BACKGROUND:Age-related differences in presentation and outcomes after laparoscopic inguinal hernia repair (LIHR) remain incompletely characterized. We evaluated age-stratified perioperative characteristics and recurrence after LIHR. METHODS:We conducted a retrospective single-center cohort study of consecutive children undergoing LIHR, stratified as <6 months, 6-12 months, 1-2 years, 2-5 years, and >5 years. The primary outcome was recurrence, while secondary outcomes included incarceration, hernia laterality, operative time, occult contralateral hernia detection, conversion to open surgery, complications, and length of stay. RESULTS:Among 8941 children, incarceration occurred in 2.1%, bilateral hernia in 47.6%, and occult contralateral hernia was detected in 39.2%. Incarceration decreased with age (15.6% in <6 months vs 0.6-0.7% in >2 years; P < 0.001), and bilateral hernia was most frequent in <6 months (74.6%; P < 0.001). Median operative time decreased from 31 min (<6 months) to 26 min (>5 years; P < 0.001). Occult contralateral hernia was most common in <6 months (52.7%; P < 0.001). Conversion was highest in <6 months (0.8%) and absent in >5 years (P = 0.009). Recurrence was 1.3% (113/8941), with none in <6 months and increasing to 2.1% in >5 years (P < 0.001). Male sex (HR 2.974, 95% CI 1.708-5.176) and unilateral presentation (HR 5.141, 95% CI 2.931-9.017) independently predicted recurrence. CONCLUSIONS:Infants, especially those <6 months, had higher rates of incarceration and bilateral disease, more frequent occult contralateral findings, and greater operative complexity. Recurrence after LIHR was low overall, absent in <6 months, and increased with age, supporting age-stratified counseling, operative planning, and follow-up.

AimsTo analyze the epidemiology, clinical characteristics, surgical outcomes, and risk factors for incarceration and recurrence in a large cohort of children undergoing inguinal hernia repair, and to evaluate differences across sex, laterality, and surgical approaches.MethodsWe retrospectively reviewed the children who underwent inguinal hernia repair at a tertiary pediatric center over ten years. Demographic characteristics, perioperative outcomes, and postoperative complications were analyzed. Subgroup comparisons were performed by sex, laterality, and surgical approach. Multivariate logistic regression models were used to identify independent predictors of incarceration and recurrence.ResultsOf 9590 children, 72.2% were male, and the median age was 2 years and 10 months. Laparoscopic surgery was performed in 93.1% of cases. Incarceration occurred in 4.2% of children and recurrence in 1.4%. Females, children ≤1 year, and unilateral hernias were independently associated with higher risk of incarceration. Male sex and age >1 year predicted recurrence, while laparoscopic technique served as a protective factor. Laparoscopy identified synchronous contralateral hernias in 39.2% of children initially diagnosed with unilateral hernia, compared with only 0.9% detected during open repair. Laparoscopic approach was also associated with shorter operative time, fewer complications, and faster recovery.ConclusionsThis large cohort study highlights the epidemiology and surgical outcomes of pediatric inguinal hernia. Age, sex, and hernia laterality were associated with clinical presentation and complication risk. Laparoscopic surgery showed favorable perioperative outcomes and facilitated detection of contralateral hernias. Because surgical approach selection was not randomized, comparisons between techniques should be interpreted cautiously. These findings emphasize the importance of individualized risk stratification and surgical decision-making.

Glioblastoma, the most lethal and pervasive subtype of glioma, has been classified according to both molecular signatures and cell identity. However, the promise of subtype-specific therapy for GBM has yet to be realized. We hypothesize that epigenetic compounds could be effective due to the crucial role of epigenetic regulation in maintaining individual GBM subtypes. We establish a mouse GBM model by expressing AKT3, DN-p53 and PDGFB (ADP) in cortical neural progenitor cells via in utero electroporation. Gene expression analysis demonstrate that ADP glioma exhibits the oligodendrocyte precursor cell (OPC) signature. A small-scale compound screening conducted in cultured ADP tumor cells identify BRM014 (dual ATPase inhibitor of chromatin remodelers BRM/BRG1) as an effective compound. Mechanistically, acute treatment of ADP tumor cells with BRM014 specifically inhibits chromatin accessibility and the expression of oligodendrocyte genes. Importantly, genetic depletion of Brg1 significantly delays tumor progression and prolongs the survival of ADP glioma-bearing mice. Importantly, BRM014 treatment selectively inhibits the growth of human GBM cells with the OPC signature. In sum, our findings demonstrate that the inhibition of ATPase activity of BRG1 is a promising epigenetic therapy for OPC-like GBM.

Purpose: There is no standard surgical approach for pancreaticobiliary maljunction (PBM) without congenital biliary dilatation (CBD). This study aimed to compare outcomes between therapeutic endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic hepaticojejunostomy (LH) for pediatric patients of PBM without obvious biliary dilatation (PBM-nonOBD). Methods: We retrospectively reviewed demographic and clinical data of pediatric patients with PBMnonOBD from 2015 to 2021. There were 33 patients in ERCP group and 35 patients in LH group. Primary outcomes included treatment efficiency, postoperative recovery, and postoperative complications. Univariate analysis was further used to explore prognostic factors for ERCP. Results: The mean diameter of the common bile duct in LH group was larger than that in ERCP group (8.6 +/- 1.3 mm vs. 6.9 +/- 2.1 mm, p 1 / 4 0.003), while there were no significant differences between the two groups in age, gender, clinical manifestations, complications, and other imaging findings. Compared with LH group, ERCP group had a shorter operation time and postoperative recovery time. The treatment effective rate of ERCP was inferior to that of LH (45.4 % vs. 85.7 %, p <0.001). For postoperative adverse events, post-ERCP pancreatitis (15.1 %) was most common in the ERCP group. 30.3 % of patients eventually required LH. Intestinal obstruction (5.7 %), recurrent cholangitis (5.7 %), gastrointestinal bleeding (2.8 %), and anastomotic stenosis (2.8 %) were observed in LH group and 8.6 % of patients required a reoperation. A long common channel may be associated with poor prognosis after ERCP. Conclusions: ERCP is associated with less surgical trauma, shorter recovery time, and fewer serious complications than LH, while the treatment effective rate of ERCP is inferior to LH. The indications for endoscopic sphincterotomy and the timing of radical surgery need to be further explored. Level of evidence: III Study type: Retrospective Comparative Study. (c) 2023 Elsevier Inc. All rights reserved.

ObjectiveThis study aims to retrospectively analyze the epidemiological and clinical characteristics of acute intussusception in a tertiary-care pediatric hospital in China over 4 years and evaluate the effectiveness and recurrence of fluoroscopy-guided pneumatic reduction (FGPR) and ultrasound-guided hydrostatic reduction (UGHR).MethodsThis retrospective study was conducted from January 2019 to December 2022 involving children admitted and managed for acute intussusception in a tertiary-care pediatric hospital in China. The epidemiology, clinical features, and therapeutic effects were analyzed using IBM SPSS Statistics 20.0.ResultsThe study included 401 boys (68.43%) and 185 girls (31.57%) aged from 2 months to 12 years. The most common symptoms reported were abdominal pain or paroxysmal crying (95.73%), vomiting (45.39%), and bloody stool (7.34%). Vomiting and bloody stool became atypical with increasing age (P < 0.001). The total success cases of reduction accounted for 563 cases (96.08%), and the recurrent cases accounted for 71 cases (12.12%). No significant difference was observed in the success or recurrence rates between FGPR and UGHR (P > 0.05). Abdominal pain was an independent protective factor for successful enema (P < 0.01, OR = 72.46), while bloody stool (P < 0.01, OR = 0.06) and older age were independent risk factors (P < 0.001, OR = 0.51). Of the 71 patients with recurrent intussusception, 29 were successfully reduced by enema, and the other 23 required surgical reduction. Twelve of the surgical cases were secondary intussusception, including three cases of Meckel's diverticulum, five cases of polyps, and four cases of non-Hodgkin lymphoma.ConclusionThe epidemiological characteristics of children with intussusception in Xiamen showed peculiarity with a higher male-to-female ratio, older age at diagnosis, and no significant seasonality. Both FGPR and UGHR were effective and safe for intussusception, and surgical reduction was essential for patients with failed enema reduction.

目的:介绍1对胸腹联体儿合并一胎心脏畸形的特殊治疗体会.方法:选择在复旦大学附属妇产科医院进行产前诊断并出生、在复旦大学附属儿科医院治疗的1对胸腹联体双胎,其中一胎合并心脏畸形,总结诊疗经过,分析特殊临床表现及治疗的个体化方案.结果:足月剖宫产,两患儿尿量差异大、体质量增加稍缓慢,且先心患儿在出生后1个月开始出现哭吵后青紫.于出生后38d行分离术,术后9d,先心患儿再次行B-T分流术.分离术后2周,无先心患儿顺利出院.先心患儿B-T分流术后20 d亦顺利出院.结论:胸腹联体虽常见,但合并心脏畸形增加手术风险,需结合心脏疾病程度与临床表现,制定个体化治疗方案,进而能提高救治成功率.

随着腹腔镜技术的发展与推广,腹腔镜技能培训也逐步成为住院医师规范化培训的重要内容.复旦大学附属儿科医院作为上海市试点的第一批小儿外科培训基地和全国第一批小儿外科培训基地,十分重视住院医师腹腔镜手术技能培训.儿外科教研室和外科专业基地共同针对小儿外科腹腔镜微创手术的特点,结合医院条件和住院医师的实际情况,制订了本院小儿外科住培腹腔镜技能培训方案,对住院医师进行针对性、系统性的培训,既突出了基本技能的训练,又结合小儿手术特点安排课程,取得了良好的培训效果.

新生儿脐炎可致命 新生儿脐炎是新生儿科较常见的感染类疾病.其主要是由于新生儿断脐及出生后脐部没有得到正确护理,造成脐部皮肤受到各种细菌感染而引起的急性炎症. 新生儿脐炎若处理不及时,将会造成炎症的进一步扩散,致使新生儿出现腹膜炎、败血症等,影响其食欲,对新生儿的生长发育极为不利,严重时还会危及新生儿生命.

Biliary atresia (BA) is the most common cause of chronic cholestasis in children. The long non-coding RNA (lncRNA) Annexin A2 pseudogene 3 (ANXA2P3) and Annexin A2 (ANXA2) have been suggested to serve pivotal roles in BA; however, the clinical significance and biological roles of ANXA2P3 and ANXA2 in BA remain to be elucidated. The present study aimed to elucidate the function of ANAX2P3 and ANXA2 in BA-induced liver injury using a human liver cell line and liver tissues from patients with BA. Reverse transcription-quantitative polymerase chain reaction, western blotting and immunohistochemistry were conducted to determine the expression levels of ANXA2 and ANXA2P3 in liver tissues from patients with BA. Classification of fibrosis was analyzed by Masson staining. The functional roles of ANXA2 and ANXA2P3 in liver cells were determined by Cell Counting kit-8 assay, and flow cytometric and cell cycle analyses. Activation of the ANXA2/ANXA2P3 signaling pathway in liver cells was evaluated by western blot analysis. According to the present results, the expression levels of ANXA2 and ANXA2P3 were significantly increased in liver tissues from patients with BA. In addition, knocking down the expression of ANXA2P3 and ANXA2 may result in reduced liver cell proliferation, cell cycle arrest in G(1) phase and increased apoptosis of liver cells in vitro. Furthermore, in cells in which ANXA2 and ANXA2P3 were overexpressed, cell apoptosis was reduced and cell cycle arrest in G(2) phase. Taken together, these results indicated that ANXA2P3 and ANXA2 may have protective effects against liver injury progression and may be considered biomarkers in patients with BA.

目的 调查小儿腹股沟斜疝术后线结反应的发生率,并分析其危险因素.方法 以2015年7月至2016年6月于枣庄市妇幼保健院儿外科接受手术治疗的1307例腹股沟斜疝患儿为研究对象,按照手术方式的不同分为开放组及腹腔镜组,所有患儿随访至少10个月.回顾性分析发生线结反应患儿的人口学资料、临床表现、过敏史、手术时间、反应侧别情况,采用χ2检验对影响线结反应发生的因素进行分析.结果 腹腔镜组和开放组的线结反应发生率分别为2.43％和0.82％,两组间无统计学差异(P=0.052).腹腔镜术后线结反应具体表现包括:皮下结节(11例)、脓肿(7例)、皮肤瘘(4例).其中术后1个月内发生9例(45％),术后1～6个月内发生9例(45％),术后6个月以后发生2例(10％).应用丝线及Prolene线的患儿线结反应的发生率无统计学差异(P=0.633).双侧斜疝患儿的线结反应发生率高于单侧斜疝患儿(P=0.011).行双侧腹腔镜斜疝修补术患儿术后发生线结反应的侧别中,左侧占42.86％(6/14),右侧占42.86％(6/14),双侧占14.29％(2/14).3例(2例左侧,1例右侧)经取出缝线后痊愈,2例(均为右侧)复发.结论 线结反应是腹腔镜腹股沟斜疝术后的重要并发症之一,术后1个月内线结反应发生率较高.双侧斜疝可能是造成腹腔镜腹股沟斜疝术后线结反应发生的高危因素之一.

目的:收集胆囊扭转病例资料,总结相关临床、辅助检查结果,提高术前诊断准确性.方法:报道1例儿童胆囊扭转病例,回顾性分析其临床表现、辅助检查图像资料、手术过程,并复习相关文献.结果:胆囊扭转多发生于老年女性,儿童发病率极低,病程短,胆囊区疼痛明显,可伴有呕吐症状,无发热、黄疸.B超下胆囊可见"8"字改变,交叉部高回声.磁共振胰胆管造影(MRCP)可观察到胆囊影中断或胆囊管呈尖状.CT极少数可能看到胆囊螺旋形改变.结论:儿童中胆囊扭转罕见,症状易混淆,B超、MRCP具有重要术前诊断价值.

目的:通过对术后并发症分析,探讨腹腔镜幽门肌切开术对先天性肥厚性幽门狭窄(CHPS)治疗的有效性、安全性.方法:对复旦大学附属儿童医院行腹腔镜幽门肌切开术治疗CHPS后需要再次手术患儿临床资料进行分析.结果:4例再手术病例中,1例患儿术后12 h因气腹行开放手术,3例患儿因幽门肌分离不全再次手术,总体再次手术率为1.52％(4/264).结论:腹腔镜幽门手术安全可靠,可以获得较好的治疗效果,再次手术主要原因是幽门肌分离不全和十二指肠穿孔.

A Meckel's scintigraphy using TcO4 was performed in a 9-year-old boy. The images revealed an elongated abnormal radiotracer accumulation in the right abdomen, which was confirmed as Meckel diverticulum combined with an omphalomesenteric cyst.

This study aimed to analyze the impact of etiologic heterogeneity and operation age on prognosis of infants with biliary atresia (BA) who received Kasai operation prior to 60 days of age.From 2004 to 2010, 158 infants received Kasai operation before turning 60 days old. According to Davenport 2012 classifications, 4 groups of BA were defined: cystic BA, syndrome BA, and associated malformation, cytomegalovirus (CMV)-associated BA, and isolated BA. Native (autologous) liver survival rates and incidence of cholangitis 2 years after operation, as well as jaundice clearance rates 3 months after operation, were recorded.Although infants who received the operation between 51 and 60 days of age had a better jaundice clearance 3 months after operation and lower incidence of cholangitis as compared with those under 40 or between 41 and 50 days of age, there was no significant difference in survival rates. Among types of BA, infants with cystic BA had the best prognosis. In the syndrome BA and associated malformations group, as well as CMV-associated group, infants who received the operation early (<40 days of age) had a worse outcome as compared with those who received the operation between 41 and 50 days or 51 and 60 days of age.Both clinical etiologic heterogeneity and operation age may influence BA prognosis.

AIM To investigate multiple polyps in a Chinese Peutz-Jeghers syndrome (PJS) infant. METHODS A nine-month-old PJS infant was admitted to our hospital for recurrent prolapsed rectal polyps for one month. The clinical characteristics, a colonoscopic image, the pathological characteristics of the polyps and X-ray images of the intestinal perforation were obtained. Serine threonine-protein kinase 11 (STK11) gene analysis was also performed using a DNA sample from this infant. RESULTS Here we describe the youngest known Chinese infant with PJS. Five polyps, including a giant polyp of approximately 4 cm × 2 cm in size, were removed from the infant's intestine. Laparotomy was performed to repair a perforation caused by pneumoperitoneum. The pathological results showed that this child had PJS. Molecular analysis of the STK11 gene further revealed a novel frameshift mutation (c.64_65het_delAT) in exon 1 in this PJS infant. CONCLUSION The appropriate treatment method for multiple polyps in an infant must be carefully considered. Our results also show that the STK11 gene mutation is the primary cause of PJS.

Objective To analyze the impact of etiologic heterogeneity and operative age on short-term outcomes of biliary atresia (BA) undergoing Kasai operation under 60 days.Methods During the period of 2004-2010,a total of 476 BA infants were reviewed.According to the preoperative examinations,4 groups could be defined clinically as cystic BA,syndromic with associated malformations,cytomegalovirus (CMV)-associated and isolated.Results Among them,158 infants underwent Kasai operation under 60 days.There were cystic (n =19),syndromic with associated malformations (n =36),CMV-associated (n =35) and isolated (n =68).The best outcomes were obtained in infants undergoing Kasai operation between 51 to 60 days (62.1％,46/74) while those operated under 40 days had the worse prognosis (42.8％,12/28).Infants with cystic BA had better outcomes than other three groups (68.4％,P＜0.05).Furthermore,this group had favorable prognosis in any operative age.Significant differences existed among the groups of syndromic with malformations (55.9％),CMV-associated BA (57.1％) and isolated BA (52.9％,P＞0.05).Syndromic with malformations group,CMVassociated BA group and those with an early operation (＜40 days) had worse outcomes than those operated between 41-50 days and 51-60 days(P =0.041＜0.05,P =0.029＜0.05).However,in isolated group,infants operated under 40 days had the same outcome with those between 41-50 days (50.0 ％ vs 57.1％,P＞0.05),and also had no significance with those from 51-60 days (50.0％ vs 54.8％,P＞0.05).Conclusions To some extent,operative age has impact on outcome.When Kasai operation is performed under 60 days,infants operated from 51-60 days achieve better outcomes.Clinical etiologic heterogeneity may have prognostic significance.

目的 分析胆道闭锁手术年龄与术后早期效果的关系以及较大年龄(＞90 d)患儿的Kasai手术指征.方法 2004-2010年复旦大学附属儿科医院收治胆道闭锁患儿452例,均经术中胆道造影确诊.手术采用标准的Kasai术,术后常规使用激素,对不同年龄组患儿术前肝功能、B超等资料及术后胆红素下降情况进行分析,总胆红素水平低于20 mmol/L定为黄疸完全消退.结果 将所有患儿根据年龄分为三组,手术年龄≤60 d者146例,手术年龄在60～90 d者222例,＞90 d者84例(90～10Od者33例,100～110 d 26例,110～120d 10例,120～ 130 d 8例,＞130 d 7例).术前各年龄组总胆红素、直接胆红素、谷丙转氨酶无显著差异.术后2周,60 d以内组,胆红素下降水平最低(P＜0.05),＜45 d患儿胆红素下降水平并未更加显著.术后3个月随访率为61.3％,各年龄组总胆红素水平无显著差异(F=0.132,P=0.970).术后6个月随访率37.4％,90 d以上组总胆红素(58.09±58.55) mmol/L,90 d以内组总胆红素水平(27.67±30.60) mmol/L(P=0.226).＞90d患儿,每间隔10d分成一组,各组间术后早期胆红素下降水平无差异(F=1.115,P=0.355).＞90 d手术患儿两年自体肝生存率为36.1％.90 d以上患儿延误手术原因:39.2％因家长未重视,51.6％因误诊婴儿肝炎耽误治疗.结论 胆道闭锁患儿＞90 d并非手术绝对禁忌,多数患儿可取得较好的早期黄疸消退,部分患儿术后6个月可以有较好的肝功能恢复.

Objective. The dosage, duration, and the benefits of high-dose steroid treatment and outcome in biliary atresia (BA) remain controversial. In this study, we evaluated the impact of high-dose steroid therapy on the outcome of BA after the Kasai procedure. Methods. Intravenous prednisolone administration was started 1 week after surgery, followed by 8 to 12 weeks of oral prednisolone. Total bilirubin (TB) levels (3, 6, and 12 months after surgery), early onset of cholangitis, and two-year native liver survival were evaluated. Results. 53.4%, 56.9%, and 58.1% of the patients in the high-dose steroid group were jaundice-free 3, 6, and 12 months after surgery, respectively; these values were significantly higher than the 38.7%, 39.4%, and 43.3% of the low-dose steroid group. One year after surgery, the incidence of cholangitis in the high-dose group (32.0%) was lower than that in the low-dose group (48.0%). Infants with native liver in the high-dose group had a better two-year survival compared to those in the low-dose steroid group (53.7% versus 42.6%). Conclusions. The high-dose steroid protocol can reduce the incidence of cholangitis, increase the jaundice-free rate, and improve two-year survival with native liver after the Kasai operation.

ABSTRACT Objectives: Biliary atresia (BA) is a devastating pediatric cholestatic liver disease. Increasing evidence indicates that nuclear factor (NF)‐κB signaling plays a key role in the pathogenesis of BA. Leucine zipper downregulated in cancer 1 (LDOC1) may control the expression of NF‐κB. The aim of this study was to evaluate the relation between LDOC1 and inflammation/apoptosis mediated by NF‐κB in the human intrahepatic biliary epithelial cells (HIBECs). Methods: HIBECs were divided into 3 treatment groups: control, mock transfection group, and LDOC1 transfection. Immunofluorescence, reverse transcription polymerase chain reaction, Western blot, and flow cytometry analysis were used to investigate the effectiveness of LDOC1‐transfected HIBECs and the expression of NF‐κB. Apoptosis was detected by Hochest/ propidium iodide staining. Interleukin (IL)‐2 and tumor necrosis factor (TNF)‐α levels were evaluated by enzyme‐linked immunosorbent assay. Results: The expression of NF‐κB was higher in the LDOC1‐transfected group when compared with the control and mock‐transfected groups as evaluated by immunofluorescence, reverese transcription polymerase chain reaction, and Western blot analysis. The rate of apoptosis was significantly lower in the LDOC1‐transfected group when compared with the control and mock‐transfected groups. The levels of IL‐2 and TNF‐α were significantly higher in the LDOC1‐transfected group when compared with the control and mock‐transfected groups. Conclusions: Upregulation of LDOC1 in HIBEC increases the expression of NF‐κB, which may promote the activation of IL‐2 and TNF‐α secretion and inhibit cell apoptosis.

Background and Study Aims: Biliary atresia (BA) is a devastating disease of infants, invariably leading to cirrhosis, end-stage liver disease, and death if untreated. The etiology of BA is unknown, although infectious, immune, and genetic causes have been suggested. This study was designed to investigate whether polymorphism of the ITGB2 (CD18) gene is associated with susceptibility to BA. Methods: The ITGB2 gene promoter and 16 exons were genotyped following amplification and sequencing, with associations assessed using Fischer's exact test in 106 patients diagnosed with BA and 108 unrelated healthy controls. Results: We found one single nucleotide polymorphism (SNP) in the ITGB2 promoter region (-680 C/T) and five SNPs in exons, including: -111 T/C in exon 1, 117 G/A in exon 3, 819 G/A in exon 7, 1101 C/A in exon 10, and 3′-UTR+145C/A in exon 16. There were no significant differences in genotype and allelic frequencies of any of the SNPs between controls and patients with BA in both the promoter and exons 1, 3, 7, and 10. 3′-UTR+145C/A showed a significant increase in the C allele frequency (OR = 2.19, 95% CI: 1.39-3.46, p = 0.0006) and a significant increase in the CC genotype (p = 0.001) in BA patients compared with healthy controls. Using a reporter gene assay, the construct that contained the risk allele (3′-UTR+145 C) showed significantly higher luciferase activity than the nonrisk A allele (p = 0.007). Conclusion: Our study provides the first evidence of a possible role of ITGB2 3′-UTR+145C/A polymorphism in the pathogenesis of BA.