10 patients. Prognosis showed 39 of the 51 patients had normal function in urination and defecation. CONCLUSIONS: CDS is an extremely rare disease with uncertain pathogenesis. Colon-rectum tubular duplication with two ARMs, duplicated bladders and urethras, double vaginas in females and penis shafts and glans in males are the most common type. Long-term prognosis is good with multidisciplinary, individualized and staged surgical procedures.

The Involvement of ALPK3 in Hypertrophic Cardiomyopathy in East Asia.

Objective: ALPK3 is associated with a recessive form of pediatric cardiomyopathy accompanied by musculoskeletal and craniofacial abnormalities. Heterozygous truncating variants in this gene (ALPK3tv) have recently been confirmed as a cause of autosomal dominant hypertrophic cardiomyopathy (HCM). Whether ALPK3 is also implicated in HCM in East Asia and the effect of missense variants in ALPK3 on HCM remains unresolved. Methods: We compared the frequency of rare deleterious variants in ALPK3 in a study cohort comprised of 793 HCM cases of East Asian descent to that in the controls subset of Genome Aggregation Database (gnomAD). Gene burden test was used to assess this association. The involvement of these variants in HCM was further validated by independent cohort. The clinical characteristics and prognoses of these carriers were compared with sarcomere-positive and negative patients. Results: Rare deleterious variants in ALPK3 were significantly enriched in HCM compared with gnomAD controls (truncating: 4/793 vs. 4/4523, P = 0.02; missense: 25/793 vs. 46/4523, P = 2.56e-5). Replication in an independent cohort provided more supporting evidence. Further comparisons revealed that ALPK3 carriers displayed more severe hypertrophy in interventricular septum (IVS) and apex, as well as greater maximal left ventricular wall thickness, relative to sarcomere negatives. Conclusion: Heterozygous rare variants in ALPK3, both missense and truncating variants, are associated with HCM in East Asians.

Diagnosis and treatment of splenic torsion in children: preoperative thrombocytosis predicts splenic infarction.

BACKGROUND: Pediatric splenic torsion is a rare entity, and the most common cause is wandering spleen. This study aimed to summarize our clinical experience in the diagnosis and surgical treatment pediatric patients with splenic torsion, and to use preoperative thrombocytosis as a preoperative predictive factor for splenic infarction. METHODS: From January 1st, 2016 to December 31st, 2021, 6 children diagnosed as splenic torsion were included. All patients were surgically treated and followed up. The clinical data was collected including clinical presentations, laboratory tests, imaging results, surgical procedures, and prognosis. Clinical experience of diagnosis and surgical treatment were summarized. RESULTS: There were 4 females and 2 males, with median age at surgery 102.6 (range 9.4-170.7) months. Abdominal pain and abdominal mass were the most common presentations. The diagnosis of splenic torsion depended on imaging studies, and adjacent organ involvement (gastric and pancreas torsion) was observed on contrast CT in one patient. Five patients were diagnosed as torsion of wandering spleen, and one was torsion of wandering accessory spleen. Emergent laparoscopic or open splenectomy was performed in all patients. Pathology revealed total splenic infarction in 4 patients, partial infarction in 1 patient, and viable spleen with congestion and hemorrhage in 1 patient. Preoperative platelet counts were elevated in all 4 patients with splenic infarction, but normal in the rest 2 with viable spleen. Postoperative transient portal vein branch thromboembolism occurred in one patient. CONCLUSIONS: Imaging modalities are crucial for the diagnosis of pediatric splenic torsion and adjacent organ involvement. Preoperative thrombocytosis may predict splenic infarction. Spleen preserving surgery should be seriously considered over splenectomy in patients with a viable spleen.

Surgical treatment of benign splenic lesions in pediatric patients: a case series of 30 cases from a single center.

BACKGROUND: Benign splenic lesions are rarely encountered. This study aimed to review the clinical characteristics and surgical outcomes in a case series of 30 pediatric patients. METHODS: From January 1st, 2001 to December 31st, 2021, 30 pediatric patients from a single center were consecutively included. Electronic medical records were reviewed and patients were followed up. Clinical presentations, imaging features, surgical procedures, pathological diagnoses, and prognoses were summarized. The lesion locations and 7-day postoperative platelet levels were compared between total and partial splenectomy patients. RESULTS: Eighteen males and twelve females were included, with mean age at surgery 116.4 ± 43.6 months. The clinical presentations included abdominal pain (16/30), splenomegaly (6/30), skin petechia (2/30), hemolytic jaundice (1/30), and no symptoms (5/30). Pathological diagnoses included congenital epithelial cyst (CEC, 17/30), vascular malformation (8/30), sclerosing angiomatoid nodular transformation (SANT, 3/30), hamartoma (1/30), and leiomyoma (1/30). Patients undergone total splenectomy were more likely to have a lesion involving the hilum than those undergone partial splenectomy (68.4% vs 31.6%, P = 0.021). The 7-day postoperative platelet level was higher in total splenectomy patients than partial splenectomy patients (adjusted means 694.4 × 109/L vs 402.4 × 109/L, P = 0.002). CONCLUSIONS: Various clinical characteristics of pediatric benign splenic lesions are summarized. The most common pathological diagnoses are congenital epithelial cyst and vascular malformation. Partial and total splenectomy result in good prognosis with a low recurrence rate, and the former is preferred to preserve splenic function if possible.

[Research progress of Curcuma kwangsiensis root tubers and analysis of liver protection and anti-tumor mechanisms based on Q-markers].

Curcuma kwangsiensis root tuber is a widely used genuine medicinal material in Guangxi, with the main active components of terpenoids and curcumins. It has the effects of promoting blood circulation to relieve pain, moving Qi to relieve depression, clearing heart and cooling blood, promoting gallbladder function and anti-icterus. Modern research has proved its functions in liver protection, anti-tumor, anti-oxidation, blood lipid reduction and immunosuppression. Considering the research progress of C. kwangsiensis root tubers and the core concept of quality marker(Q-marker), we predicted the Q-markers of C. kwangsiensis root tubers from plant phylogeny, chemical component specificity, traditional pharmacodynamic properties, new pharmacodynamic uses, chemical component measurability, processing methods, compatibility, and components migrating to blood. Curcumin, curcumol, curcumadiol, curcumenol, curdione, germacrone, and ß-elemene may be the possible Q-markers. Based on the predicted Q-markers, the mechanisms of the liver-protecting and anti-tumor activities of C. kwangsiensis root tubers were analyzed. AKT1, IL6, EGFR, and STAT3 were identified as the key targets, and neuroactive ligand-receptor interaction signaling pathway, nitrogen metabolism pathway, cancer pathway, and hepatitis B pathway were the major involved pathways. This review provides a basis for the quality evaluation and product development of C. kwangsiensis root tubers and gives insights into the research on Chinese medicinal materials.

Whole-exome sequencing reveals genetic risks of early-onset sporadic dilated cardiomyopathy in the Chinese Han population.

To reveal genetic risks of early-onset sporadic dilated cardiomyopathy (DCM) patients in the Chinese Han population, we enlisted 363 DCM cases and 414 healthy controls. Whole-exome sequencing and phenotypic characterization were conducted. In total, we identified 26 loss-of-function (LOF) candidates and 66 pathogenic variants from 33 genes, most of which were novel. The deleterious variants can account for 25.07% (91/363) of all patients. Furthermore, rare missense variants in 21 genes were found to be significantly associated with DCM in burden tests. Other than rare variants, twelve common SNPs were significantly associated with an increased risk of DCM in allele-based genetic model association analysis. Of note, in the cumulative risk model, high-risk subjects had a 3.113-fold higher risk of developing DCM than low-risk subjects. Also, DCM in the high-risk group had a younger age of onset than that in the low-risk group. In terms of cardiac function, the mean left ventricular ejection fraction of patients with the deleterious variants was lower than those without (27.73%±10.02% vs. 30.61%±10.85%, P=0.026). To conclude, we mapped a comprehensive atlas of genetic risks in Chinese patients with DCM that might lead to new insights into the mechanisms and risk stratification for DCM.

The utility of shear wave elastography and serum biomarkers for diagnosing biliary atresia and predicting clinical outcomes.

To investigate the utility of liver stiffness measurement by shear wave elastography (SWE) and several commonly used biomarkers in differentiating biliary atresia (BA) from other causes of cholestasis (non-BA) patients within 45 days and in predicting the postoperative prognosis. A consecutive series of medical records of patients presenting with cholestasis within 45 days in our institution between February 2016 and December 2020 was collected. The BA diagnosis was confirmed by intraoperative cholangiography (IOC). Other causes of cholestasis were confirmed by IOC, liver biopsy, genetic analysis, or recovery after conservative treatment. Preoperative and postoperative data were analyzed. A total of 156 patients were included, consisting of BA (n = 83) and non-BA (n = 73) cases. SWE and serum gamma-glutamyl transferase (GGT) showed better discriminative utility. The optimal cutoff values for SWE and GGT were > 7.10 kPa and > 195.4 U/L, with AUC of 0.82 (95% CI, 0.76-0.89; p < 0.0001) and 0.87 (95% CI, 0.82-0.93; p < 0.0001), respectively. Subgroup analysis showed the increased discriminative performance of SWE with age. Multivariable logistic regression analysis showed better diagnostic performance for SWE (adjusted OR, 35.03; 95% CI, 7.12-172.50) and GGT (adjusted OR, 24.70; 95% CI, 6.55-93.18) after adjusting for other confounders. The 30-day postoperative to preoperative serum direct bilirubin (DB) level, DB (post-30:pre), of > 0.3 showed the best predictive value for the need of liver transplantation, with HR of 6.15 (95% CI 1.95-19.38, P = 0.042).Conclusion: Serum GGT level and liver stiffness measurement by SWE showed the best discriminative utility. The diagnostic performance of SWE increased with age. A DB (post-30:pre) value > 0.3 was associated with the need for liver transplantation in later life. What is Known: • Liver stiffness measurement by shear wave elastography (SWE) could help discriminate biliary atresia (BA) from other causes of cholestasis, with sensitivity of 70-90%. • The postoperative total bilirubin less than 2 mg/dL within the first 3 months was a predictor of transplant-free survival. What is New: • The diagnostic performance of liver stiffness measurement by SWE increased with age. • The 30-day postoperative direct bilirubin (DB) level to preoperative DB level, DB (post-30:pre), is a predictor for short-term clinical outcomes.