Objective To evaluate the outcomes of jejunal turnover and bowel plication (JTBP) in high jejunal atresia of neonates. Materials and Methods The clinical data of neonates that met the criteria were retrospectively analyzed from January 2012 to December 2021. The neonates were divided into the JTBP group and control group according to the surgical procedure. Demographics, postoperative morphology of the duodenum and proximal jejunum, intestinal recovery time, and complications were compared. Results A total of 75 patients were allocated to the JTBP (n = 30) and control (n = 45) groups, respectively. There was no significant difference between the two groups in terms of gestational age, birth weight, age at surgery, the pathological classification, and concomitant disease. Upper gastrointestinal contrast study showed that the diameter of the proximal bowel of the anastomotic stoma was normal and the duodenum and proximal jejunum were in streamline shape in the JTBP group. While the duodenum was dilated, the shape of Trojan angle was classified into sharp angle and blunt round angle in the control group. The duration of total parenteral nutrition, postoperative oral feeding time, and oral feeding time of 40 mL/3 h were significantly different between the JTBP group and control group (sharp and blunt round type): 9.0 +/- 3.5, 7.0 +/- 2.1, and 11.0 +/- 6.0 versus 16.9 +/- 4.2, 14.0 +/- 5.0, and 19.0 +/- 7.4 versus 11.9 +/- 8.3, 8.2 +/- 3.9, and 15.8 +/- 3.6 days (p < 0.05). Conclusions JTBP for neonatal high jejunal atresia can significantly change the diameter of the proximal bowel and the course of duodenum jejunum flexure. Postoperative bowel movement was more in line with fluid dynamics, which was conducive to the recovery of the intestinal function and resulted in fewer complications.

Congenital chylous ascites (CCA) is a rare cause of ascites in newborn infants. The main causes include congenital lymphatic obstruction due to atresia or stenosis of the major lacteals, mesenteric cysts and lymphangiomatosis. The mainstay of treatment for CCA is conservative management including medium-chain triglycerides (MCT)-based diet or total parenteral nutrition (TPN), and the addition of octreotide. Surgical exploration is reserved for those cases in whom conservative management has failed. The core problem of chylous abdominal surgery is to find the leakage; once the exact chylous leakage is found, the problem will be solved. The authors used a new carbon nanopartides material to accurately locate the location of chylous leakage. The operation is simple and fast, easy to use, and the effect is remarkable.

BACKGROUND AND AIMS:Natural killer (NK) cells play an important role in biliary atresia (BA) pathogenesis; human poliovirus receptor (PVR) is an important NK-cell modulator. Here, we explored the role of PVR in BA pathogenesis.METHODS:Poliovirus receptor expression and NK cell-associated genes were detected in human BA samples and a rotavirus-induced BA mouse model using quantitative PCR and immunofluorescence staining. Chemically modified small interfering RNA silenced PVR expression in the BA model, and its effects on the population and function of intrahepatic NK cells were investigated using flow cytometry (FCM). The effects of PVR overexpression and knockdown on proliferation, apoptosis and NK-cell-mediated lysis of cultured human cholangiocytes were analysed using FCM and cell viability assays. Serum PVR, high-mobility group box 1 (HMGB1), and interleukin-1beta (IL-1beta) levels were measured in a cohort of 50 patients using ELISA.RESULTS:Poliovirus receptor expression was upregulated in the biliary epithelium of BA patients and BA model and was positively correlated with the population and activation of intrahepatic NK cells. Silencing of PVR expression impaired the cytotoxicity of NK cells, reduced inflammation and protected mice from rotavirus-induced BA. Activation of the TLR3-IRF3 signalling pathway induced PVR expression in cultured cholangiocytes. PVR overexpression promoted proliferation and inhibited the apoptosis of cholangiocytes but exacerbated NK cell-mediated cholangiocyte lysis. Serum PVR levels were elevated in BA patients and were positively correlated with HMGB1 and IL-1beta levels.CONCLUSIONS:Poliovirus receptor contributes to BA pathogenesis by regulating NK cell-mediated bile duct injury; PVR has the value as a biomarker of BA.

Objectives To identify factors associated with outcomes of Kasai portoenterostomy (KPE), and predictors of 2- and 5- year native liver survival (NLS) for infants achieved jaundice clearance (JC) within 6 months of KPE.Methods This retrospective cohort study was conducted on 151 patients with type III biliary atresia (BA) who underwent KPE at our center. Univariate analysis and logistic regression analyses were performed to identify factors associated with NLS in infants achieved JC. Kaplan–Meier curves and log-rank tests were used to estimate the NLS, and the Cox proportional hazards regression model identified variables most associated with 2- and 5-year NLS at 6 months post-KPE. A receiver operating characteristic (ROC) curve was used to evaluate the predictive value of these factors.Results The 2- and 5-year NLS of infants achieved JC at 3 months post-KPE were not different from those achieved JC earlier. Operation age and total bile acid (TBA) were factors associated with JC. For infants who have achieved JC, DB was the only factor associated with 2-year NLS, the AUC was 0.872, the cutoff value was 14 μmol/L; ALB and DB were factors associated with 5-year NLS, the AUCs were 0.894 and 0.95, and the cutoff values were 39 g/L and 14 μmol/L, respectively.Conclusions NLS should be estimated at 6 months post-KPE. Preoperative factors are not predictive of NLS. For infants cleared jaundice, DB and ALB can predict NLS with good performance.What’s Known on This Subject Age, liver stiffness, and CMV infections are factors associated with outcomes of Kasai portoenterostomy. Jaundice clearance is directly associated with native liver survival; however, even with successful surgery, liver pathology in most cases will progress to end-stage cirrhosis.What This Study Adds No preoperative factors are predictive of native liver survival (NLS). Infants cleared jaundice after 3 months of KPE can achieve the same NLS as those cleared jaundice earlier. For infants cleared jaundice, 6-month postoperative DB and Albumin are predictive of NLS.How this study might affect research, practice or policy In this study, we argued that 6 months post-KPE was the appropriate timing for predicting NLS; direct bilirubin (DB) and albumin (ALB) at 6 months post-KPE can be used to predict 2- and 5-year NLS with good performance.Article Summary Retrospective analysis revealed it’s difficult to predict outcomes of Kasai portoenterostomy (KPE) preoperatively; jaundice clearance should be evaluated at 6 months after KPE, for infants cleared jaundice, 6-month postoperative DB and Albumin are predictive of NLS.### Competing Interest StatementThe authors have declared no competing interest.### Funding StatementNational Natural Science Foundation of China, 82170529; the National Key Researech and Development Program, 2021YFC2701003; Liaoning Revitalization Talents Program, XLYC1908008.### Author DeclarationsI confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained.YesThe details of the IRB/oversight body that provided approval or exemption for the research described are given below:Ethical approval for this study was obtained from the Research Ethics Committee of Shingjing Hospital, China Medical UniversityI confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals.YesI understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance).YesI have followed all appropriate research reporting guidelines and uploaded the relevant EQUATOR Network research reporting checklist(s) and other pertinent material as supplementary files, if applicable.YesAll data produced in the present study are available upon reasonable request to the authors All data produced in the present work are contained in the manuscript* KPE : Kasai portoenterostomy BA : biliary atresia JC : jaundice clearance JUC : jaundice unclearance LT : liver transplantation NLS : native liver survival PLT : platelet INR : international normalized ratio ALT : alanine aminotransferase AST : aspartate aminotransferase GGT : gamma-glutamyl transpeptidase TBA : total bile acid ALB : albumin TB : total bilirubin DB : direct bilirubin IB : indirect bilirubin APRI : AST/PLT CMV : cytomegalovirus ROC : receiver operating characteristic curve AUC : area under the ROC

Objective:To explore the clinical characteristics of extensive necrotizing enterocolitis (NEC-T) in preterm infants and the related factors of total intestinal necrosis.Methods:From January 2011 to December 2020, clinical data were reviewed retrospectively for 42 NEC-T preterm infants (NEC-T group). And, during the same period, no necrotizing enterocolitis totalis (NEC-nonT) preterm infants (NEC-nonT group, including operation and non-operation sub-groups) were compared.The related factors of total intestinal necrosis were analyzed compared with NEC-nonT.Results:Forty-two NEC-T children accounted for 6.1%(42/686) of the total number of NEC during the same period.Compared with NEC-nonT counterparts, NEC-T preterm infants had a lower birth weight [(1501±740) vs.(1709±1102) grams] and a higher asphyxia rate[76.2%(32/42) vs.25%(161/644)]. The differences were statistically significant.Compared with NEC-nonT group, age of onset was younger [(7.8±7.6) vs.(14.6±13.4) days], time interval between onset and operation was shorter [(6.6±6.5) vs. (30.4±24.4) days], higher ratio of Bell phase Ⅲ[85.7%(36/42) vs.62.7%(404/644)], greater rate of post-onset shock [76.2%(32/42) vs.36.6%(236/644)] and higher mortality rate[100%(42/42) vs.5.0%(32/644)]. The differences were statistically significant.Analysis of related factors of intestinal necrosis: severe subwall pneumatosis in two groups in acute stage [52.4%(22/42) vs.21.0%(76/362)], hypotension [69.0%(29/42) vs.40.3%(146/362)], pneumoperitoneum[66.7%(28/42) vs.40.3%(176/362)], hyponatremia [85.7%(36/42) vs.51.4%(186/362)], abdominal erythema [88.1%(37/42) vs.18.2%(66/362)] and thrombocytopenia[85.7%(36/42) vs.15.5%(56/362)]. NEC-T group was significantly higher than NEC-nonT group and the difference was statistically significant; Logistic multiple stepwise regression analysis indicated that abdominal erythema, severe subwall pneumatosis, hyponatremia and thrombocytopenia were independent risk factors for NEC-T ( P<0.05). NEC-T score: 3 had 92% sensitivity and 68% specificity to NEC-T while the sensitivity of 5 decreased by 54% and the specificity was significantly higher than 98%. Conclusion:NEC-T premature infants have severe clinical symptoms, rapid disease progression and a high mortality.Newnates with NEC-T risk factors require close observations, reasonable analysis, early prevention and intervention to reduce the incidence and mortality of NEC.

Transcription factor p63 has critical functions in epidermal, hindgut/anorectal, and limb development. Human mutations in P63 correlate with congenital syndromes affecting the skin, anorectal, and limbs. Nevertheless, less are detected regarding networks and functions controlled by P63 mutations in dermal fibroblasts, which are closely related to skin physiology. To screen for new targets, we employed microarray technology to investigate the R226Q P63 mutation with regards to the resulting circular RNA (circRNA) profiles from P63 point mutations in human dermal fibroblasts (HDFs). In this study, we show that P63-mutant HDFs display reduced proliferation, collagen synthesis, and myofibroblast differentiation; circAMD1 was also downregulated in P63-mutant HDFs compared with wild-type HDFs. Furthermore, overexpressing circAMD1 rescued the functional and phenotypic alterations of p63-mutant HDFs. We as well determined that miR-27a-3p was circAMD1 target involved in effects of circAMD1 in P63-mutant HDFs. Collectively, our data show that circAMD1 functions as a miR-27a-3p sponge that inhibits the functional and phenotypical alteration of P63-mutant HDFs and may be a critical marker in pathogenesis regarding P63-associated traits.

Objective:To analyze the clinical characteristics and prognosis of twin premature infants with necrotizing enterocolitis (NEC).Methods:The clinical data of twin preterm infants with NEC treated in Shengjing Hospital of China Medical University from January 2009 to December 2018 were retrospectively analyzed and compared with singleton preterm infants, thus clarifying clinical characteristics and treatment outcomes of twin preterm infants with NEC.Results:The incidence of NEC in twin premature infants was significantly higher than that in singleton premature infants [8.1% (124 /1 539 cases) vs.3.8% (497/13 198 cases), χ2=62.887, P<0.001]. The number of small twins in natural delivery group was more than that of large twins [(23 cases vs.5 cases), χ2=8.09, P<0.05]. Compared with singleton NEC preterm infants, twin NEC preterm infants had significantly lower birth weight [(1 424±439) g vs.(1 761 ± 596) g, t=-15.07, P<0.001], higher rate of mechanical ventilation after birth [37.1% (46/124 cases) vs.17.9%(89 / 497 cases), χ2=15.539, P<0.001], and higher mortality [13.7%(17/124 cases) vs.7.0%(35/497 cases), χ2=5.401, P<0.05]. Compared with singleton preterm infants with NEC, twin preterm infants with NEC had significantly higher surgical treatment rate [54.8%(68/124 cases) vs.43.9%(218/497 cases), χ2=27.885], younger operation age [(20.6 ± 17.5) d vs.(29.4 ± 24.4) d, t =-5.673], higher degrees of anemia [(118.284 ± 22.429) g/L vs.(127.460±28.352) g/L , t=-3.398], thrombocytopenia [(213.57 ± 150.548)×10 9/L vs.(220.25 ± 169.610)×10 9/L, t =-3.238], metabolic acidosis(7.215 ± 0.211 vs.7.355±0.418, t=-4.207), rate of shock [(52.9%(36/68 cases) vs.36.7%(80/218 cases), χ2= 5.673], and the rate of mechanical ventilation[54.4% (37/68 cases) vs.35.8%(78/218 cases), χ2=7.484](all P<0.05). Extensive intestinal necrosis was the main cause of death in either singleton or twin preterm infants with NEC.After 1 year of follow-up, there was no significant difference in the proportion of growth retardation, the proportion of serious neurodevelopmental problems and mortality between the 2 groups (all P>0.05). Conclusions:Twin preterm infants born with poor physical fitness, and they have a high incidence of NEC with a rapid progression that require the early intervention.The operation rate of twin preterm infants with NEC is high and the postoperative complications are serious.Close observation, reasonable analysis, early prevention and intervention are needed to reduce the incidence and mortality of twin preterm infants with NEC and improve the prognosis.

Objective To identify the disease-causing gene mutation in two families of Hirschsprung's disease (HSCR).Methods Two HSCR families from Liaoning Province were collected to analyze genomic DNA.Whole-exome genome mutation screening and copy number variation (CNV) analysis were performed on whole-genome DNA extracted from blood using nextgeneration sequencing (NGS) technology.Combining the hazard and pathogenicity analysis of mutation and clinical phenotype of family members screening susceptible pathogenic gene for sequencing results.Then classical Sanger' s method was applied for verifying the obtained results.Results Among 4 persons in family 1,both children were affected,the mother had similar clinical manifestations but the diagnosis was absent,the father had no phenotype.After filtering out common mutations and synonymous mutations,633 SNPs and 35 InDel mutations were detected by wholeexome sequencing.The sequencing results revealed heterozygous mutation c.2599G＞T in exon 14 encoding region of RET gene and it was confirmed as a pathogenicity mutation through the hazard and pathogenicity analysis of mutated protein.Among 4 persons in family 2,both offsprings were identified as HSCR,but one of them dying in neonatal period failed to obtain a peripheral blood sample.Sequencing results revealed 609 SNPs and 30 InDel mutations.Comprehensive analysis of mutations,genetic patterns,clinical features and other factors failed to detect a distinct pathogenic mutation in this family.Conclusions NGS can screen out new HSCR-related gene mutations and provide etiological insights of HSCR.

目的 总结近5年产前诊断的先天性膈疝(congenital diaphragmatic hernia，CDH)的围生期诊治经验，探讨CDH在胎儿期的多学科会诊制度，新生儿期的手术治疗时机、手术方法及临床疗效。 方法 收集我院2012年5月至2017年5月通过产前诊断的先天性膈疝患儿38例，对病例进行回顾性分析。 结果 6例患儿生后家长放弃治疗，其中2例经高频通气及一氧化氮吸入仍不能维持有效血氧饱和度，余患者均行手术治疗。胸腔镜手术31例；5例中转开胸，其中2例膈肌巨大缺损，3例患儿不能耐受气胸。获得随访29例(90.6%)，随访7个月～4年10个月，平均(29±13)个月，均无复发或严重并发症。 结论 CDH的诊治需针对每个患儿的呼吸循环稳定性、肺发育情况、合并畸形等具体情况来制定个体化精准治疗方案。胸腔镜膈肌修补手术对于新生儿先天性膈疝的治疗是安全可行的方法。

Objective: To report newly identified mutations in two families in China with cystic renal disease. Case presentations: Two fetuses were found by prenatal ultrasound to have symmetrically enlarged kidneys with increased echogenicity and cystic changes. We isolated fetal and parental genomic DNAs from umbilical cord blood and circulating leukocytes, performed next generation sequencing for mutations, followed by Sanger sequencing for confirmation. We discovered two new heterozygous mutations in PKHD1: c.2507_2515delTGAAGGAGG (p.Val836_Glu838del) in exon 24 among the fetus and father, as well as c.6840G>A (p.Trp2280*) in exon 42 among the fetus and mother. A mutation of c.2507_2515delTGAAGGAGG caused deletion of three amino acids. Two heterozygous mutations in AHI1, c.1304G>A (p.Arg435Gln), and c.3257A>G (p.Glu1086Gly) were identified in the second fetus, while the former was also found in the mother. The mutated locus in AHI1 is highly conserved among humans, dogs, mice, and monkeys. Conclusions: We report two newly identified mutations in PKHD1 and AHI1. An accurate genetic diagnosis is crucial for genetic counseling of parents with offspring carrying cystic renal disease.

Objective To investigate the fetal management of prenatally diagnosed fetal mediastinal masses and the initial experience of neonatal thoracoscopic minimally invasive treatment. Methods We per-formed a retrospective study from November 2015 to November 2016 of all newborns affected by mediastinal masses and treated by thoracoscopic surgery. This group of cases were found with mediastinal masses by pre-natal ultrasound. The earliest detection of abnormal time was 16 to 31 weeks of pregnancy,with an average of 25 weeks. In the fetal period,the patients were treated with multidisciplinary consultation and individual man-agement. Prenatal examinations helped us except for chromosomal abnormalities and other organ abnormali-ties. After birth,the patients underwent CT and MRI examination. The diameter of the tumor was 1. 7 to 5. 7 cm,with an average of 3. 2 cm. The operative age was 4 to 29 days,with an average of 12. 4 days. This group of newborns were performed thoracoscopic mass resection and confirmed by intraoperative pathological exam-ination. Results After individualized precise prenatal management,all children were born successfully and confirmed that prenatal diagnosis was accurate. All mediastinal masses were completely excised in the neo-natal period. Five mediastinal masses were completely excised. One posterior mediastinum immature teratoma was converted to open thoracotomy. The mean operative duration was 112 min(100 to 150 min). There was no operative complication with a minimal amount of blood loss. With a smooth recovery,the hospital stay was 11-17 days. Pathological results included:1 esophageal duplication,2 bronchogenic cysts,1 lymphangioma, 1 cystic teratoma of anterior mediastinum,1 immature teratoma of posterior mediastinum. During a mean fol-low-up period of 8-14 months,neither complication nor recurrence occurred. Conclusion These are the pre-conditions for early treatment of neonatal patients with mediastinal masses,including definite prenatal diagno-sis,multidisciplinary consultation system and individualized and accurate fetal management. Throcoscopic ex-cision of mediastinal masses is both feasible and safe in neonates. Proper preoperative case selection may pre-vent a conversion into thoracotomy due to huge solid mass.

Objective To summarize the clinical experiences of 28 infants ovarian cysts treated by laparoscopic-assisted transumbilical extracorporeal cystectomy ( LATEC) in the last 4 years. Methods Twenty-eight neonatal and small infancy ovarian cysts were collected from June 2012 to June 2016. Retrospective analysis their clinical symptoms, discovery time, length of hospitalization, cyst size and nature, imaging characteristics, prenatal intervention, surgical treatment, postoperative pathological examination and follow-ups. Results The involved sides were unilateral (n=23) and bilateral (n=5). The monthly ages were <1 (n=11), 1-3 (n=11) and 3-6 (n=6). The manifestations included abdominal mass (n=27) and abdominal distension (n=1). 20 cases were found in fetal period, of which 1 case was treated by prenatal ultrasound guided cyst decompression. The average length of hospitalization was 7. 5 (4 -20) days. The sizes of ovarian cyst size were 4-5 cm (n=11), 5-10 cm (n=15) and >10 cm (n=2). All patients underwent laparoscopic-assisted transumbilical extracorporeal cystectomy ( LATEC) , including excision of ipsilateral appendectomy ( n=15) and simple cystectomy (n=13). The natures of ovarian cyst were simple (n=21), follicular (n=4) and serous (n=3). All 28 cases were cured and remained recurrence-free during follow-ups. Conclusion LATEC is both safe and effective for neonatal and infantile ovarian cysts. When abdominal cystic mass is larger than 5 cm or cysts fail to disappear and even expand further, surgery is required. When cyst size is<5 cm but its origin comes from ovary or other sites, exploratory surgery is indicated. For huge cysts in fetus, it is necessary to perform such a fetal mini-invasive procedure as puncture decompression.

Objective Dickkopf1 (Dkk1) is an extracellular negative regulator of Wnt signaling pathway.This study was intended to explore the differential expression of Dkk1 in cloaca and hindgut in normal and anorectal malformation (ARM) rat embryos and examine potential association between Dkk1 and abnormal development of cloaca and hindgut in ARM.Methods Ethylenethiourea (ETU) was administered to 24 pregnant rats at gestational Day 10 via gastric gavage.And another 16 control pregnant rats received saline alone.The gestational ages of collected specimens were 12-17,19 and 21 days respectively.Based upon anorectal morphology,the embryos were divided into two groups.Group ARM:ARM fetuses born from pregnant rats received ETU (n =96) and control group (n =96).Normal fetuses were from pregnant rats receiving saline.Cloaca and hindgut tissues were dissected microscopically for extracting total RNA and protein.Real-time polymerase chain reaction (RT-PCR) and Western blot were performed to detect the expressions of Dkk1 mRNA and protein respectively.The experiment was analyzed by two-way ANOVA of randomized block design.Results Dkk1 mRNA and protein were detected in both groups.The expression of Dkk1 peaked at Day 16 in cloaca and hindgut of control group (2.77 ± 0.06,3.20 ± 0.76) while the expression of Dkk1 fluctuated in ARM group.The expression levels of Dkk1 mRNA and protein were remarkably different during cloacal and hindgut development between norrnal (2.00 ± 0.52,1.98 ± 0.65) and ARM embryos (0.96 ± 0.14,0.87 ± 0.14,P＜0.05).Conclusions A down-regulation of Dkk1 in cloaca and hindgut development may be partially related to maldevelopment of cloaca and hindgut in ARM.

目的 探讨基于微信的翻转课堂(Flipped classroom)教学模式在小儿外科教学中的可行性和应用效果.方法 选择中国医科大学附属盛京医院小儿外科45名硕士研究生为研究对象,构建小儿外科教学微信群,开展翻转课堂教学活动,对学生进行问卷调查.结果《学生对翻转课堂模式教学的执行情况调查表》和《学生对翻转课堂教学模式评价表》的调查结果表明,88％ 的学生能够在课前利用业余时间通过微视频进行学习,学生对翻转课堂学习模式比较满意,但在教学过程中仍存在较大的改进空间.结论 基于微信的翻转课堂教学模式有助于提高学生的学习积极性和学生临床思维的形成,是一种适合中国临床医学现状,切实可行并行之有效的临床医学教学方法.

The epithelial-mesenchymal transition (EMT) is important for generating multiple tissues during organismal development.This process is particularly essential for the gastrulation of metazoans and neural crest delamination of vertebrates.EMT could be induced by multiple environmental factors,identifing the underlying mechanisms of important EMT factors is essential for indicating a precise role for EMT in embryonic stem cells,and characterizing the relationship between EMT and ESCs,Which might contribute in the pathogenesis of the genetic diseases and congenital malformations.

目的 评价添加益生元对胎龄小于37周的新生儿坏死性小肠结肠炎(necrotizing enterocolitis，NEC)发病率、晚发型败血症和病死率的预防作用。 方法 系统检索PubMed、OVID、Web of science英文数据库以及CNKI、万方、维普中文数据库，查找所有研究预防性使用益生元对降低新生儿NEC的发病率的随机临床对照试验。按纳入排除标准进行文献筛选、资料提取后应用Stata 11.0软件进行Meta分析。 结果 7篇随机对照试验纳入本次研究。试验组NEC发生率低于对照组，但差异无统计学意义(RR＝0.73，95%CI＝0.49－1.17，P＝0.195)。试验组晚发型败血症和病死率显著降低(RR＝0.71，95%CI＝0.54－0.93，P＝0.014；RR＝0.40，95%CI＝0.19－0.87，P＝0.021)。 结论 添加益生元可有效降低早产儿晚发型败血症发生率以及病死率。

Biliary atresia (BA) is a rapidly progressive disease in neonates characterized by fibro-obliteration of the extrahepatic biliary tree that results in chronic cholestasis and biliary cirrhosis. Although many studies have been conducted to explore the progressive mechanisms in and reasons for this disease, its etiology still remains unclear. Previous studies have suggested that epithelium-mesenchymal-transition (EMT) was involved in the progression of fibrosis and eventual disappearance of the biliary tract. It has been confirmed that the TGF-beta 1-SMAD-BMP7 signaling pathway is an important inducing factor in the EMT process, while CTGF and BMP7, which are important effective factors of this signaling pathway, contribute to EMT progression and fibrosis, respectively. In this study, we studied the expression of CTGF and BMP7 in the extrahepatic bile duct and liver tissue of rhesus rotavirus (RRV) by inducing biliary atresia in mouse models and explored the potential roles of CTGF and BMP7 in the development of BA. Methods: BALB/c mice were infected with RRV intraperitoneally within 24 hours after birth, while the control group was administered saline. The animals were observed, and their general condition was recorded. The animals were sacrificed on day 1, 3, 5, 7, 10, 15, and 20 after inoculation; extrahepatic bile ducts and liver tissues were collected; the CTGF and BMP7 expression levels in the BA and control groups were detected with immunohistochemistry; and real-time quantitative RT-PCR and Western blot results were compared between the BA group and normal control using Student's t test. Results: The animals that were inoculated with RRV displayed symptoms of cholestasis beginning on the fifth day of life, including jaundice, grey stool, oily fur, lethargy, and growth retardation. CTGF expression in the BA group increased shortly after injection and continued at high levels; the BMP7 expression increased after CTGF, and the difference between the two groups became less evident beginning on day 15. Conclusions: CTGF and BMP7 expression in the hepatobiliary tissue of MMU18006-exposed mice significantly increased between the BA and control groups, indicating their potential role in the pathogenesis of BA; the duration of high BMP7 expression was shorter than that of CTGF that suggested the anti-fibrosis role was limited.

Objective To explore a predisposing gene research platform for congenital malformations in mice caused by transplacental RNAi-EphB2.Methods The EphB2 down-regulatory plasmid SiEphB2 and vector in Ringer's solution were delivered into tail vein of mice 9.5 days of pregnancy.Plasmids for SiRNA contained three different sites of RNAi for silencing EphB2 gene.The embryos were removed 48 h after injection and reabtime quantitative polymerase chain reaction (PCR) and Western blot were used for detecting the expression of EphB2.The anorectal development of newborn and1-week-old mice was observed.Results The expressions of EphB2 mRNA in all three SiEphB2 groups (SiEphB2-1 : 0.27 ± 0.03;SiEphB2-2: 0.34 ± 0.04;SiEphB2-3: 0.42 ± 0.02) were significantly lower than those in Ringers group (0.99 ± 0.06) and in vector group (0.87 ± 0.07).The mRNA expression of EphB2 in SiEphB2 embryos was down-regulated by (65.3 ± 6.6) ％ versus Ringers group and by (60.6 ± 8.6) ％ versus vector group (P＜0.05).The expression of EphB2 protein in SiEphB2 groups (SiEphB2-1 : 0.20 ± 0.03;SiEphB2-2: 0.33 ± 0.02;SiEphB2-3: 0.39 ± 0.02) decreased markedly versus that in Ringer' s group (1.00 ± 0.05) and vector group (0.93 ± 0.09).The protein level of EphB2 decreased by (69.3± 7.8)％ in SiEphB2 embryo group versus Ringer's group and by (66.9 ± 9.6)％ versus vector group(P＜0.05).In SiEphB2 group, normal development of anus and tail was confirmed by gross observation and hernatoxylin & eosin (HE) staining of sagittal section.No anorectal malformation was found in SiEphB2 group.Conclusions Systemic delivery of SiEphB2 provides a valuable approach to gene silencing in embryos.

Objective To retrospectively evaluate different methods (laboratory tests,liver / gallbladder ultrasound and magnetic resonance cholangiopancreatography)in differentiating biliary atresia from infant hepatitis syndrome.Methods Seventy infants with cholestatic jaundice,50 cases of biliary atresia and 20 cases of infant hepatitis syndrome were studied prospectively from January 2010 to December 2012.All cases underwent abdominal ultrasound and magnetic resonance cholangiopancreatography.The accuracy,sensitivity,specificity and predictive values of these various methods were compared.Also the laboratory parameters were statistically analyzed and compared between groups.Results Patients with BA had significantly higher GGT values at presentation [(743.5 ± 564) IU/L] compared with infants with IHS [(198.8 ± 197.8) IU/L],showing statistically significant difference (P ＜ 0.05).The sensitivity,specificity,and accuracy of the ultrasound in diagnosis of BA were 84.0％ 、100.0％ 、88.6％ respectively(P ＜ 0.05).The values for magnetic resonance cholangiopancreatography were 82.0％ 、80.0％ 、81.4％ (P ＜ 0.05).Conclusion Currently,the method of ultrasound is more reliable than MRCP for differentiating biliary atresia from infant hepatitis syndrome.

The aims of this study were to identify the mutation gene of a Chinese family with anorectal malformation (ARM) associated with split hand–foot malformation and to determine the spatiotemporal expression of the mutated gene during hindgut and anorectum development in human embryos.