BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic pati...BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.展开更多
Background Follicular atresia significantly impairs female fertility and hastens reproductive senescence.Apoptosis of granulosa cells is the primary cause of follicular atresia.Pyroptosis and necroptosis,as additional...Background Follicular atresia significantly impairs female fertility and hastens reproductive senescence.Apoptosis of granulosa cells is the primary cause of follicular atresia.Pyroptosis and necroptosis,as additional forms of pro-grammed cell death,have been reported in mammalian cells.However,the understanding of pyroptosis and necrop-tosis pathways in granulosa cells during follicular atresia remains unclear.This study explored the effects of pro-grammed cell death in granulosa cells on follicular atresia and the underlying mechanisms.Results The results revealed that granulosa cells undergo programmed cell death including apoptosis,pyroptosis,and necroptosis during follicular atresia.For the first time,we identified the formation of a PANoptosome com-plex in porcine granulosa cells.This complex was initially identified as being composed of ZBP1,RIPK3,and RIPK1,and is recruited through the RHIM domain.Additionally,we demonstrated that caspase-6 is activated and cleaved,interacting with RIPK3 as a component of the PANoptosome.Heat stress may exacerbate the activation of the PANop-tosome,leading to programmed cell death in granulosa cells.Conclusions Our data identified the formation of a PANoptosome complex that promoted programmed cell death in granulosa cells during the process of follicular atresia.These findings provide new insights into the molecular mechanisms underlying follicular atresia.展开更多
BACKGROUND Air embolism(AE)is a rare but potentially fatal complication of intestinal endoscopy(IE).CASE SUMMARY Herein,we report the case of an 18-year-old woman who underwent a successful Kasai portoenterostomy(KPE)...BACKGROUND Air embolism(AE)is a rare but potentially fatal complication of intestinal endoscopy(IE).CASE SUMMARY Herein,we report the case of an 18-year-old woman who underwent a successful Kasai portoenterostomy(KPE)for biliary atresia but died of AE during intraoperative IE for stone removal at the portoenterostomy site.Our review of the English literature identified only four similar cases of fatal AE during IE in patients undergoing KPE.The common clinical setting in the five patients,including our case,was high-pressure air insufflation into the blind closed afferent loop of the KPE to secure visibility.We hypothesize that the highly pressurized air injected into the closed loop entered the bile canaliculi—previously opened by KPE for bile drainage—passed through the tiny,microscopic pores of the fenestrated liver sinusoid endothelial cells,and finally entered the bloodstream with ease,resulting in fatal AE.CONCLUSION Meticulous performance of IE,especially on the KPE blind loop,is warranted owing to the risk of AE.展开更多
BACKGROUND Congenital intestinal atresia(CIA)is a common intestinal malformation in the neonatal period,and surgery is currently the main treatment method.The choice of postoperative feeding is crucial for the recover...BACKGROUND Congenital intestinal atresia(CIA)is a common intestinal malformation in the neonatal period,and surgery is currently the main treatment method.The choice of postoperative feeding is crucial for the recovery of gastrointestinal function in children.AIM To compare and analyze the effects of different postoperative feeding methods on gastrointestinal function reconstruction in newborns with CIA.METHODS Twenty-six children diagnosed with neonatal CIA,treated with minimally invasive surgery at Shijiazhuang Maternal and Child Health Hospital between January 2021 and May 2024,were selected for this single-center prospective randomized controlled study.They were divided into two groups using envelope randomization:Enteral nutrition(EN)group(n=13)and parenteral nutrition(PN)group(n=13).Baseline and clinical characteristics were collected,and recovery time of bowel sounds and time to first defecation were used as evaluation indices for gastrointestinal functional reconstruction.Differences between the groups were analyzed using t-test,χ2 test,and Fisher’s exact test.Spearman’s correlation tests and linear regression models were employed to analyze factors influencing time to first defecation.RESULTS The time to bowel sound recovery(51.54 vs 65.85,P=0.013)and first defecation(58.15 vs 76.62,P<0.001)was shorter in the EN group compared to the PN group.Clinical improvements in the EN group,including discharge weight(P=0.044),hospital stay(P=0.027),white blood cell count(P=0.023),albumin content(P=0.013),and direct bilirubin content(P=0.018),were also better than those in the PN group.No substantial differences in postoperative complications were found between the groups.Correlation analysis indicated that abdominal infection and operation time may relate to time to first defecation.Linear regression analysis demonstrated a considerable association between EN feeding and shorter time to first defecation.Abdominal infection and an operation time>2 hours may be risk factors for prolonged time to first defecation.CONCLUSION EN substantially promotes the recovery of gastrointestinal function after CIA in neonates and can improve clinical outcomes in children.Future research should explore optimal EN practices to enhance clinical application and child health.展开更多
Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia;but,at the present time,should not be the first therapeutic option in those cases where the surgeon can per...Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia;but,at the present time,should not be the first therapeutic option in those cases where the surgeon can perform a primary anastomosis of the two ends of the esophagus with acceptable tension.展开更多
BACKGROUND We report a low-birth-weight child(1.8 kg)with neonatal type III congenital esophageal atresia(CEA)combined with symptomatic patent ductus arteriosus(PDA).After comprehensive evaluation,esophageal anastomos...BACKGROUND We report a low-birth-weight child(1.8 kg)with neonatal type III congenital esophageal atresia(CEA)combined with symptomatic patent ductus arteriosus(PDA).After comprehensive evaluation,esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications,and arterial catheter ligation was performed at the same time.Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature.CASE SUMMARY We report a 6-day-old female child with type III CEA and PDA.The patient presented with foam at the mouth after birth,cough and shortness of breath after feeding.At another hospital,she was considered to have neonatal pneumonia,neonatal jaundice and congenital heart disease and transferred to our hospital.After iodine oil radiography of the esophagus and echocardiography we con-firmed diagnosis of CEA and PDA.The diameter of the PDA was 8 mm,with obvious left to right shunting.We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis,and ligation of PDA via left axilla straight incision after 5 d of hospitalization.The operations were successful,and the incision healed after 12 d,and the patient was discharged.We re-examined the patient 1 mo after surgery.She did not vomit when she ate rice flour.Esophageal angiography showed no stricture of the anastomotic stoma.The patient weighed 3.2 kg.CONCLUSION For CEA patients with multiple risk factors,comprehensive,timely and accurate diagnosis and evaluation,and early treatment may improve prognosis.展开更多
BACKGROUND The Kasai procedure(KPE)is an important treatment for biliary atresia(BA),the most common cause of neonatal obstructive jaundice.AIM To investigate the efficacy of robotic-assisted Kasai portoenterostomy(RA...BACKGROUND The Kasai procedure(KPE)is an important treatment for biliary atresia(BA),the most common cause of neonatal obstructive jaundice.AIM To investigate the efficacy of robotic-assisted Kasai portoenterostomy(RAKPE)in patients with BA.METHODS Clinical data of 10 patients with BA who underwent RAKPE at the Seventh Medi-cal Center of the People's Liberation Army General Hospital between December 2018 and December 2021 were retrospectively analyzed.One patient underwent Open Kasai portoenterostomy(OKPE)due to intraoperative bleeding.Consequ-ently,nine patients were included in this study.Fifty-two patients who under-went OKPE during the same period served as the control group.Preoperative and postoperative biochemical indexes,surgery-related indexes,and postoperative clearance of jaundice(CJ)were recorded and statistically analyzed.RESULTS RAKPE was successfully completed in all nine patients,with an average total operative time of 352.2 minutes(including intraoperative cholangiography).Milk feeding resumed on an average 9.89 days postoperatively,and the average time of drainage tube removal was 18.11 days.All patients were followed up for 6 mon-ths to 2 years.The liver function indicators and bilirubin levels in 8 patients returned to normal within 3 months after surgery.Three patients developed recu-rrent cholangitis after discharge,with elevated white blood cell counts,liver function indicators,and bilirubin levels,requiring hospitalization for intravenous antibiotic treatment.The duration of cholangitis ranged from 5 to 8 months post-surgery.To date,no subsequent cases of cholangitis have occurred.All patients have normal liver function and bilirubin levels,with no intrahepatic bile duct dilatation on ultrasonography.Statistical analysis comparing these indicators with those of patients who underwent OKPE showed that the RAKPE group had longer operative times and postoperative drainage tube removal durations.However,there were no significant differences in intraoperative blood loss,postoperative oral milk intake resumption,postoperative hospital stay,or CJ at 3 months post-surgery.CONCLUSION RAKPE is technically feasible,safe,and effective for treating BA.Once the technique is mastered,RAKPE may achieve CJ outcomes comparable to those of OKPE.展开更多
BACKGROUND Ileal atresia is a congenital abnormality where there is significant stenosis or complete absence of a portion of the ileum.The overall diagnostic accuracy of prenatal ultrasound in detecting jejunal and il...BACKGROUND Ileal atresia is a congenital abnormality where there is significant stenosis or complete absence of a portion of the ileum.The overall diagnostic accuracy of prenatal ultrasound in detecting jejunal and ileal atresia is low.We report a case of ileal atresia diagnosed prenatally by ultrasound examination with the“keyboard sign”and“coffee bean sign”.CASE SUMMARY We report a case of ileal atresia diagnosed in utero at 31 weeks'of gestation.Prenatal ultrasound examination revealed two rows of intestines arranged in an‘S’shape in the middle abdomen.The inner diameters were 1.7 cm and 1.6 cm,respectively.A typical“keyboard sign”was observed.The intestine canal behind the“keyboard sign”showed an irregular strong echo.There was no normal intestinal wall structure,showing a typical“coffee bean sign”.Termination of the pregnancy and autopsy findings confirmed the diagnosis.CONCLUSION The prenatal diagnosis of ileal atresia is difficult.The sonographic features of the“keyboard sign”and“coffee bean sign”are helpful in diagnosing the location of congenital jejunal and ileal atresia.展开更多
BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed first.However,due to the high failure rate of portoenterostomy,liver trans...BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed first.However,due to the high failure rate of portoenterostomy,liver transplantation has been advocated as the primary procedure for patients with BA.It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes.AIM To investigate the effect of prior portoenterostomy in infants un-dergoing liver transplantation for BA.METHODS This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil.Patients with BA were divided into two groups:Those undergoing primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy.Continuous variables were compared using the Student’s t-test or the Kruskal-Wallis test,and categorical variables were compared using theχ2 or Fisher’s exact test,as appropriate.Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis.Patient and graft survival analyses were conducted with the Kaplan–Meier product-limit estimator,and patient subgroups were compared using the two-sided log-rank test.RESULTS Forty-two patients were included in the study(25[60%]girls),23 undergoing liver transplantation without prior portoenterostomy,and 19 undergoing liver transplantation with prior portoenterostomy.Patients with prior portoenterostomy were older(12 vs 8 months;P=0.02)at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores(13.2 vs 21.4;P=0.01).The majority of the patients(35/42,83%)underwent livingdonor liver transplantation.The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis(39 vs 11%),but this result did not reach statistical significance.Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation,graft-to-recipient weight ratio,and use of vascular grafts.Finally,the groups did not significantly differ in terms of post-transplant survival.CONCLUSION In our study,prior portoenterostomy did not significantly affect the outcomes of liver transplantation.展开更多
Choanal atresia (CA) is a rare occlusion of the posterior choanae. Unilateral cases have been reported more than bilaterally, and it’s more often right-sided in those patients. According to the literature, mixed bony...Choanal atresia (CA) is a rare occlusion of the posterior choanae. Unilateral cases have been reported more than bilaterally, and it’s more often right-sided in those patients. According to the literature, mixed bony-membranous atresia is the most common type. There is a high incidence of craniofacial and visceral anomalies associated with congenital choanal atresia. Therefore, investigation for associated congenital anomalies is an important step before the surgery. We report 2 cases of incidental finding of unilateral choanal atresia in a 21- and 17-year-old with nasal discharge being the only complaint in the former and nasal obstruction with headache in the latter. The patients were then scheduled for day-surgery as a case of choanal atresia for transnasal, endoscopic repair and posterior septectomy. The patients were discharged home on the same day with the absence of restenosis or other complications.展开更多
Background:Construction of a central shunt by connection of hypoplastic true pulmonary arteries to the ascending aorta(AO)can be performed as a first step in staged repair of pulmonary atresiawith ventricular septal d...Background:Construction of a central shunt by connection of hypoplastic true pulmonary arteries to the ascending aorta(AO)can be performed as a first step in staged repair of pulmonary atresiawith ventricular septal defect and major aorto-pulmonary collateral arteries(PA-VSD-MAPCAs)intended to promote growth and development of the central pulmonary arteries.Methods:To determine early and intermediate-termgrowth of true pulmonary arteries after their connection to the AO as a first step in staged repair of PA-VSD-MAPCAs,we reviewed all angiographic studies and CT imaging of patients,treated in our tertiary referral center in the last 26 years(1991 until 2017)for PAVSD-MAPCAs with such a shunt.Results:A total of 13 patients(6 male)underwent direct end-to-side connection of the true pulmonary artery with the AO.The absolutemedian size of both PAs showed statistically significant absolute growth over time.The absolute LPA growth was estimated to be 0.013 mm/day(p<0.001).The absolute RPA growth was estimated to be 0.010 mm/day(p=0.001).In total 9/13(69%)reached total repair and 4 were considered not suitable for final repair.Conclusions:Creation of a central shunt by connection of true pulmonary arteries to the AO promotes their growth in patients with PA-VSD-MAPCAs,particular the LPA,although not reaching normal size.Patients with pulmonary atresia,and diminutive pulmonary arteries are a high-risk group,with a lower complete repair rate compared to those with better sized PAs.Alternative techniques or interventions should be explored in these patients to augment the size of the pulmonary arteries.展开更多
基金Supported by the Beijing Municipal Science and Technology Commission,No.Z191100006619002Haiyou Health High-Caliber Talent Project,No.202412the Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment,Chinese Academy of Medical Sciences,No.2021RU015.
文摘BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.
基金National Key Research and Development Program of China(2022YFD1300405)National Natural Science Foundation of China(32202671)Open project of State Key Laboratory of Animal Biotech Breeding(Grant No.2024SKLAB6-5).
文摘Background Follicular atresia significantly impairs female fertility and hastens reproductive senescence.Apoptosis of granulosa cells is the primary cause of follicular atresia.Pyroptosis and necroptosis,as additional forms of pro-grammed cell death,have been reported in mammalian cells.However,the understanding of pyroptosis and necrop-tosis pathways in granulosa cells during follicular atresia remains unclear.This study explored the effects of pro-grammed cell death in granulosa cells on follicular atresia and the underlying mechanisms.Results The results revealed that granulosa cells undergo programmed cell death including apoptosis,pyroptosis,and necroptosis during follicular atresia.For the first time,we identified the formation of a PANoptosome com-plex in porcine granulosa cells.This complex was initially identified as being composed of ZBP1,RIPK3,and RIPK1,and is recruited through the RHIM domain.Additionally,we demonstrated that caspase-6 is activated and cleaved,interacting with RIPK3 as a component of the PANoptosome.Heat stress may exacerbate the activation of the PANop-tosome,leading to programmed cell death in granulosa cells.Conclusions Our data identified the formation of a PANoptosome complex that promoted programmed cell death in granulosa cells during the process of follicular atresia.These findings provide new insights into the molecular mechanisms underlying follicular atresia.
文摘BACKGROUND Air embolism(AE)is a rare but potentially fatal complication of intestinal endoscopy(IE).CASE SUMMARY Herein,we report the case of an 18-year-old woman who underwent a successful Kasai portoenterostomy(KPE)for biliary atresia but died of AE during intraoperative IE for stone removal at the portoenterostomy site.Our review of the English literature identified only four similar cases of fatal AE during IE in patients undergoing KPE.The common clinical setting in the five patients,including our case,was high-pressure air insufflation into the blind closed afferent loop of the KPE to secure visibility.We hypothesize that the highly pressurized air injected into the closed loop entered the bile canaliculi—previously opened by KPE for bile drainage—passed through the tiny,microscopic pores of the fenestrated liver sinusoid endothelial cells,and finally entered the bloodstream with ease,resulting in fatal AE.CONCLUSION Meticulous performance of IE,especially on the KPE blind loop,is warranted owing to the risk of AE.
文摘BACKGROUND Congenital intestinal atresia(CIA)is a common intestinal malformation in the neonatal period,and surgery is currently the main treatment method.The choice of postoperative feeding is crucial for the recovery of gastrointestinal function in children.AIM To compare and analyze the effects of different postoperative feeding methods on gastrointestinal function reconstruction in newborns with CIA.METHODS Twenty-six children diagnosed with neonatal CIA,treated with minimally invasive surgery at Shijiazhuang Maternal and Child Health Hospital between January 2021 and May 2024,were selected for this single-center prospective randomized controlled study.They were divided into two groups using envelope randomization:Enteral nutrition(EN)group(n=13)and parenteral nutrition(PN)group(n=13).Baseline and clinical characteristics were collected,and recovery time of bowel sounds and time to first defecation were used as evaluation indices for gastrointestinal functional reconstruction.Differences between the groups were analyzed using t-test,χ2 test,and Fisher’s exact test.Spearman’s correlation tests and linear regression models were employed to analyze factors influencing time to first defecation.RESULTS The time to bowel sound recovery(51.54 vs 65.85,P=0.013)and first defecation(58.15 vs 76.62,P<0.001)was shorter in the EN group compared to the PN group.Clinical improvements in the EN group,including discharge weight(P=0.044),hospital stay(P=0.027),white blood cell count(P=0.023),albumin content(P=0.013),and direct bilirubin content(P=0.018),were also better than those in the PN group.No substantial differences in postoperative complications were found between the groups.Correlation analysis indicated that abdominal infection and operation time may relate to time to first defecation.Linear regression analysis demonstrated a considerable association between EN feeding and shorter time to first defecation.Abdominal infection and an operation time>2 hours may be risk factors for prolonged time to first defecation.CONCLUSION EN substantially promotes the recovery of gastrointestinal function after CIA in neonates and can improve clinical outcomes in children.Future research should explore optimal EN practices to enhance clinical application and child health.
文摘Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia;but,at the present time,should not be the first therapeutic option in those cases where the surgeon can perform a primary anastomosis of the two ends of the esophagus with acceptable tension.
基金Supported by Kunming Health Science and Technology Talent Training Project,No.2018-SW-25.
文摘BACKGROUND We report a low-birth-weight child(1.8 kg)with neonatal type III congenital esophageal atresia(CEA)combined with symptomatic patent ductus arteriosus(PDA).After comprehensive evaluation,esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications,and arterial catheter ligation was performed at the same time.Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature.CASE SUMMARY We report a 6-day-old female child with type III CEA and PDA.The patient presented with foam at the mouth after birth,cough and shortness of breath after feeding.At another hospital,she was considered to have neonatal pneumonia,neonatal jaundice and congenital heart disease and transferred to our hospital.After iodine oil radiography of the esophagus and echocardiography we con-firmed diagnosis of CEA and PDA.The diameter of the PDA was 8 mm,with obvious left to right shunting.We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis,and ligation of PDA via left axilla straight incision after 5 d of hospitalization.The operations were successful,and the incision healed after 12 d,and the patient was discharged.We re-examined the patient 1 mo after surgery.She did not vomit when she ate rice flour.Esophageal angiography showed no stricture of the anastomotic stoma.The patient weighed 3.2 kg.CONCLUSION For CEA patients with multiple risk factors,comprehensive,timely and accurate diagnosis and evaluation,and early treatment may improve prognosis.
基金Supported by Innovation and Cultivation Fund Project of the Seventh Medical Center,PLA General Hospital,No.QZX-2023-7.
文摘BACKGROUND The Kasai procedure(KPE)is an important treatment for biliary atresia(BA),the most common cause of neonatal obstructive jaundice.AIM To investigate the efficacy of robotic-assisted Kasai portoenterostomy(RAKPE)in patients with BA.METHODS Clinical data of 10 patients with BA who underwent RAKPE at the Seventh Medi-cal Center of the People's Liberation Army General Hospital between December 2018 and December 2021 were retrospectively analyzed.One patient underwent Open Kasai portoenterostomy(OKPE)due to intraoperative bleeding.Consequ-ently,nine patients were included in this study.Fifty-two patients who under-went OKPE during the same period served as the control group.Preoperative and postoperative biochemical indexes,surgery-related indexes,and postoperative clearance of jaundice(CJ)were recorded and statistically analyzed.RESULTS RAKPE was successfully completed in all nine patients,with an average total operative time of 352.2 minutes(including intraoperative cholangiography).Milk feeding resumed on an average 9.89 days postoperatively,and the average time of drainage tube removal was 18.11 days.All patients were followed up for 6 mon-ths to 2 years.The liver function indicators and bilirubin levels in 8 patients returned to normal within 3 months after surgery.Three patients developed recu-rrent cholangitis after discharge,with elevated white blood cell counts,liver function indicators,and bilirubin levels,requiring hospitalization for intravenous antibiotic treatment.The duration of cholangitis ranged from 5 to 8 months post-surgery.To date,no subsequent cases of cholangitis have occurred.All patients have normal liver function and bilirubin levels,with no intrahepatic bile duct dilatation on ultrasonography.Statistical analysis comparing these indicators with those of patients who underwent OKPE showed that the RAKPE group had longer operative times and postoperative drainage tube removal durations.However,there were no significant differences in intraoperative blood loss,postoperative oral milk intake resumption,postoperative hospital stay,or CJ at 3 months post-surgery.CONCLUSION RAKPE is technically feasible,safe,and effective for treating BA.Once the technique is mastered,RAKPE may achieve CJ outcomes comparable to those of OKPE.
文摘BACKGROUND Ileal atresia is a congenital abnormality where there is significant stenosis or complete absence of a portion of the ileum.The overall diagnostic accuracy of prenatal ultrasound in detecting jejunal and ileal atresia is low.We report a case of ileal atresia diagnosed prenatally by ultrasound examination with the“keyboard sign”and“coffee bean sign”.CASE SUMMARY We report a case of ileal atresia diagnosed in utero at 31 weeks'of gestation.Prenatal ultrasound examination revealed two rows of intestines arranged in an‘S’shape in the middle abdomen.The inner diameters were 1.7 cm and 1.6 cm,respectively.A typical“keyboard sign”was observed.The intestine canal behind the“keyboard sign”showed an irregular strong echo.There was no normal intestinal wall structure,showing a typical“coffee bean sign”.Termination of the pregnancy and autopsy findings confirmed the diagnosis.CONCLUSION The prenatal diagnosis of ileal atresia is difficult.The sonographic features of the“keyboard sign”and“coffee bean sign”are helpful in diagnosing the location of congenital jejunal and ileal atresia.
文摘BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed first.However,due to the high failure rate of portoenterostomy,liver transplantation has been advocated as the primary procedure for patients with BA.It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes.AIM To investigate the effect of prior portoenterostomy in infants un-dergoing liver transplantation for BA.METHODS This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil.Patients with BA were divided into two groups:Those undergoing primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy.Continuous variables were compared using the Student’s t-test or the Kruskal-Wallis test,and categorical variables were compared using theχ2 or Fisher’s exact test,as appropriate.Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis.Patient and graft survival analyses were conducted with the Kaplan–Meier product-limit estimator,and patient subgroups were compared using the two-sided log-rank test.RESULTS Forty-two patients were included in the study(25[60%]girls),23 undergoing liver transplantation without prior portoenterostomy,and 19 undergoing liver transplantation with prior portoenterostomy.Patients with prior portoenterostomy were older(12 vs 8 months;P=0.02)at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores(13.2 vs 21.4;P=0.01).The majority of the patients(35/42,83%)underwent livingdonor liver transplantation.The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis(39 vs 11%),but this result did not reach statistical significance.Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation,graft-to-recipient weight ratio,and use of vascular grafts.Finally,the groups did not significantly differ in terms of post-transplant survival.CONCLUSION In our study,prior portoenterostomy did not significantly affect the outcomes of liver transplantation.
文摘Choanal atresia (CA) is a rare occlusion of the posterior choanae. Unilateral cases have been reported more than bilaterally, and it’s more often right-sided in those patients. According to the literature, mixed bony-membranous atresia is the most common type. There is a high incidence of craniofacial and visceral anomalies associated with congenital choanal atresia. Therefore, investigation for associated congenital anomalies is an important step before the surgery. We report 2 cases of incidental finding of unilateral choanal atresia in a 21- and 17-year-old with nasal discharge being the only complaint in the former and nasal obstruction with headache in the latter. The patients were then scheduled for day-surgery as a case of choanal atresia for transnasal, endoscopic repair and posterior septectomy. The patients were discharged home on the same day with the absence of restenosis or other complications.
文摘Background:Construction of a central shunt by connection of hypoplastic true pulmonary arteries to the ascending aorta(AO)can be performed as a first step in staged repair of pulmonary atresiawith ventricular septal defect and major aorto-pulmonary collateral arteries(PA-VSD-MAPCAs)intended to promote growth and development of the central pulmonary arteries.Methods:To determine early and intermediate-termgrowth of true pulmonary arteries after their connection to the AO as a first step in staged repair of PA-VSD-MAPCAs,we reviewed all angiographic studies and CT imaging of patients,treated in our tertiary referral center in the last 26 years(1991 until 2017)for PAVSD-MAPCAs with such a shunt.Results:A total of 13 patients(6 male)underwent direct end-to-side connection of the true pulmonary artery with the AO.The absolutemedian size of both PAs showed statistically significant absolute growth over time.The absolute LPA growth was estimated to be 0.013 mm/day(p<0.001).The absolute RPA growth was estimated to be 0.010 mm/day(p=0.001).In total 9/13(69%)reached total repair and 4 were considered not suitable for final repair.Conclusions:Creation of a central shunt by connection of true pulmonary arteries to the AO promotes their growth in patients with PA-VSD-MAPCAs,particular the LPA,although not reaching normal size.Patients with pulmonary atresia,and diminutive pulmonary arteries are a high-risk group,with a lower complete repair rate compared to those with better sized PAs.Alternative techniques or interventions should be explored in these patients to augment the size of the pulmonary arteries.
