Large electronegativity differences between adjacent atomic sites activate and stabilize ZnIn2S4 for efficient photocatalytic overall water splitting.

Photocatalytic overall water splitting into hydrogen and oxygen is desirable for long-term renewable, sustainable and clean fuel production on earth. Metal sulfides are considered as ideal hydrogen-evolved photocatalysts, but their component homogeneity and typical sulfur instability cause an inert oxygen production, which remains a huge obstacle to overall water-splitting. Here, a distortion-evoked cation-site oxygen doping of ZnIn2S4 (D-O-ZIS) creates significant electronegativity differences between adjacent atomic sites, with S1 sites being electron-rich and S2 sites being electron-deficient in the local structure of S1-S2-O sites. The strong charge redistribution character activates stable oxygen reactions at S2 sites and avoids the common issue of sulfur instability in metal sulfide photocatalysis, while S1 sites favor the adsorption/desorption of hydrogen. Consequently, an overall water-splitting reaction has been realized in D-O-ZIS with a remarkable solar-to-hydrogen conversion efficiency of 0.57%, accompanying a ~ 91% retention rate after 120 h photocatalytic test. In this work, we inspire an universal design from electronegativity differences perspective to activate and stabilize metal sulfide photocatalysts for efficient overall water-splitting.

Single cell RNA-sequencing analysis reveals that N-acetylcysteine partially reverses hepatic immune dysfunction in biliary atresia.

Background & Aims: Our previous study indicated that CD177+ neutrophil activation has a vital role in the pathogenesis of biliary atresia (BA), which is partially ameliorated by N-acetylcysteine (NAC) treatment. Here, we evaluated the clinical efficacy of NAC treatment and profiled liver-resident immune cells via single cell RNA-sequencing (scRNA-seq) analysis to provide a comprehensive immune landscape of NAC-derived immune regulation. Methods: A pilot clinical study was conducted to evaluate the potential effects of intravenous NAC treatment on infants with BA, and a 3-month follow-up was carried out to assess treatment efficacy. scRNA-seq analysis of liver CD45+ immune cells in the control (n = 4), BA (n = 6), and BA + NAC (n = 6) groups was performed and the effects on innate cells, including neutrophil and monocyte-macrophage subsets, and lymphoid cells were evaluated. Results: Intravenous NAC treatment demonstrated beneficial efficacy for infants with BA by improving bilirubin metabolism and bile acid flow. Two hepatic neutrophil subsets of innate cells were identified by scRNA-seq analysis. NAC treatment suppressed oxidative phosphorylation and reactive oxygen species production in immature neutrophils, which were transcriptionally and functionally similar to CD177+ neutrophils. We also observed the suppression of hepatic monocyte-mediated inflammation, decreased levels of oxidative phosphorylation, and M1 polarisation in Kupffer-like macrophages by NAC. In lymphoid cells, enhancement of humoral immune responses and attenuation of cellular immune responses were observed after NAC treatment. Moreover, cell-cell interaction analysis showed that innate/adaptive proinflammatory responses were downregulated by NAC. Conclusions: Our clinical and scRNA-seq data demonstrated that intravenous NAC treatment partially reversed liver immune dysfunction, alleviated the proinflammatory responses in BA by targeting innate cells, and exhibited beneficial clinical efficacy. Impact and implications: BA is a serious liver disease that affects newborns and has no effective drug treatment. In this study, scRNA-seq showed that NAC treatment can partially reverse the immune dysfunction of neutrophil extracellular trap-releasing CD177+ neutrophils and Kupffer cells, and lower the inflammatory responses of other innate immune cells in BA. In consequence, intravenous NAC treatment improved the clinical outcomes of patients with BA in term of bilirubin metabolism.

Modified single-port versus multiport laparoscopic choledochal cysts excision and Roux-en-Y hepaticojejunostomy: a retrospective comparative cohort study.

Background: The feasibility, benefit, and safety of multiport laparoscopic choledochal cyst (CDC) excision and Roux-en-Y hepaticoenterostomy (MPCH) have been consistently confirmed. Single-port laparoscopic CDC excision and Roux-en-Y hepaticoenterostomy (SPCH) has advantages of less traumatic and more cosmetic beneficial, it has been reported in some case series, but it is technically challenging. We propose a modified technique to reduce technical difficulty in performing SPCH. The safety and feasibility of modified SPCH were compared with those of conventional multiport laparoscopic CDC excision. Methods: A total of 43 consecutive patients who diagnosed with CDC by preoperative magnetic resonance cholangiopancreatography (MRCP) and underwent SPCH (n=24) and MPCH (n=19) for choledochal cyst (CDC) by a single surgeon between January 1, 2018, and January 1, 2021, were enrolled. The baseline clinical characteristics, efficacy and safety outcomes of short-term were compared. Results: The baseline clinical characteristics of the MPCH and SPCH groups are comparable. Average postoperative length of hospital stay was shorter in the SPCH group than in the MPCH group, but the difference was not statistically significant (7.00 vs. 7.58 days; P>0.99). The operation time (281.75 vs. 277.3 min; P=0.58) and the amount of blood loss (9.33 vs. 16.68 mL; P=0.57) were similar in both groups. A significantly greater number of drainage tubes were placed in the MPCH group compared to the SPCH group (11 vs. 5; P=0.01). One patient suffered from hepaticoenterostomy anastomosis stricture in the SPCH group. Conclusions: The short-term outcome of modified SPCH is comparable with MPCH according to our study. It is easily adaptable treatment of CDC.

Nomogram for Estimating the Risks of Intestinal Ischemia and Necrosis in Neonates With Midgut Volvulus: A Retrospective Study.

Background: Delayed diagnosis and inaccurate judgment of the severity of the disease may be the principal reasons for the poor prognosis associated with neonatal midgut volvulus. We aimed to develop a nomogram model that timely assesses the risks of intestinal ischemia and necrosis in the neonate with midgut volvulus. Materials and Methods: We retrospectively analyzed the clinical data from neonates with midgut volvulus who were admitted to Guangzhou Women and Children's Medical Center from January 2009 to December 2019. Univariate and multivariate analyses were used to obtain independent factors to build a predictive model. The independent factors were used to develop the nomogram model. Results: Heart rate, mean arterial pressure, serum C-reactive protein, serum sodium, serum albumin, and pH levels were independent predictors for intestinal ischemia and necrosis in patients with midgut volvulus. The area under the receiver operating characteristic curve (AUC) of the predictive model was 0.985 (95% confidence interval, 0.966-0.999; P < 0.001). The sensitivity was 90.48%, and the specificity was 93.10%. A nomogram model was established using the six independent predictors, with a C-index of 0.859 and a favorable consistency between the predicted and actual intestinal ischemia and necrosis rates according to the internal validation. Conclusion: The constructed nomogram model could be a superior tool for predicting intestinal ischemia and necrosis in neonates with midgut volvulus.

CD177+ cells produce neutrophil extracellular traps that promote biliary atresia.

BACKGROUND & AIMS: We have previously reported on the potential pathogenic role of neutrophils in biliary atresia (BA). Herein, we aimed to delineate the role of CD177+ neutrophils in the pathogenesis of BA. METHODS: Immune cells from the livers of mice with rhesus rotavirus-induced BA were analysed. Single-cell RNA-sequencing was performed to specifically analyse Gr-1+ (Ly6C/Ly6G+) cells in the liver. Gene expression profiles of CD177+ cells were analysed using the Smart-Seq RNA-sequencing method, and the pathogenesis of BA was examined in Cd177-/- mice. Neutrophil extracellular trap (NET) inhibitors were used to determine the role of CD177+ cell-derived NETs in BA-associated bile duct damage, and a pilot clinical study evaluated the potential effects of N-acetylcysteine on NET release in BA. RESULTS: Increased levels of Gr-1+ cells were observed in the livers of mice with rhesus rotavirus-induced BA. RNA-sequencing analysis revealed that CD177+ cells were the main population of Gr-1+ cells and expressed elevated levels of both interferon-stimulated and neutrophil degranulation genes. Cd177-/- BALB/c mice exhibited delayed disease onset and reduced morbidity and mortality. High numbers of mitochondria were detected in CD177+ cells derived from mice with BA; these cells were associated with increased levels of reactive oxygen species and increased NET formation, which induced the apoptosis of biliary epithelial cells in cocultures. In a pilot clinical study, the administration of N-acetylcysteine to patients with BA reduced CD177+ cell numbers and reactive oxygen species levels, indicating a potential beneficial effect. CONCLUSIONS: Our data indicate that CD177+ cells play an important role in the initiation of BA pathogenesis via NET formation. CLINICAL TRIAL REGISTRATION: The pilot study of N-acetylcysteine treatment in patients with BA was registered on the Chinese Clinical Trial Registry (ChiCTR2000040505). LAY SUMMARY: Neutrophils (a type of innate immune cell, i.e. an immune cell that doesn't target a specific antigen) are thought to play a role in the development of biliary atresia (a rare but potentially lethal condition of the bile ducts that occurs in infants). Herein, we found that neutrophils expressing a particular protein (CD177) played an important role in bile duct damage by releasing a special structure (NET) that can trap and kill pathogens but that can also cause severe tissue damage. A pilot study in patients with biliary atresia showed that inhibiting NETs could have a beneficial effect.

Timing of operation in children with a prenatal diagnosis of choledochal cyst: A single-center retrospective study.

BACKGROUND/PURPOSE: There is currently no consensus on the timing of operative correction for patients with a prenatal diagnosis of choledochal cyst (CDC). This study aims to retrospectively analyze patients with prenatally diagnosed CDCs to identify the optimal timing of operative correction and the importance of cyst size as a predictor of the appearance of symptoms related to the CDC. METHODS: We reviewed 125 patients with a prenatal diagnosis of CDC who were admitted to Guangzhou Women and Children's Medical Center from July 2015 to July 2020. After dividing the patients into a symptomatic group (n = 37) and an asymptomatic group (n = 88), according to whether they had any clinical symptoms at the time of their operation, we compared their clinical data and postoperative outcomes. The asymptomatic group was divided into a <1 month group; a ≥1 month and <4 months group; and a ≥4 months group according to their postnatal age at operation; postoperative complications of the three groups were then compared. We were also interested in the effect of cyst size (width and length) for predicting the development of symptoms related to the CDC. RESULTS: The time of onset of symptoms after birth was mainly concentrated in the first 3 months (48.6%). The median width and length of cysts measured by preoperative magnetic resonance cholangiopancreatography in the symptomatic group were greater than those in the asymptomatic group (43 mm vs 28 mm and 71 mm vs 45 mm, respectively; P < .05). The serum levels of the liver-related enzymes ALT, AST, and GGT, and the serum level of DBIL, were greater in the symptomatic group than in the asymptomatic group (P < .05). The operative time, intraoperative blood loss, and duration of postoperative hospital stay in the symptomatic group were greater than those in the asymptomatic group (P < .05). In the asymptomatic group, there were no statistically significant differences in the surgical data and postoperative complications between the <1 month group, the ≥1 month and <4 months group, and the ≥4 months group. The area under the receiver operating characteristic curve (AUROC) of the length of the cyst in predicting symptoms was 0.747, the best cut-off point was 5.2 cm, and the sensitivity and specificity were 78% and 70%, respectively. The AUROC of the width of the cyst was 0.704, the best cut-off point was 4.1 cm, and the sensitivity and specificity were 68% and 75%, respectively. CONCLUSION: We maintain that it is advantageous to receive surgical treatment in the asymptomatic period for patients with a prenatally diagnosed CDC. A cyst size of length >5.2 cm and width >4.1 cm suggested that clinical symptoms might appear, and that surgery should be carried out as soon as possible, even in the neonatal period.

Flexible endoscopic identification and catheterization of congenital H-type tracheoesophageal fistula using a laryngeal mask.

Endoscopy for revealing the orifice of congenital H-type tracheoesophageal fistula (cTEF) is important for diagnostics and therapeutics. To facilitate the identification and catheterization of cTEF, we developed a new modified flexible endoscopy technique using a laryngeal mask with intermittent airflow. A retrospective case series study was conducted from April 2016 to July 2019 at a national regional children's medical center. Twelve infants with cTEF underwent this flexible endoscopy technique. The intermittent positive pressure airflow through laryngeal mask was able to reveal the orifice of cTEF easily in tracheal lumen. Under the visual flexible endoscope, cannulation with a 3-Fr ureteral catheter in fistula was successfully used in all cases. There were no immediate or delayed complications. This case series shows that the flexible endoscopy technique is a safe, easy, and technically efficient approach for diagnosis and cannulation of cTEF.

Long-term prophylactic intravenous antibiotics after Kasai portoenterostomy for biliary atresia do not reduce the risks of post-operative cholangitis, a retrospective study.

BACKGROUND: Postoperative cholangitis (PC) is the most common and serious complication of biliary atresia (BA) patients post-Kasai portoenterostomy (KPE). The duration of prophylactic intravenous antibiotics (IVA) after KPE varies with no clear consensus. We conducted a retrospective cohort study to explore the effects of IVA duration on preventing post-operative cholangitis and analyze the risk factors for cholangitis and short-term prognosis. METHODS: All patients diagnosed with BA and received KPE in Guangzhou Women and Children's Hospital in 2018, were included in this study. The patients received prophylactic IVA after KPE. Firstly, the patients were divided into two groups based on the presence or absence of PC (PC and NPC group). The correlation between PC and the IVA duration was analyzed, followed by a comparison of short-term prognosis, outcome, and other risk factors between the groups. Next, the patients were divided based on the median IVA duration of 11 days (long IVA and short IVA group), followed by a comparison of the incidence of PC, short-term prognosis, outcome, and other risk factors between the two groups. RESULTS: Totally 89 patients were included in this study. Amount them, eleven patients who were lost during follow-up, were excluded from the study. The prophylactic IVA duration of the PC (n=52) and NPC (n=25) groups was 12.6±8.5 and 13.0±4.5 days, respectively (P=0.79). Further, the jaundice clearance rate of the two groups was similar (PC: 31/52, NPC: 13/25, P=0.53). There was no difference in the incidence and frequency of cholangitis between the short (n=42) and long (n=35) IVA groups (27/42, 25/35, P=0.51), and the duration of IVA had no effect on jaundice clearance (24/42, 20/35, P=1.00). The short IVA group had a significantly shorter hospital stay than the long IVA group (16.2±5.1, 25.3±8.3, P=8.95×10-8). Patients undergoing KPE at an older age were at a higher risk of cholangitis (NPC: 60.6±19.7, PC: 72.3±17.8, P=0.01). CONCLUSIONS: A long duration of IVA after KPE for BA may not be necessary. Early diagnosed patients had timely surgery had a lower incidence of PC. Our findings may help in promoting the scientific use of antibiotics and reducing the LHS.

Early enteral feeding versus traditional feeding in neonatal congenital gastrointestinal malformation undergoing intestinal anastomosis: A randomized multicenter controlled trial of an enhanced recovery after surgery (ERAS) component.

PURPOSE: the aim of this clinical trial was to evaluate the safety and efficacy of early enteral feeding (EEN) following intestinal anastomosis in neonates with congenital gastrointestinal malformation. METHODS: a multicenter, prospective, randomized controlled trial (registered under chictr.org.cn Identifier no.ChiCTR-INR-17014179) was conducted between 2018 and 2019. Four centers in China analyzed 156 newborns of congenital gastrointestinal malformation undergoing intestinal anastomosis to EEN group (n = 78) or control (C) group (n = 78). The primary outcomes of this study were length of postoperative stay (LOPS) and time to full feeds. Secondary outcomes included morbidity of complications, parenteral nutrition (PN) duration, feeding intolerance, 30 day mortality rate and 30 day readmission rate. RESULTS: the mean time to full feeds and LOPS in the EEN group were 15.0 (9.8-22.8) days and 17.6 (12.0-29.8) days, while that were 18.0 (12.0-24.0) days and 20.0 (15.0-30.3) days in C groups respectively. There was no significant difference between two groups(P >0.05). No significant intergroup difference was found with respect to postoperative morbidity, PN duration or feeding intolerance(P >0.05). CONCLUSIONS: early enteral feeding following intestinal anastomosis in neonates with congenital gastrointestinal malformation is safe. Post-operative outcomes demonstrated a trend toward improvement. LEVEL OF EVIDENCE: Level Ⅰ.

Extracorporeal membrane oxygenation for congenital diaphragmatic hernia: how to begin?

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is critical for congenital diaphragmatic hernia (CDH), who fails to achieve adequate oxygenation with conventional management. We aim to evaluate initial experiences with ECMO support in a tertiary women and children's medical center in mainland China. METHODS: We retrospectively reviewed the establishment of ECMO for CDH Program in our center and analysis of five CDH neonates, who underwent repair during ECMO between December 2016 and December 2018. RESULTS: The first ECMO for CDH Program in our institution was established and managed by a multidisciplinary team since December 2016. An alert of ECMO was prenatally created for moderate-severe pulmonary hypoplasia. Of sixteen admissions prenatally diagnosed moderate-severe CDH, eight neonates (50%) required ECMO but five (31%) received eventually. Veno-arterial ECMO was established from 3 hours to 41 hours of age (median 20 h). All the five underwent CDH repair after a stabilization period on ECMO, which ranged from 12 h to 122 h (median 58 h). There were no clotting complications, related to coagulating during CDH repair. Bleeding was the most common complication. Cannula malposition was detected in one case. Their median ECMO duration was 437 (range 85-946) hours. Neonatal survival was 80% (4/5) and 3 survived (60%) in the first three months of life. CONCLUSIONS: Multidisciplinary team work, precise prenatal evaluation and skillful cannulation assist the successful beginning of ECMO for CDH. Our preliminary results would encourage other institutions, whose ECMO is not well-established.

Protocol for enhanced recovery after surgery with 3D laparoscopic excision for choledochal cysts can benefit the recovery process.

OBJECTIVE: To explore the clinical value of enhanced recovery after surgery (ERAS) with laparoscopic choledochal cyst (CDC) excision in children. METHODS: A retrospective review was performed on the clinical data from 33 in-patients whose final diagnosis was CDC. We included 18 patients who underwent the traditional treatment for CDC from April 2017 to October 2017 as the control group and 15 patients who underwent the enhanced recovery protocol (ERP) from November 2017 to May 2018 as the ERAS group. All the patients had received three-dimensional (3D) laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy by the same group of pediatric surgeons. The time of initial water intake, postoperative time to total enteral nutrition (TEN), postoperative hospital stay, total cost in hospital, postoperative complications, and readmission rate within 30 days were analysed. RESULTS: The postoperative time of initial water intake, postoperative time to TEN, postoperative hospital stay, and total cost in hospital were (21.5 ± 2.1) h, (4.3 ± 0.5) days, (5.3 ± 0.6) days, and (35,945.49 ± 6071.46) China Yuan (CNY) in the ERAS group and (44.1 ± 3.5) h, (7.7 ± 2) days, (9.1 ± 2.5) days, and (45,609.08 ± 11,439.80) CNY in the control group, respectively. These values in the ERAS group were significantly lower than those in the control group (p < 0.05). There was no significant difference between the two groups in terms of postoperative complications. No readmission patient within 30 days was encountered in either of the two groups. CONCLUSION: Enhanced recovery protocols can shorten postoperative hospital stay, relieve perioperative discomfort, lighten the financial burden, and result in substantial improvements.

Preparation and Characterization of Nano-Laponite/PLGA Composite Scaffolds for Urethra Tissue Engineering.

The purpose of this study was to construct a biomimetic urethral repair substitute. The nano-Laponite/polylactic acid-glycolic acid copolymer (PLGA) fiber scaffolds were produced to replicate the natural human urethra tissue microenvironment. PLGA (molar ratio 50:50) and Laponite were used in this study as raw materials. The nano-Laponite/PLGA scaffolds were fabricated via electrospinning technology. After preparing the material, the microstructural and mechanical properties of the nano-Laponite/PLGA scaffold were tested via scanning electron microscopy and electronic universal testing. The effects of different amounts of Laponite on the degradation of the nano-Laponite/PLGA scaffold were studied. Human umbilical vein endothelial cells (HUVECs) were co-cultured with PLGA and nano-Laponite/PLGA scaffolds for 24, 48, or 72 h. Scanning electron microscopy results illustrated that the microstructure of the scaffold fabricated by electrospinning was similar to that of the natural extracellular matrix. When the electrospinning liquid contained 10% Laponite, the nano-Laponite/PLGA stress-strain curve illustrated that the scaffold has strong elastic deformation ability. HUVECs exhibited good growth on the nano-Laponite/PLGA scaffold. When the scaffold contained 1% Laponite, the cell proliferation rate in the CCK-8 test was significantly better than that for the other three materials, displaying good cell culture characteristics. The 1% nano-Laponite/PLGA composite scaffold can be used as a suitable urethral repair material, but its performance requires further development and research.

Comparison of outcomes following three surgical techniques for patients with severe jejunoileal atresia.

BACKGROUND: Severe jejunoileal atresia is associated with prolonged parenteral nutrition, higher mortality and secondary surgery. However, the ideal surgical management of this condition remains controversial. This study aimed to compare the outcomes of patients with severe jejunoileal atresia treated by three different procedures. METHODS: From January 2007 to December 2016, 105 neonates with severe jejunoileal atresia were retrospectively reviewed. Of these, 42 patients (40.0%) underwent the Bishop-Koop procedure (BK group), 49 (46.7%) underwent primary anastomosis (PA group) and 14 (13.3%) underwent Mikulicz double-barreled ileostomy (DB group). Demographics, treatment and outcomes including mortality, morbidity and nutrition status were reviewed and were compared among the three groups. RESULTS: The total mortality rate was 6.7%, showing no statistical difference among the three groups (P = 0.164). The BK group had the lowest post-operative complication rate (33.3% vs 65.3% for the PA group and 71.4% for the DB group, P = 0.003) and re-operation rate (4.8% vs 38.8% for the PA group and 14.3% for the DB group, P < 0.001). Compared with the BK group, the PA group showed a positive correlation with the complication rate and re-operation rate, with an odds ratio of 4.15 [95% confidence interval (CI): 1.57, 10.96] and 12.78 (95% CI: 2.58, 63.29), respectively. The DB group showed a positive correlation with the complication rate when compared with the BK group, with an odds ratio of 7.73 (95% CI: 1.67, 35.72). The weight-for-age Z-score at stoma closure was -1.22 (95% CI: -1.91, -0.54) in the BK group and -2.84 (95% CI: -4.28, -1.40) in the DB group (P = 0.039). CONCLUSIONS: The Bishop-Koop procedure for severe jejunoileal atresia had a low complication rate and re-operation rate, and the nutrition status at stoma closure was superior to double-barreled enterostomy. The Bishop-Koop procedure seems to be an appropriate choice for severe jejunoileal atresia.

Middle-term bowel function and quality of life in low-type anorectal malformation.

BACKGROUND: Low-type anorectal malformations (ARMs) are considered benign; however, in China, data regarding such conditions are limited. Thus, this study aimed to assess the middle-term bowel functions and quality of life (QOL) among children with low-type ARM. METHODS: Children > 3 years of age who underwent treatment for low-type ARM (vestibular fistula [VF] and perineal fistula [PF]) during 2013 and healthy children were included. The children were interviewed during their outpatient visits. The primary outcome measures were bowel function, as assessed using the Baylor Continence Scale (BCS), and QOL, as measured using the Pediatric Quality of Life Inventory (PedsQL 4.0). RESULTS: A total of 82 patients responded; mean patient age was 6.8 (range, 3-12) years. Mean BCS score in the control group (7.94 ± 4.74) was significantly lower than that in the VF (18.69 ± 11.11, P < 0.001) and PF (15.47 ± 6.50, P < 0.001) groups. However, there were no differences in PedsQL 4.0 scores among the groups. The patients scored the lowest for emotional function and the highest for physical function. Nearly all measurements of QOL significantly decreased with increased BCS score. CONCLUSIONS: Children with low-type ARM can achieve good bowel control and QOL. However, although ARMs are benign, several children with this condition suffer from anal function problems that affect QOL. Redo operations, mislocated anus, and incorrect constipation treatment are the iatrogenic causes of fecal incontinence.

[Cannulation for extracorporeal membrane oxygenation in infants less than 5 kg].

OBJECTIVE: To summarize the experience of cannulation for extracorporeal membrane oxygenation (ECMO) in infants less than 5 kg. METHODS: Eleven infants with ECMO support who weighed less than 5 kg were admitted to critical care medicine of Guangzhou Women and Children's Medical Center from June 2016 to June 2018 were enrolled. Retrospective analysis of support type, configuration, site of cannula and complication of ECMO was performed. RESULTS: The 11 infants consisted of 9 males and 2 females. The weight on ECMO of 1.96-4.60 kg, with an average of (3.14±0.65) kg; age 0.1-30.0 days, with a median of 5.6 (1.5, 8.3) days. Four cases were given ECMO because of congenital diaphragmatic hernia with severe pulmonary hypertension and other 7 cases were cannulated due to complication of congenital cardiac surgery. All infants were received veno-arterial (V-A) ECMO. In 4 cases, the cannulas were placed in the right internal jugular vein for drainage (8-10 French catheter with 6.0-7.5 cm depth) and the right carotid artery for infusion (6-8 French catheter with 2.5-3.5 cm depth); the average time of cannulation in right carotid and jugular vessels was (73±20) minutes (range 55-100 minutes). In other 7 cases, the cannulas were inserted into the right atrium (12-14 French catheter with 2.8-3.0 cm depth) for draining blood and returning it to the ascending aorta (6-8 French catheter with 1.0-2.0 cm depth); the average time of cannulation in central vessels was (64±31) minutes (range 35-110 minutes) with exclusion of 2 cases intraoperative cannulation. There were three infants with complications. One episode was shown in vascular rupture, one in catheter site hemorrhage and one in cannula malposition with later repositioning. There was no case of insertion site infection, cannula-related bloodstream infection and accident detached cannula. CONCLUSIONS: Cannulation for ECMO can be performed in infants less than 5 kg with a high rate of success and a low rate of complication owing to appropriate catheter and skillful cannulation.

Analysis of the clinical outcomes of fetal bowel dilatation combined with other abnormal ultrasonographic features.

OBJECTIVES: To explore the significance of fetal bowel dilatation combined with other abnormal ultrasound features in the diagnosis of gastrointestinal malformation. METHODS: A retrospective study of fetuses with bowel dilatation was performed, from August 2012 to October 2015. All the cases were identified from the ultrasound database and all observations of the relationship of prenatal abnormal abdominal ultrasound features and intestinal malformation were performed through the infancy stage. RESULTS: We found 52 fetuses with prenatal suspicion of bowel dilatation. Of these, 20 cases were surgically confirmed to have intestinal malformation, 13 cases had no abnormal bowel loops after birth, 8 cases had abnormal intestinal features while no surgical intervention was performed after birth, 10 cases were lost to follow-up and 1 fetus died in utero at 34 weeks of gestation. Forty cases with full data were divided into three groups, including Group A (Small bowel dilatation combined with other features vs. Isolated small bowel dilatation), Group B (Colonic bowel dilatation combined with other features vs. Isolated colonic bowel dilatation) and Group C (Bowel dilatation combined with other features vs. Isolated bowel dilatation). The intestinal malformation occurrence rates were 73.33% vs. 31.25% in Group A, 50% vs. 25% in Group B, and 70% vs. 30% in Group C. These results suggest that malformation occurs at a lesser frequency in simple bowel dilatation versus bowel dilatation in combination with other abnormal ultrasound features (p = .026), similarly in simple small bowel dilatation versus small bowel dilatation in combination with other abnormal ultrasound features (p = .032). CONCLUSIONS: Prenatal bowel dilatation in combination with other abnormal ultrasound features, especially small bowel dilatation in combination with other abnormal ultrasound features, detected in the second and third trimesters, tended to indicate intestinal malformation, which contributes to enhance the accuracy of prenatal diagnosis of intestinal malformation.

The Role of Neonatal Gr-1+ Myeloid Cells in a Murine Model of Rhesus-Rotavirus-Induced Biliary Atresia.

Activation of innate immunity together with cholangiocyte damage occurs in biliary atresia (BA). However, detailed information on the inflammatory cells involved is lacking. This study investigates both the pathophysiology of CD11b+Gr-1+ cells in a mouse model of BA and their presence in BA patients. CD11b+Gr-1+ cells were targeted by an anti-Ly6G antibody in murine BA induced by inoculation with rhesus rotavirus. Expression of the Ly6G homolog CD177+ was examined in biopsies from BA patients. The symptoms of BA were ameliorated, and survival was prolonged in those mice receiving 5 to 10 µg of antibody per mouse every 3 days for four times compared with the mice treated with virus alone. However, the mice later developed chronic BA with persistent low body weight and jaundice. Hepatic inflammatory cells were reduced compared with acute BA. Blockade of extrahepatic bile ducts occurred, whereas intrahepatic ductules were partially preserved, and a progressive increase in liver fibrosis was observed. High levels of CD11b+Gr-1+ cells were present in these mice. The administration of an anti-Ly6G antibody again in those chronic BA mice reduced jaundice and restored body weight. In BA patients CD177+ cells were highly expressed in the liver. Our data suggest that the chronic mouse BA model shares key characteristics with clinical BA and indicates the importance of CD11b+Gr-1+ cells in the initiation and progression of BA.

Is the Bishop-Koop procedure useful in severe jejunoileal atresia?

PURPOSE: The aim of this study was to report our experience using the Bishop-Koop procedure for the treatment of various surgical problems of jejunoileal atresia including luminal discrepancy, complex meconium peritonitis, type IIIb and type IV atresia which we defined as severe jejunoileal atresia. METHODS: This retrospective study was performed on the patients with severe jejunoileal atresia who underwent Bishop-Koop procedure at a tertiary center in China over a five year of period. The mortality, complication rate, nutrition status and the risk factors for postoperative adverse outcomes were explored. RESULTS: A total of 41 neonates underwent the Bishop-Koop procedure. The median duration of the hospital stay and total parenteral nutrition and the point at which oral feeding was initiated postoperatively were 24 days (95% CI =18.99-29.01), 13 days (95% CI = 9.03-16.97) and 11 days (95% CI = 10.17-11.83) respectively. The mortality rate was 7.32% (3/41). The complication rate was 41.4% (17/41) including anastomotic leak, intestinal obstruction, high output stoma and cholestasis. The weight for age Z-score at stoma closure was restored to normal levels (-0.86, 95% confidence interval (CI) = -1.44, -0.28). The main factor associated with adverse outcomes in severe jejunoileal atresia was premature delivery (odds ratio (OR) = 4.44, 95% CI = 1.06-18.67). CONCLUSIONS: Bishop-Koop procedure appears to be a technically efficient method for severe jejunoileal atresia, although larger studies are needed to compare Bishop-Koop procedure and other operation techniques. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: Level IV.

Autoimmune liver disease-related autoantibodies in patients with biliary atresia.

AIM: To investigate the prevalence and clinical significance of autoimmune liver disease (ALD)-related autoantibodies in patients with biliary atresia (BA). METHODS: Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies (ANAs), by line-blot assay; ANA and anti-neutrophil cytoplasmic antibody (ANCA), by indirect immunofluorescence assay; specific ANCAs and anti-M2-3E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA (i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient. RESULTS: The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls (14.8% vs 2.2%, P < 0.05). Accordingly, 32 (25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3E. By comparison, the controls had a remarkably lower frequency of anti-M2-3E (P < 0.05), with 6/92 (8.6%) of patients with other liver diseases and 2/48 (4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls (3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs (ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls (37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis (r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis. CONCLUSION: High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmune-mediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.