Current landscape of primary small bowel leiomyosarcoma: cases report and a decade of insights.

The incidence of leiomyosarcoma (LMS) is about 4-5/100,000 individuals per year. LMSs occurring in the small bowel are even rarer, and their preoperative diagnosis is very difficult. We described two patients with pathologically confirmed small bowel LMS and analyzed their clinical and medical imaging features. Similar cases reported in English in Pubmed database over the past decade were reviewed and summarized. These tumors were categorized by the growth direction and relationship with the intestinal lumen into three types: intraluminal (n = 10), intermural (n = 3), and extraluminal (n = 7). Notably, among the three types of LMS, the intramural leiomyosarcoma stands out as a noteworthy subtype. Emerging evidence suggests that smaller tumor size (< 5 cm) and the intraluminal type may serve as favorable prognostic indicators, while the extraluminal type is associated with relatively poor prognosis. Furthermore, the integration of imaging features with CA125 and LDH biomarkers holds promise for potential diagnostic value in LMS.

Primary leiomyosarcoma of the thyroid gland: A rare case report and literature review.

INTRODUCTION: Primary thyroid leiomyosarcoma is an extremely rare soft tissue sarcoma, characterized by high malignancy and poor prognosis. Currently, only 13 cases of primary thyroid leiomyosarcoma have been described in the medical literature (limited to English). CASE PRESENTATION: A 76-year-old female presented with a giant neck mass. Physical examination revealed a large, firm mass in the left thyroid gland. No symptoms such as hoarseness or dysphagia were noted at the time of presentation. The patient underwent unilateral thyroidectomy and cervical lymph node dissection. CLINICAL DISCUSSION: Pathologic findings revealed a low-grade sarcoma with spindle-shaped tumor cells in an interwoven, sheet-like distribution. Immunohistochemistry showed positivity for desmin, SMMHC, STAT6, CK19, and Galectin3, but negativity for S-100, MyoD1, CD34, CK (AE1/AE3), CD117, and CD56. The findings were consistent with thyroid leiomyosarcoma. CONCLUSION: The treatment of primary thyroid LMS presents challenges due to its atypical symptoms and high malignance, highlighting the imperative for further exploration of therapeutic strategies to improve the outcomes.

Opting for conservative management over surgery for neonatal ovarian cysts.

OBJECTIVE: To explore the suitability of conservative management for neonatal ovarian cysts in newborns. METHODS: A retrospective cohort study was conducted, involving infants diagnosed with neonatal abdominal/pelvic cysts at two separate medical institutions from January 2015 through July 2021. Data collection included clinical characteristics, imaging results, pathological findings, and postnatal outcomes. Statistical analyses were performed using the Student's t-test, Mann-Whitney U-test, and receiver operating characteristic (ROC) curve. RESULTS: In total, 34 cases of neonatal abdominal/pelvic cystic masses were detected, with mean birth weight of 3401 ± 515 g. Of these, 22 patients underwent postnatal cystectomy/oophorectomy. Pathological assessments revealed 16 uncomplicated cysts, 5 complex cysts, and 1 ovarian cyst with torsion complications. Notably, the cysts' dimensions at the time of surgical intervention had significantly decreased from the initial measurements (p = 0.015). The ROC curve analysis presented an area under the curve of 0.642, indicating moderate accuracy in employing cyst size as a discriminative feature to differentiate complex from simple ovarian cysts. Additionally, a short-term follow-up of nonsurgical cases indicated a 100% resolution rate by 24 months of age (n = 9). CONCLUSION: Given their predominantly benign nature, the majority of neonatal ovarian cysts seem to be amenable to conservative management. This approach remains justified for larger cysts with minimal torsion risk, as well as considering the observed reduction in cyst size at birth, which further supports the case against surgical intervention.

Effect of WeChat-Based Medication Guidance on Symptoms and Serological Parameters in Children with Mycoplasma Pneumoniae.

Objective: The aim of this study was to assess the effect of microsoft-based medication guidance on the level of symptoms and serological indicators in children receiving budesonide nebulisation combined with terbutaline for the treatment of Mycoplasma pneumoniae pneumoniae (MPP). Methods: A total of 109 children with MPP treated in The First Affiliated Hospital of Ningbo University of China between October 2022 and April 2023 were divided into the conventional group (n=54, with medication guidance by telephone follow-up) and the WeChat group (n=55, with medication guidance based on the WeChat platform) using a randomized number table. The time to resolution of symptoms, serological index levels, incidence of adverse drug events, medication adherence scores and satisfaction rate of family guidance were compared between the two groups. Results: The disappearance time of symptoms such as wheezing and cough in the WeChat group was shorter than that in the conventional group (P < .05). After treatment, the C-reactive protein (CRP), interleukin-6 (IL-6) and calcitoninogen (PCT) levels and the incidence of adverse drug events were lower in the WeChat group than in the conventional group (P < .05). After treatment, the levels of forceful spirometry (FVC), 1st-second expiratory volume (FEV1), peak expiratory flow rate (PEF), medication compliance score and family guidance satisfaction rate were higher in the WeChat group than in the conventional group (P < .05). Conclusion: WeChat-based medication guidance can optimize the therapeutic effect of MPP, improve children's medication compliance and satisfaction rate of family guidance, and reduce the occurrence of adverse drug events.

Prevalence and association of early onset severe hyperbilirubinemia in newborn in the East China region: Retrospective medical record analyses.

Research on the prevalence and association of hyperbilirubinemia is controversial because of different cultures, demographics, and clinical conditions. The etiology of hyperbilirubinemia is affected by the environment and other factors in the newborn. The World Health Organization recommended a 1-day hospital stay after uncomplicated delivery, jaundice assessment before discharge, and screening on 3rd and 7th days after birth for hyperbilirubinemia. However, the implementation of these recommendations is difficult in China. The objective of this study was to evaluate the prevalence and association of early onset severe hyperbilirubinemia in newborns in East China. Retrospective medical record analyses for 250 cesarean sections or vaginal deliveries, ≥2 kg body weight, and negative for Hepatitis B surface antigen by birth newborns were performed. A biochemical analyzer, quantitative assay, and quantitative polymerase chain reaction were used to evaluate total serum bilirubin, glucose-6-phosphate dehydrogenase (G6PD) deficiency, and gene variant phenotyping, respectively. A total in 33 (13%) newborns were reported with early onset severe hyperbilirubinemia (according to the American Academy of Pediatrics, total serum bilirubin ≥ 342 µmol/L within 6 hours of birth). All newborns with severe hyperbilirubinemia were hospitalized and underwent phototherapy. The mothers of all newborns had a gestational age ≥ 35 weeks. Hospitalization included artificial feeding, and breastfeeding was rare (P < .0001). ABO incompatibility ("O" blood type for mother and either "A" or "AB" or "B" blood type for newborn, P = .0411), G6PD deficiency (G6PD/6-phosphogluconate dehydrogenase ≤ 1.0 in quantitative assay, P = .0422), Rh incompatibility (the mother's blood type was Rh negative and newborn blood type was Rh positive, P = .0416), fewer genotype rs4149056 frequencies (P = .0452), higher genotype rs2306283 frequencies (P = .0461), and higher genotype rs1805173 frequencies (P = .0471) were independent parameter for early onset severe hyperbilirubinemia of newborns. The prevalence of early onset severe hyperbilirubinemia in Chinese newborns is 13% in the East China region. Blood incompatibility, G6PD deficiency, fewer genotype rs4149056 frequencies, higher genotype rs2306283 frequencies, and higher genotype rs1805173 frequencies were independent predictors of early onset severe hyperbilirubinemia among newborns in the East China region (Level of Evidence: IV; Technical Efficacy: Stage 5).

Seasonal advance of intense tropical cyclones in a warming climate.

Intense tropical cyclones (TCs), which often peak in autumn1,2, have destructive impacts on life and property3-5, making it crucial to determine whether any changes in intense TCs are likely to occur. Here, we identify a significant seasonal advance of intense TCs since the 1980s in most tropical oceans, with earlier-shifting rates of 3.7 and 3.2 days per decade for the Northern and Southern Hemispheres, respectively. This seasonal advance of intense TCs is closely related to the seasonal advance of rapid intensification events, favoured by the observed earlier onset of favourable oceanic conditions. Using simulations from multiple global climate models, large ensembles and individual forcing experiments, the earlier onset of favourable oceanic conditions is detectable and primarily driven by greenhouse gas forcing. The seasonal advance of intense TCs will increase the likelihood of intersecting with other extreme rainfall events, which usually peak in summer6,7, thereby leading to disproportionate impacts.

Frontotemporal giant extradural dermoid cyst: illustrative case.

BACKGROUND: Dermoid cyst is a rare benign tumor exhibiting a typical radiological pattern and most commonly located along the midline. Laboratory examination was always normal. However, the features of some rare cases are atypical that can be easily misdiagnosed as other tumors. OBSERVATIONS: A 58-year-old patient presented with tinnitus, dizziness, blurred vision, and gait unsteadiness. Laboratory examination showed the serum levels of carbohydrate antigen 19-9 (CA19-9) were significantly increased (186 U/mL). A computed tomography (CT) scan revealed a predominant hypodense lesion in the left frontotemporal region with a hyperdense mural nodule. The lesion appeared as an intracranial extradural mass with a mural nodule on the sagittal image, displaying mixed signal on T1- and T2-weighted imaging. A left frontotemporal craniotomy was performed for cyst resection. Histological results confirmed a diagnosis of dermoid cyst. No tumor recurrences were observed at the 9-month follow-up. LESSONS: Extradural dermoid cyst with a mural nodule is extremely rare. When a hypodense lesion on CT shows mixed signal on T1- and T2-weighted imaging with a mural nodule, even if it is located in the extradural areas, it is important to consider a dermoid cyst. Serum CA19-9 combined with atypical imaging features may contribute to the diagnosis of dermoid cysts. Only recognition of atypical radiological features can avoid misdiagnosis.

Novel pathogenic variant (c.2947C > T) of the carbamoyl phosphate synthetase 1 gene in neonatal-onset deficiency.

Background: Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is a rare autosomal recessive urea cycle disorder characterized by hyperammonaemia. The biochemical measurement of the intermediate metabolites is helpful for CPS1D diagnosis; it however cannot distinguish CPS1D from N-acetylglutamate synthetase deficiency. Therefore, next-generation sequencing (NGS) is often essential for the accurate diagnosis of CPS1D. Methods: NGS was performed to identify candidate gene variants of CPS1D in a Asian neonatal patient presented with poor feeding, reduced activity, tachypnea, lethargy, and convulsions. The potential pathogenicity of the identified variants was predicted by various types of bioinformatical analyses, including evolution conservation, domain and 3D structure simulations. Results: Compound heterozygosity of CPS1D were identified. One was in exon 24 with a novel heterozygous missense variant c.2947C > T (p.P983S), and another was previously reported in exon 20 with c.2548C > T (p.R850C). Both variants were predicted to be deleterious. Conservation analysis and structural modeling showed that the two substituted amino acids were highly evolutionarily conserved, resulting in potential decreases of the binding pocket stability and the partial loss of enzyme activity. Conclusion: In this study, two pathogenic missense variants were identified with NGS, expanding the variants pectrum of the CPS1 gene. The variants and related structural knowledge of CPS enzyme demonstrate the applicability for the accurate diagnosis of CPS1D.

The Utility of Peripheral Blood Leucocyte Ratios as Biomarkers in Neonatal Sepsis: A Systematic Review and Meta-Analysis.

Background: Early stage diagnosis of neonatal sepsis (NS) remains a major roadblock due to non-specific symptoms and the absence of precise laboratory index tests. The full blood count is a relatively cheap, universal, and rapid diagnostic test. Method: This study assessed the diagnostic accuracies of immature-to-total neutrophil ratio (ITR), immature-to-mature neutrophil ratio (IMR), neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR) used in the diagnosis of NS. Included studies were retrieved by searching four major databases and relevant references, and reviewed based on the inclusion/exclusion criteria. Pooled sensitivities and specificities were calculated, I 2 was utilized to test for heterogeneity, and the source was investigated via meta-regression analysis. Results: Finally, 38 studies passed the eligibility criteria. A total of thirty-one studies (6,221 neonates) included data on the ITR, eight studies (1,230 neonates) included data on the IMR, seven studies (751 neonates) included data on the NLR, and two studies (283 neonates) included data on the PLR. The summary sensitivity estimates with 95% confidence interval (CI) for the ITR, IMR, NLR, and PLR tests were, respectively, 0.74 (95% CI: 0.66-0.80), 0.74 (95% CI: 0.54-0.88), 0.73 (95% CI: 0.68-0.78), and 0.81 (95% CI: 0.55-1.00). The summary specificity values for the ITR, IMR, NLR, and PLR tests were 0.83 (95% CI: 0.77-0.87), 0.89 (95% CI: 0.80-0.94), 0.69 (95% CI: 0.57-0.79), and 0.93 (95% CI: 0.81-1.00), respectively. The area under the summary receiver operating characteristic curves for the ITR, IMR, and NLR tests were 0.85 (95% CI: 0.82-0.88), 0.91 (95% CI: 0.88-0.93), and 0.75 (95% CI: 0.71-0.79). The PLR could not be evaluated because only two studies included pertinent data. Conclusion: The NLR test might not be sufficiently accurate in precisely diagnosing NS. The ITR and IMR tests alone can improve the accuracy of NS diagnosis, but the marked heterogeneity and the limited number of studies prevented us from reaching any definitive conclusions. Thus, further studies are warranted to validate these findings. Systematic Review Registration: [https://www.crd.york.ac.uk/prospero/], identifier [CRD42021247850].

LUBAC regulates ciliogenesis by promoting CP110 removal from the mother centriole.

Primary cilia transduce diverse signals in embryonic development and adult tissues. Defective ciliogenesis results in a series of human disorders collectively known as ciliopathies. The CP110-CEP97 complex removal from the mother centriole is an early critical step for ciliogenesis, but the underlying mechanism for this step remains largely obscure. Here, we reveal that the linear ubiquitin chain assembly complex (LUBAC) plays an essential role in ciliogenesis by targeting the CP110-CEP97 complex. LUBAC specifically generates linear ubiquitin chains on CP110, which is required for CP110 removal from the mother centriole in ciliogenesis. We further identify that a pre-mRNA splicing factor, PRPF8, at the distal end of the mother centriole acts as the receptor of the linear ubiquitin chains to facilitate CP110 removal at the initial stage of ciliogenesis. Thus, our study reveals a direct mechanism of regulating CP110 removal in ciliogenesis and implicates the E3 ligase LUBAC as a potential therapy target of cilia-associated diseases, including ciliopathies and cancers.

Associations between air pollutant and pneumonia and asthma requiring hospitalization among children aged under 5 years in Ningbo, 2015-2017.

Introduction: Exposure to ambient air pollutants is associated with an increased incidence of respiratory diseases such as pneumonia and asthma, especially in younger children. We investigated the relationship between rates of hospitalization of children aged under 5 years for pneumonia and asthma and the concentration of air pollutants in Ningbo between January 1, 2015 and August 29, 2017. Methods: Data were obtained from the Ningbo Air Quality Data Real-time Publishing System and the big data platform of the Ningbo Health Information Center. A generalized additive model was established via logarithmic link function and utilized to evaluate the effect of pollutant concentration on lag dimension and perform sensitivity analysis. Results: A total of 10,301 cases of pneumonia and 115 cases of asthma were identified over the course of this study. Results revealed that PM2.5, PM10, SO2 and NO2 were significantly associated with hospitalization for pneumonia and asthma in children under 5 years of age. For every 10-unit increase in lag03 air pollutant concentration, hospitalization for pneumonia and asthma due to PM2.5, PM10, SO2 and NO2 increased by 2.22% (95%CI: 0.64%, 3.82%), 1.94% (95%CI: 0.85%, 3.04%), 11.21% (95%CI: 4.70%, 18.10%) and 5.42% (95%CI: 3.07%, 7.82%), respectively. Discussion: Adverse effects of air pollutants were found to be more severe in children aged 1 to 5 years and adverse effects due to PM2.5, PM10 and SO2 were found to be more severe in girls. Our findings underscore the need for implementation of effective public health measures to urgently improve air quality and reduce pediatric hospitalizations due to respiratory illness.

The Risk Assessment and Clinical Research of Bile Duct Injury After Transcatheter Arterial Chemoembolization for Hepatocellular Carcinoma.

PURPOSE: To retrospectively evaluate the risk factors and the clinical outcomes of bile duct injury after transcatheter arterial chemoembolization (TACE) for hepatocellular carcinoma (HCC) and to evaluate factors that aid clinical detection and subsequent treatment of the injured bile duct. MATERIALS AND METHODS: All patients undergoing TACE for HCC were retrospectively reviewed for identification of bile duct injury. The clinical spectrum of all the patients analyzed including patients' demographics, laboratory data, radiologic imaging and mode of treatment. RESULTS: From January 2015 to December 2017, a total of 21 patients (4.3%) out of 483 patients with 693 TACE procedures were identified to have bile duct injury at our single institution. There were 17 males and 4 females, with a mean age of 59.8±11.6 years (range 34-84). About 14.3% (3/21) patients show the high-density shadow around the bile duct wall in one week non-enhanced CT, and 76.2% (16/21) cases ALP>200 U/L, all these patients showed bile duct injury on the subsequent follow-up CT. Post-TACE follow-up blood biochemistry showed that alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP) and gamma glutamyl transpeptidase (GGT) increased significantly compared with pre-TACE level. The incidence of various types of bile duct injuries on CT was intrahepatic bile duct dilatation (57.1%), biloma (25.7%) and hepatic hilar biliary strictures (17.1%), respectively. Patients with prior hepatectomy as well as proximal arterial chemoembolization carried a higher risk of post-TACE bile duct injury in terms of microvascular damage to the peribiliary capillary plexus. CONCLUSION: Bile duct injury complicating TACE is not caused by a single factor, but by a variety of factors, and is closely related to the microvascular compromise of the bile ducts and subsequent chronic biliary infection. Lipiodol deposited along the bile duct wall and the sharp rise of ALP>200 U/L in one week after TACE can predict bile duct injury and early intervention may prevent the occurrence of serious complications. The probability of bile duct injury in patients with prior hepatectomy and proximal arterial chemoembolization increases significantly.

ZGDHu-1 for cancer therapy.

N,N'-di-(m-methylphenyl)-3,6-dimethyl-1,4-dihydro-1,2,4,5-tetrazine-1,4-dicarboamide (ZGDHu-1) is a novel tetrazine derivative that was initially designed and produced by Professor W.X. Hu, and which has been reported by our group to exhibit antitumor activity. Accumulating evidence suggests that the anticancer mechanisms of ZGDHu-1 may be involved indifferent biological activities, particularly in acute myeloid leukemia (AML) cells. At a high concentration, ZGDHu-1 has been demonstrated to inhibit the proliferation of the leukemia cells by arresting the cell cycle at the G2/M phase, and by inducing cell apoptosis via inducing the accumulation of reactive oxygen species, the translocation of phosphatidylserine across the plasma membrane and the loss of mitochondrial membrane potential. Furthermore, at a low concentration, it was demonstrated to induce the differentiation and degrade the AML1-eight-twenty-one fusion protein in AML cells. Finally, results from a previous study indicate that ZGDHu-1 is a potential proteasome inhibitor. Overall, our preliminary research suggests that ZGDHu-1 may be a promising anticancer drug; however, further research is warranted to identify the exact drug target and potential clinical application in leukemia cells or solid tumors. In the present review, the application of ZGDHu-1 in cancer research, in addition to the specific underlying targets of ZGDHu-1, are discussed.

[Efficacy of different preparations of budesonide combined with pulmonary surfactant in the treatment of neonatal respiratory distress syndrome: a comparative analysis].

OBJECTIVE: To study the efficacy of different preparations of budesonide combined with pulmonary surfactant (PS) in improving blood gas levels and preventing bronchopulmonary dysplasia (BPD) in preterm infants with neonatal respiratory distress syndrome (NRDS). METHODS: A total of 184 preterm infants who developed NRDS within 4 hours after birth were randomly administered with PS + continuous inhalation of budesonide aerosol (continuous aerosol group), PS+budesonide solution (solution group), PS + single inhalation of budesonide aerosol (single aerosol group), and PS alone, with 46 neonates in each group. The changes in arterial blood gas levels, rate of invasive mechanical ventilation after treatment, time of assisted ventilation, rate of repeated use of PS, and the incidence of BPD were compared between the four groups. RESULTS: On the 2nd to 4th day after treatment, pH, PCO2, and oxygenation index (FiO2/PaO2) showed significant differences among the four groups, and the continuous aerosol group showed the most improvements of all indicators, followed by the solution group, single aerosol group, and PS alone group. The continuous aerosol group had a significantly shorter time of assisted ventilation than the other three groups (P<0.05). The solution group had a significantly shorter time of assisted ventilation than the single aerosol and PS alone groups (P<0.05). The rate of invasive mechanical ventilation after treatment, rate of repeated use of PS, and incidence of BPD showed significant differences among the four groups (P<0.05), and the continuous aerosol group had the lowest rates, followed by the solution group. CONCLUSIONS: A combination of PS and continuous inhalation of budesonide aerosol has a better efficacy in the treatment of NRDS than a combination of PS and budesonide solution. The difference in reducing the incidence of BDP between the two administration methods awaits further investigation with a larger sample size.

N,N'-di-(m-methylphenyi)-3,6-dimethyl-1,4-dihydro-1,2,4,5-tetrazine-1,4-dicarboamide (ZGDHu-1) suppresses the proliferation of PANC-1 pancreatic cancer cells via apoptosis and G2/M cell cycle arrest.

Pancreatic cancer is one of the human gastrointestinal malignancies with a high mortality and poor prognosis. Approximately eighty percent of patients are diagnosed with unresectable or metastatic disease. Thus, development of novel chemicals in the treatment of pancreatic cancer is imperative. This study aimed to investigate the anticancer effects of N,N'-di-(m-methylphenyi)-3,6-dimethyl-1,4-dihydro-1,2,4,5-tetrazine-1,4-dicarboamide (ZGDHu-1), a new tetrazine derivative, on the PANC-1 pancreatic cancer cell line and clarify the underlying molecular mechanism. Using an MTT assay, we found that ZGDHu-1 significantly suppressed the proliferation of PANC-1 cells in a time- and dose-dependent manner. Moreover, according to the morphological and flow cytometric analysis, the results indicated that ZGDHu-1 induced PANC-1 cell apoptosis and G2/M cell cycle arrest in a dose-dependent manner. In the western blot analysis, expression of the pro-apoptotic Bax gene was upregulated while the anti-apoptotic Bcl-2 gene was downregulated following treatment with ZGDHu-1. ZGDHu-1 also activated pro-caspase-3 and PARP and increased the expression of NF-κB inhibitor IκB. Furthermore, the expression levels of G2/M regulatory molecules such as cyclin B1 and cdc2 were decreased while that of Chk1 was increased. These results suggested that ZGDHu-1 suppressed the proliferation of pancreatic cancer cells, rendering it a potential therapeutic drug for the treatment of pancreatic cancer.

Application of transport timescales to coastal environmental assessment: a case study.

A numerical model based on the random walk particle tracking technique is developed to simulate the transport of the contaminants discharged into the coastal area of Guangxi, China from rivers. The model couples a 1D river flow module and a 2D coastal circulation module. Two transport timescales, i.e., the age and the arrival time, are introduced to characterize the contaminant transport process. The age is the time for a particle taken to enter the domain of interest after it left the place where the age is initially set to zero. The arrival time, on the other hand, is the age of the particle arriving at a particular place most quickly after release. It is useful for emergency responses to such an accident as toxic substance leakage. By the numerical model, the age and the arrival time under various conditions in the coastal area of Guangxi, China are studied in details. The age distribution and the arrival time of particles at a specific location are shown to closely rely on the coastal hydrodynamic environment in addition to the distance of the location from the source where particles are released. Particles released at spring tide and at ebb tide are found to spread more quickly than those released at neap tide and at flood tide, respectively. A large carrying discharge of river reduces the ages of the contaminants from the river concerned when transported to a place within the coastal area, while it has less influence on the arrival time, particularly in a place along the coast.

[Comparison of the software safety evaluation methods in medical devices].

The article intends to analyze the software safety problems in high-risk medical devices based on the investigation of software R & D Quality control procedures in Shanghai medical device manufacturing enterprises. The idea of improving the software pre-market safety evaluation method in China is also explored through the way of comparing those in U.S. and Europe.