Tracking changes in image-defined risk factors during neoadjuvant chemotherapy and their predictive value for surgical outcomes based on the International Neuroblastoma Surgical Report Form.

BACKGROUND: The capacity of presurgical image-defined risk factors (IDRFs) to predict secondary surgical outcomes in patients with neuroblastoma is controversial. METHODS: The International Neuroblastoma Surgical Report Form (INSRF) was employed to retrospectively collect the clinical data of 53 patients diagnosed with neuroblastoma at our hospital from April 2014 to April 2020. IDRFs were identified at the time of diagnosis and reassessed during the course of neoadjuvant chemotherapy. Various statistical tests were used to evaluate the correlation between IDRFs and secondary surgical outcomes. RESULTS: A total of 195 IDRFs were identified. Notably, by two courses of neoadjuvant chemotherapy, the number of "two body compartments," "intraspinal tumor extension," and "trachea-compressing" IDRFs decreased significantly (p = .001). The primary tumor volumes and the number of IDRFs decreased significantly by four courses of neoadjuvant chemotherapy, especially in "intraspinal tumor extension" IDRFs (p = .034). The median number of IDRF per patient was four (interquartile range [IQR]: 1-5) at diagnosis, which diminished to one (IQR: 1-3) subsequent to neoadjuvant chemotherapy. The presence of preoperative IDRFs was not associated with surgical complications (p = .286) or the extent of surgery (p = .188). However, the number of preoperative IDRFs linked to the extent of surgery (p = .002), not to operative complications (p = .669). Specifically, presurgery "renal vessel contact" IDRFs were predictive of surgical complications, while presurgery "infiltration of vital structures" IDRFs were associated with the extent of surgery. CONCLUSION: The number of IDRFs decreased significantly by four courses of neoadjuvant chemotherapy. The number and type of presurgery IDRFs may predict secondary surgical outcomes, surpassing the mere consideration of their presence or absence.

Laparoscopic-assisted sclerotherapy in pediatric retroperitoneal lymphatic malformations.

Background: Retroperitoneal lymphatic malformations (LMs) are rare. Currently, the treatment of retroperitoneal LMs remains challenging. This study aimed to examine the safety and efficacy of laparoscopic-assisted sclerotherapy for retroperitoneal LMs in pediatric patients. Methods: We retrospectively reviewed patients treated with laparoscopic-assisted sclerotherapy for retroperitoneal LMs in a single tertiary medical center between July 2020 and February 2023. Doxycycline was prepared into a solution with a concentration of 10 mg/ml for use in sclerotherapy. Demographic data, clinical features, details of management, and outcomes were collected and analyzed. Results: A total of six patients, comprising three males and three females, were identified. The LMs were categorized into four macrocystic and two mixed-cystic types. The mean age and weight were 52.2 months (range, 11-108 months) and 20 kg (range, 12.5-27.5 kg), respectively. Three patients presented with abdominal pain or distension, while the other three patients were asymptomatic. All six patients underwent a total of eight sclerotherapy sessions. Two patients experienced intra-cystic hemorrhage and required a second sclerotherapy session. Only one patient presented with vomiting after sclerotherapy, which resolved spontaneously. Five patients met the complete response criteria, and one patient met the effective criteria. The mean reduction in lesion size was 92.3% (range, 69.9%-99.6%). No further complications or recurrence were recorded during follow-up. Conclusion: Laparoscopic-assisted sclerotherapy is a safe and effective approach for treating retroperitoneal LMs. This technique is applicable for both macrocystic and mixed-cystic retroperitoneal LMs.

Effectiveness of preoperative ultrasonography in predicting metachronous contralateral inguinal hernia in children: a single-arm prospective study using a historical control for comparison.

PURPOSE: To analyze the value of ultrasonography in predicting metachronous contralateral inguinal hernia (MCIH) and diagnosing contralateral persistent processus vaginalis (CPPV) in children with unilateral inguinal hernia, a prospective study was conducted. METHODS: All participants underwent a preoperative ultrasound on the contralateral groin. Patients in group A1 received operating procedure according to ultrasound results (patients with negative contralateral US results received hernia repair on the affected side), and patients in group A2 received operation according to laparoscopic results (patients received hernia repair and CPPV ligation). All patients were followed up 2 years and compared to a historical control (group B) who underwent open hernia repair only on the affected side regardless of contralateral US results. RESULTS: In groups A1 and A2, laparoscopic exploration revealed the presence of a CPPV in 490 cases. Ultrasound was found to be accurate in 104 out of the 490 cases with four false-positive and 386 false-negative results. This yielded an accuracy of 59.3%, a sensitivity of 21.2%, and a specificity of 99.2%. 10 patients in group A1, and 74 patients in group B developed MCIH. The accuracy, sensitivity, and specificity of the value of ultrasonography in predicting MCIH were 89.3%, 52.4%, and 92.5%, respectively. CONCLUSIONS: Preoperative ultrasonography of the contralateral groin is currently unable to accurately detect CPPV, but it appears to be a promising method in predicting MCIH by using rigorous diagnosing criteria.

Targeted LC-MS/MS profiling of bile acids reveals primary/secondary bile acid ratio as a novel biomarker for necrotizing enterocolitis.

Bile acids (BAs) are involved in the development of necrotizing enterocolitis (NEC), which mainly occurs in preterm infants. We aim to identify the change of BAs in preterm infants and validate its potential value in the detection of NEC. Targeted liquid chromatography-tandem mass spectrometry (LC-MS/MS) was performed to measure the plasma BAs in healthy preterm infants and patients with NEC. By analyzing the level of BAs in healthy preterm infants, we found that the plasma concentrations of BAs were related to sex, gestational/postnatal age, birth weight, mode of birth, and feeding type after birth. The plasma levels of TCA, GCA, TCDCA, GCDCA, primary BAs, and total BAs and the primary/secondary BA ratio were decreased, while DCA, UDCA, and secondary BAs were increased in NEC. The primary/secondary BA ratio (cutoff point 62.9) can effectively differentiate NEC from healthy preterm infants, with an AUC of 0.9, a sensitivity of 94.5%, and a specificity of 78.1%. Combining the ratio with high-risk factors of NEC can better distinguish between NEC and control, with an AUC of 0.95. Importantly, significantly lower levels of primary/secondary BA ratio were found in infants with surgical NEC than in nonsurgical NEC cases. The cutoff point of 28.7 identified surgical NEC from nonsurgical NEC with sensitivity and specificity of 76.9% and 100%. Thus, our study identified that the primary/secondary BA ratio in the plasma can differentiate NEC from healthy preterm infants and effectively differentiate the surgical NEC from nonsurgical NEC. Therefore, LC-MS/MS was expected to be a novel measurement platform used to distinguish infants who are most in need of close monitoring or early surgical intervention.

Misdiagnosis of scrotal and retroperitoneal lymphangioma in children.

BACKGROUND: Scrotal and retroperitoneal lymphangioma (SRL) in children is relatively rare and its clinical symptoms are usually difficult to distinguish from other conditions such as hydrocele and incarcerated inguinal oblique hernia. This study aimed to explore the clinical diagnosis and treatment of abdominal scrotal lymphangioma in children, and thus, to increase our understandings of this disease in clinical practice. METHOD: This study enrolled nine boys, aged 1-10, who were admitted to Shanghai Children's Hospital from January 2019 to December 2020 and who were finally confirmed with lymphangioma in the inguinal area. The clinical manifestations, diagnosis, and treatment of these children were analyzed retrospectively. The length of diagnostic process ranged from 3 weeks to 20 months. We also reviewed other cases of initially misdiagnosed cases of SRL in English publications from 2000 to 2022. RESULTS: The nine cases were misdiagnosed as hydrocele, hematoma, or inguinal hernia. Three patients received intracystic injection of bleomycin, three underwent laparoscopic mass resection, and three underwent resection of the inguinal lymphangioma under direct vision. Postoperative pathological analysis of the surgical specimens confirmed the diagnosis of benign cystic lesions and lymphangioma. Meanwhile, among the 14 cases of SRL in literature review, eight were misdiagnosed. Six were initially diagnosed as hydrocele, one as inguinal oblique hernia, and one as testicular tumor, all of which underwent ultrasonography scans. All cases were confirmed as lymphangioma after pathological examination. CONCLUSION: The non-specific clinical manifestations may contribute to the misdiagnosis of scrotal masses in children. A detailed and accurate medical history, careful physical examination, and imaging findings are important factors contributing to the preoperative differential diagnosis of scrotal lumps in children, but the final diagnosis is based on pathological examination.

Elevated expression of histone deacetylase HDAC8 suppresses arginine-proline metabolism in necrotizing enterocolitis.

Epigenetic alterations are especially important in necrotizing enterocolitis (NEC). Here, we reported that histone deacetylase 8 (HDAC8) plays a previously unknown role in modulating arginine metabolism via acetylation of histone 3 lysine 9 (acetyl-H3K9) regulation during the pathogenesis of NEC. We found that HDAC8 was upregulated in humans and mice intestinal samples with NEC, while selective inhibition of HDAC8 expression ameliorated NEC. HDAC8 regulates enzymes involved in the metabolic conversion of proline to arginine (PRODH, PRODH2, OAT, and OTC) and arginine to ornithine (ARG1). The results showed that H3K9ac signal in the PRODH/PRODH2 promoter region was mediated by HDAC8. Additionally, the decreased concentration of butyric acid was strongly correlated with elevated HDAC8 levels and circulating arginine, which may result from an unbalanced Firmicutes/Bacteroidetes ratio. These results reveal previously underappreciated roles of microbial metabolites and HDAC8 to coordinate the arginine metabolism during NEC development.

Pathogenicity of p.Phe147del in RET in familial Hirschsprung's disease.

OBJECTIVE: The study aimed to explore the pathogenicity of RET p.Phe147del in a Hirschsprung'irschspru (HSCR) family and facilitate the deeper understanding of HSCR families. METHODS: Whole-exome sequencing (WES) was performed to decipher a HSCR family. We used a "GlycoEP" tool to analyze RET protein glycosylation. A series of molecular biological approaches including mutated plasmid construction, cell transfection, polymerase chain reaction, immunofluorescence and immunoblotting were introduced to determine the mutation status and altered expression of RET as well as its related genes or proteins. MG132 was applied to analyze the mechanism of mutated RET. RESULTS: WES and Sanger results revealed that p.Phe147del in-frame mutation (IM) was a potential pathogenetic factor for familial HSCR. Moreover, the IM led to disrupted N-glycosylation of RET accompanied with protein structural change, resulting in the decreased transcriptional and protein level of RET, CCND1, VEGF and BCL2, and the decreased protein level of phosphorylated ERK and STAT3. Further studies revealed that the IM-evoked RET decline was reversed by inhibiting proteosome in a dose dependent manner, thus suggesting that the decrease in intracellular RET protein levels interrupted the transportation of RET protein from the cytoplasm to the cell surface. CONCLUSION: The newly found p.Phe147del IM of RET is pathogenic to familial HSCR and it disrupts RET structure and abundance via the proteasome pathway, representing evidence for the early prevention, clinical diagnosis and treatment of HSCR.

The composition of the gut microbiota is altered in biliary atresia with cholangitis.

Aim: To detect the composition of the gut microbiota in biliary atresia after Kasai surgery. Methods: Infants within six months after the Kasai operation who were diagnosed by cholangiography at Shanghai Children's Hospital were enrolled in the study. Fecal samples were collected from diapers, placed into sterile tubes in the inpatient department or outpatient department and frozen at -80°C within half an hour. The gut microbiota was detected by 16S rRNA sequences. Then, the patients that were followed up to one year after the Kasai operation who suffered from cholangitis at least one time were grouped into the BAcho group, and the others were grouped into the BAnoncho group. Results: Nine of 18 BA patients were grouped into the BAcho group, and the others were grouped into the BAnoncho group. In the BAcho group, AST, ALT and GGT were significantly increased compared to the BAnoncho group. The number of total OTUs (operational taxonomic units) in feces was more elevated in the BAnoncho group than in the BAcho group. In the BAnoncho group, the Chao index at the OTU level was significantly increased compared to that in the BAcho group (66.37 ± 21.5 vs. 45.64 ± 11.25, p = 0.02 < 0.05). Bifidobacterium was the most abundant genus in the BAnoncho group, accounting for 22.14%, and Klebsiella accounted for 22.74% in the BAcho group. Compared with the BAnoncho group, Bacteroides was significantly decreased in the BAcho group (p = 0.037). Conclusion: The composition of the gut microbiota was different between BA with cholangitis and BA without cholangitis.

A retrospective study about incidental appendectomy during the laparoscopic treatment of intussusception.

Purpose: We aim to see incidental appendectomy (IA) was worth or not during the laparoscopic treatment of intussusception. Methods: This study included forty-eight patients who underwent a laparoscopic procedure for idiopathic intussusception without intestinal resection between April 2014 and April 2021. The Chi-square or Fisher's exact tests for categorical variables and the Student t-test for continuous variables were used to analyze and compare patient characteristics. Results: IA was performed on 63% (30/48) of patients after surgical reduction, while 18 (37%), did not. Patients who underwent IA had a higher total cost (16,618 ± 2,174 vs.14,301 ± 5,206, P = 0.036), and a longer mean operation duration (59 ± 19 vs.45 ± 21, P = 0.025). The distribution of the PO time, length of hospital stay, PCs, and RI did not differ significantly. The histopathological evaluation of the 30 resected appendices revealed five (17%) with signs of acute inflammation, 20 (66%) with chronic signs of inflammation, and five (17%) with inconspicuous appendices. Conclusion: IA is linked to a longer average operation time and a higher total cost. There is insufficient evidence to recommend IA during laparoscopic intussusception treatment. The risks and benefits of IA need further study.

Clinical outcomes and risk factors for postoperative complications in children with Hirschsprung's disease.

OBJECTIVE: To explore and analyze the risk factors for postoperative complications in patients with Hirschsprung's disease (HD). METHODS: Patients with HD admitted to the hospital from 2015 to 2020 were reviewed in this retrospective study. Follow-up data collected included constipation, fecal incontinence, anastomotic fistula, Hirschsprung's-associated enterocolitis (HAEC), and readmission. The putative risk factors for postoperative complications in patients with HD were as follows: clinical classification, gender, operative age, hemoglobin and serum albumin levels and preoperative HAEC. A follow-up survey was conducted for all patients by telephone. Data were analyzed statistically using SPSS version 23.0. RESULTS: A total of 154 patients were included in the study, of whom 119 patients were followed up successfully. 53 patients who had complications postoperatively were compared to 66 patients who had no related complications. Among patients with complications: 8 had constipation, 22 had fecal incontinence and 33 had HAEC. The gender, operative age, hemoglobin levels and preoperative HAEC did not differ significantly between the two groups. However, significant differences were observed between the 2 groups in clinical classification, surgical method, serum albumin level, and whether or not a one-stage operation was performed (P<0.05). CONCLUSION: Clinical classification, surgical method, preoperative albumin level and whether or not a one-stage operation was performed emerged as risk factors affecting the postoperative complications of patients with HD. Therefore, the prognosis in HD can be improved by strengthening the preoperative nutritional support and selecting appropriate surgical methods according to the clinical subtype.

Exstrophy-Epispadias Complex With Isolated Ectopic Bowel Segment: A Case Series and Literature Review.

OBJECTIVE: To present a case series of the exstrophy-epispadias complex (EEC) with isolated ectopic bowel segment (IEBS) with the literature review, highlighting the clinical findings and treatments. MATERIALS AND METHODS: We present 3 cases of bladder exstrophy (BE) with IEBS in our institute and reviewed the literature in PubMed with the terms "("bladder exstrophy" OR "epispadias") AND ("visceral sequestration" OR "sequestered" OR "ectopic bowel")." RESULTS: There were 2 males and 1 female. The IEBS was detected by physical examination in 2 cases and by ultrasonography in another one. All cases were BE accompanying with lower abdominal mass which adhered to the bladder wall but was separated from the digestive system. All cases underwent the IEBS excision and BE repair simultaneously. Pathological result of IEBS suggested the histological structures of colon. There were totally 13 cases of EEC with IEBS reported in the literature, including 2 (15%) epispadias, 9 (69%) covered BE, 1 (8%) duplicate BE and 1 (8%) classic bladder exstrophy. Although their clinical manifestations were various, IEBS excision were safely conducted in all cases. CONCLUSION: EEC with IEBS is an extremely rare congenital malformation. Physical and imaging examinations are important for diagnoses. Surgical excision is safe and effective for managing IEBS.

An unusual case of appendicitis due to appendiceal neuroendocrine tumor in a patient: A case report.

Appendiceal neuroendocrine tumor (NET) is the most common type of appendiceal cancer and is rare in the pediatric population. The clinical characteristics of this cancer are not specific and are highly similar to those of acute appendicitis. By contrast, acute appendicitis is a common surgical indication that is caused by obstruction of the appendix lumen. With a detection rate of 0.5-1% in all appendectomy specimens, appendiceal NET is rare histopathologically and can easily be missed. However, detecting an appendiceal NET in a patient with appendicitis is highly difficult. Therefore, clinicians must be aware of this much under-reported and rare tumor in children. In the present report, a case was reported, of a 13-year-old female child who initially presented with clinical presentation of acute appendicitis, but was subsequently diagnosed with appendiceal NET by histopathological examination after an emergency appendectomy. Follow-up examination including abdominal enhanced CT and enteroscopy 2 years after surgery revealed normal results.

The relationship between contralateral patent processus vaginalis and metachronous contralateral inguinal hernia in children with unilateral inguinal hernia: a prospective observational study.

PURPOSE: Laparoscopic contralateral patent processus vaginalis (CPPV) repair in children is debatable due to the high CPPV rate, but low metachronous contralateral inguinal hernia (MCIH) rate. We conducted this study to find risk factors for MCIH. METHOD: We conducted a prospective, observational trial with patients recruited from Shanghai Children's Hospital. Eligible participants were patients under 16 years old with unilateral inguinal hernia whose parents did not opt for simultaneous CPPV repair. The subjects were followed for 24 to 34.1 months. Patients who developed MCIH were analyzed to identify the relationship between CPPV and MCIH. RESULTS: Between October 17, 2018, and July 31, 2019, we included 184 patients and 182 completed follow-up. MCIH occurred in ten patients, of which 7 (7.53%) had CPPV and three (3.37%) had no CPPV. Univariate analysis showed that age (p = 0.025, OR = 0.938) and CPPV diameter (p = 0.003, OR = 1.783) were related to the development of MCIH. In multivariate analysis, only diameter of CPPV (p = 0.008, OR = 1.411) was associated with MCIH. The receiver operating characteristic (ROC) curve was used to test, and it was found that when the diameter of CPPV was greater than 4 mm, the Youden index was the highest, with a specificity of 62.8% and a sensitivity of 100%. CONCLUSION: The incidence of MCIH was not statistically higher in patients with CPPV compared with those without CPPV, so there is no indication for routine CPPV repair. The risk of MCIH development increases with CPPV diameter. 4 mm is the optimal cutoff point. Large CPPVs (> 4 mm) could be treated to prevent future hernias. TRIAL REGISTRATION: The Chinese Clinical Trial Registry ( www.chictr.org.cn ), number ChiCTR2000041307.

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.

Ontogeny of RORγt+ cells in the intestine of newborns and its role in the development of experimental necrotizing enterocolitis.

BACKGROUND: Neonates possess an immature and plastic immune system, which is a major cause of some diseases in newborns. Necrotizing enterocolitis (NEC) is a severe and devastating intestinal disease that typically affects premature infants. However, the development of intestinal immune cells in neonates and their roles in the pathological process of NEC have not been elucidated. RESULTS: We examined the ontogeny of intestinal lamina propria lymphocytes in the early life of mice and found a high percentage of RORγt+ cells (containing inflammatory Th17 and ILC3 populations) during the first week of life. Importantly, the proportion of RORγt+ cells of intestinal lamina propria further increased in both NEC mice and patients tissue than the control. Furthermore, the application of GSK805, a specific antagonist of RORγt, inhibited IL-17A release and ameliorated NEC severity. CONCLUSIONS: Our data reveal the high proportion of RORγt+ cells in newborn mice may directly contribute to the development of NEC.

DNA Methylome Mapping Identifies Epigenetic Abnormalities in Intestinal Lymphocyte Regulation in Human Necrotizing Enterocolitis.

BACKGROUND: Epigenetic changes occur in response to environmental factors during the pathogenesis of necrotizing enterocolitis (NEC) in animal models, but the DNA methylation signature in human patients with NEC has not been examined. AIM: To illustrate the signature and function of DNA methylation in the intestine of human NEC. METHODS: DNA methyltransferases (DNMTs) were compared between intestinal tissue with NEC and control. Genome-wide DNA methylation was analyzed by reduced representation bisulfite sequencing (RRBS). The biological functions of the potential methylation regulated genes were analyzed by Gene Ontology. Gene methylation and expression were confirmed by bisulfite genomic sequencing (BGS) and RT-qPCR. RESULTS: By screening the expression of DNMTs, we identified a marked reduction in DNMT3A at both the mRNA and protein levels in NEC. Genome-wide variation of DNA methylation was detected in NEC lesions. The CG methylation level in almost all unique regions except CpG islands (CGIs) was lower in NEC compared with control. A total of 287 differentially methylated regions (DMRs) were identified across the whole genome in NEC, 123 of them are located on the CGI in the promoter. The DMR-associated genes were linked to intestinal epithelial permeability, platelet aggregation, and lymphocyte proliferation. Four genes (ZNF335, MPL, RASAL3, and KDM6A) with roles in the regulation of lymphocytes that may predispose the intestine to imbalanced immune processes were further confirmed to be hypermethylated and transcriptionally downregulated. CONCLUSIONS: These findings underscore the novel relationship between epigenetic changes and lymphocyte regulation in human NEC, which may have potential diagnostic and therapeutic relevance for NEC.

Case report and literature review: An intrahepatic sewing needle in a child.

Background: The presence of intrahepatic foreign bodies is a rare occurrence at the emergency department. Normally, foreign bodies reach the liver through migration. Incidence is lower among children than among adults, and the circumstances of children are often different. We report a 19-month-old boy with a sewing needle in the liver and review the previous reports of intrahepatic sewing needle in the PubMed database from the last three decades. Case presentation: A 19-month-old boy was transferred to our center from a local hospital presenting intermittent cough and rhinorrhea. A chest radiograph to exclude pulmonary disease revealed an incidental finding of a high-density shadow in the hepatic region. On admission, the boy had no gastrointestinal symptoms. Abdominal physical examinations were unremarkable. His mother, a worker in a textile factory, denied any history of trauma. Abuse was excluded based on investigation. Preoperative routine test results were normal. Contrast-enhanced computed tomography (CT) revealed that the sewing needle was located in hepatic segment IV and the tip had close relationship with intrahepatic portal vein. Initially, laparoscopy was performed without success. We eventually converted to laparotomy to completely remove the rusty sewing needle. The patient resumed feeding soon after the operation and was discharged in a few days. Conclusions: Intrahepatic sewing needle has high incidence among boys and developing countries. Combined with contrast-enhanced CT, knowledge of the pediatric patient's family background and medical history would help judge the route of entry and determine the management and surgical strategy. Laparoscopic procedure is not suitable for rusty sewing needles.

DRG1 Maintains Intestinal Epithelial Cell Junctions and Barrier Function by Regulating RAC1 Activity in Necrotizing Enterocolitis.

BACKGROUND: An immature intestine is a high-risk factor for necrotizing enterocolitis (NEC), which is a serious intestinal disease in newborns. The regulation of developmentally regulated GTP-binding protein 1 (DRG1) during organ development suggests a potential role of DRG1 in the maturation process of the intestine. AIM: To illustrate the function of DRG1 during the pathogenesis of NEC. METHODS: DRG1 expression in the intestine was measured using immunohistochemistry and q-PCR. Immunoprecipitation coupled with mass spectrometry was used to identify the interacting proteins of DRG1. The biological functions of the potential interactors were annotated with the Database for Annotation, Visualization and Integrated Discovery. Caco2 and FHs74Int cells with stable DRG1 silencing or overexpression were used to investigate the influence of DRG1 on cell junctions and intestinal barrier permeability and to elucidate the downstream mechanism. RESULTS: DRG1 was constitutively expressed during the intestinal maturation process but significantly decreased in the ileum in the context of NEC. Protein interaction analysis revealed that DRG1 was closely correlated with cell junctions. DRG1 deficiency destabilized the E-cadherin and occludin proteins near the cell membrane and increased the permeability of the epithelial cell monolayer, while DRG1 overexpression prevented lipopolysaccharide-induced disruption of E-cadherin and occludin expression and cell monolayer integrity. Further investigation suggested that DRG1 maintained cell junctions, especially adherens junctions, by regulating RAC1 activity, and RAC1 inhibition with NSC23766 attenuated intestinal injury and led to improved barrier integrity in experimental NEC. CONCLUSIONS: Our findings illustrate the mechanism underlying the effect of DRG1 deficiency on epithelial cell permeability regulation and provide evidence supporting the application of RAC1 inhibitors for protection against NEC.

Teratoid Wilms Tumor and Classical Wilms Tumor: A Retrospective 10-Year Single-Center Study and Literature Review.

BACKGROUND: One of the most prevalent forms of renal tumors detected among pediatric patients is the Wilms tumor (WT). Teratoid WT is a rare WT subclassification that is characterized by teratoma-like characteristics that include the features of many diverse tissue categories. Less than 70 teratoid Wilms tumor (TWT) cases have been explained up to now. METHODS: Between 2010 and 2020, patients with classical WT and TWT admitted to our hospital were included in this study. Clinicopathological characteristics, intraoperative findings, histopathological parameters, and prognostic outcomes were then compared between classical WT and TWT. To compare these variables, TWT and WT cases were matched at a 1:3 ratio. RESULTS: A total of 67 total WT cases, i.e., five diagnosed with TWT, were enrolled. While no significant differences in analyzed variables were detected between these groups, tumor volumes were notably larger in the TWT group relative to the classical WT group (203.30 ± 109.89 vs. 104.30 ± 66.97 cm3) despite similar tumor weight values in both groups (471.00 ± 80.65 vs. 432.67 ± 109.25 g). As for five patients diagnosed with TWT, all were alive during the follow-up, while one of them was diagnosed with pelvic metastasis. CONCLUSIONS: This study is the first to our knowledge to have reported on the incidence of TWT among Chinese children, and our results preliminarily suggest that a combination of surgery and chemotherapy may be appropriate for the treatment of patients with WT, although prognostic outcomes varied substantially among patients with different stages of the disease. TWT tumor density may be lower than classical WT tumor density. Further research regarding the basic biological characteristics of TWT and relevant theranostic markers associated with this tumor type is warranted to better guide the development of individualized treatments for this rare cancer type.

Pyriform sinus fistula in children: A comparison of endoscopic-assisted surgery and endoscopic radiofrequency ablation.

BACKGROUND: Pyriform sinus fistula (PSF) is a rare congenital anomaly, and the preferred definitive treatment is yet to be verified. In this study, we investigated the treatment outcomes of PSF specifically comparing endoscopic-assisted surgery and endoscopic radiofrequency ablation (RA). METHODS: The medical records of patients treated for PSF at the Shanghai Children's Hospital between October 2016 and September 2019 were retrospectively evaluated. RESULTS: There were 93 girls and 98 boys. The median age at onset and operation was 3 years and 5 years, respectively. Endoscopic-assisted surgery was performed in 143 patients. During the same period, RA was performed in 48 patients, and 10 of them concurrently underwent incision and drainage of neck abscesses. Longer hospital stay was found in the endoscopic-assisted surgery group than in the RA group (10.50 ±â€¯3.93 vs. 5.02 ±â€¯3.30 days, P < 0.001). Postoperative complications were not significantly different between the two groups, except for neck infection (0 vs. 8.3%, P = 0.004). After a median follow-up period of 21 months, no significant difference was found between the two groups in terms of recurrence (1.4% vs. 0, P = 0.560). CONCLUSION: Patients treated with RA had a significantly shorter hospital stay than those treated with endoscopic-assisted surgery. Outcomes of endoscopic-assisted surgery and RA were not significantly different for the management of PSF and treatment method should be tailored to the patient. LEVEL OF EVIDENCE: IV.