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 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
BACKGROUND Congenital biliary atresia(CBA)is a serious hepatobiliary disease in children with unknown etiology.Its outcome is often liver transplantation or death.Clarifying the etiology of CBA is of great significanc...BACKGROUND Congenital biliary atresia(CBA)is a serious hepatobiliary disease in children with unknown etiology.Its outcome is often liver transplantation or death.Clarifying the etiology of CBA is of great significance for prognosis,treatment,and genetic counseling.CASE SUMMARY A male Chinese infant at an age of 6 mo and 24 d was hospitalized because of"yellow skin for more than 6 mo".Soon after birth,the patient developed jaundice,which then progressively intensified.A"laparoscopic exploration"indicated"biliary atresia".After coming to our hospital,genetic testing suggested a GPC1mutation[loss 1(exons 6-7)].The patient recovered and was discharged after living donor liver transplantation.After discharge,the patient was followed up.The condition was controlled by oral drugs,and the patient’s condition was stable.CONCLUSION CBA is a complex disease with a complex etiology.Clarifying the etiology is of great clinical importance for treatment and prognosis.This case reports CBA caused by a GPC1 mutation,which enriches the genetic etiology of biliary atresia.However,its specific mechanism needs to be confirmed by further research.展开更多
Background:Pulmonary atresia(PA)is a group of heterogeneous complex congenital heart disease.Only one study modality might not get a correct diagnosis.This study aims to investigate the diagnostic power of dualsource ...Background:Pulmonary atresia(PA)is a group of heterogeneous complex congenital heart disease.Only one study modality might not get a correct diagnosis.This study aims to investigate the diagnostic power of dualsource computed tomography(DSCT)for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography(TTE).Materials and Methods:This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis.All associated malformations and clinical information,including treatments,were recorded and compared among the three groups.The diagnostic power of DSCT and TTE on all associated malformations were compared.The surgical index(McGoon ratio,pulmonary arterials index(PAI),and total neopulmonary arterial index)and radiation dose were calculated on the basis of DSCT.Results:Of the patients,32,30,and 17 were divided into the groups of PA with ventricular septal defect(VSD),PA with VSD and major aortopulmonary collateral arteries,and PA with other major malformations,respectively.Consequently,182,162,and 13 intracardiac,extracardiac,and other major malformations were diagnosed,respectively.Moreover,DSCT showed a better diagnostic performance in extracardiac deformities(154 vs.117,p<0.001),whereas TTE could diagnose intracardiac deformities better(159 vs.139,p=0.001).The McGoon ratio,PAI,and treatment methods were significantly different among the three groups(p=0.014,p=0.008,and p=0.018,respectively).Conclusion:More than one imaging modality should be used to make a correct diagnosis when clinically suspecting PA.DSCT is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.展开更多
Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospec...Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.展开更多
BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-yea...BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed.Diagnosis was made based on the patient's clinical symptoms,signs,imaging showing the fistula,X-ray and magnetic resonance imaging results.The preoperative examination was improved.Anorectoplasty was performed.The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up.CONCLUSION Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.展开更多
BACKGROUND Esophageal atresia(EA)is a life-threatening congenital malformation in newborns,and the traditional repair approaches pose technical challenges and are extremely invasive.Therefore,surgeons have been active...BACKGROUND Esophageal atresia(EA)is a life-threatening congenital malformation in newborns,and the traditional repair approaches pose technical challenges and are extremely invasive.Therefore,surgeons have been actively investigating new minimally invasive techniques to address this issue.Magnetic compression anastomosis has been reported in several studies for its potential in repairing EA.In this paper,the primary repair of EA with magnetic compression anastomosis under thoracoscopy was reported.CASE SUMMARY A full-term male weighing 3500 g was diagnosed with EA gross type C.The magnetic devices used in this procedure consisted of two magnetic rings and several catheters.Tracheoesophageal fistula ligation and two purse strings were performed.The magnetic compression anastomosis was then completed thoracoscopically.After the primary repair,no additional operation was conducted.A patent anastomosis was observed on the 15th day postoperatively,and the magnets were removed on the 23rd day.No leakage existed when the transoral feeding started.CONCLUSION Thoracoscopic magnetic compression anastomosis may be a promising minimally invasive approach for repairing EA.展开更多
BACKGROUND Esophageal replacement(ER)with gastric pull-up(GPU)or jejunal interposition(JI)used to be the standard treatment for long-gap esophageal atresia(LGEA).Changes of the ER grafts on a macro-and microscopic lev...BACKGROUND Esophageal replacement(ER)with gastric pull-up(GPU)or jejunal interposition(JI)used to be the standard treatment for long-gap esophageal atresia(LGEA).Changes of the ER grafts on a macro-and microscopic level however,are unknown.AIM To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA.METHODS A cohort study was conducted including all LGEA patients≥16 years who had undergone GPU or JI between 1985-2003 at two tertiary referral centers in the Netherlands.Patients underwent clinical assessment,contrast study and endoscopy with biopsy.Data was collected prospectively.Group differences between JI and GPU patients,and associations between different outcome measures were assessed using the Fisher’s exact test for bivariate variables and the Mann-Whitney U-test for continuous variables.Differences with a P-value<0.05 were considered statistically significant.RESULTS Nine GPU patients and eleven JI patients were included.Median age at follow-up was 21.5 years and 24.4 years,respectively.Reflux was reported in six GPU patients(67%)vs four JI patients(36%)(P=0.37).Dysphagia symptoms were reported in 64%of JI patients,compared to 22%of GPU patients(P=0.09).Contrast studies showed dilatation of the jejunal graft in six patients(55%)and graft lengthening in four of these six patients.Endoscopy revealed columnar-lined esophagus in three GPU patients(33%)and intestinal metaplasia was histologically confirmed in two patients(22%).No association was found between reflux symptoms and macroscopic anomalies or intestinal metaplasia.Three GPU patients(33%)experienced severe feeding problems vs none in the JI group.The median body mass index of JI patients was 20.9 kg/m^(2) vs 19.5 kg/m^(2) in GPU patients(P=0.08).CONCLUSION The majority of GPU patients had reflux and intestinal metaplasia in 22%.The majority of JI patients had dysphagia and a dilated graft.Follow-up after ER for LGEA is essential.展开更多
Unilateral pulmonary vein atresia(UPVA)in children is a rare disease that is characterized by a recurrent pulmonary infection and hemoptysis in childhood.This paper is a report of a case of pulmonary venous atresia tr...Unilateral pulmonary vein atresia(UPVA)in children is a rare disease that is characterized by a recurrent pulmonary infection and hemoptysis in childhood.This paper is a report of a case of pulmonary venous atresia treated by a right sub-axillary incision.Hopefully,more literature can be produced to improve the awareness and treatment level of pulmonary vein atresia.展开更多
AIM: To describe the ages at diagnosis and operation of biliary atresia(BA) and its incidence over a 15-year period in Taiwan.METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan...AIM: To describe the ages at diagnosis and operation of biliary atresia(BA) and its incidence over a 15-year period in Taiwan.METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases,Ninth Revision(ICD-9) code of BA 751.61 plus Kasai operation(ICD-9 procedure code 51.37) or liver transplantation(LT,ICD-9 procedure code 50.5). The patients' characteristics including sex,age at diagnosis,age at receiving Kasai operation and age at receiving LT were compared among three birth cohorts:(1) 1997 to 2001;(2) 2002 to 2006; and(3) 2007 to 2011.RESULTS: There were a total of 540 BA cases(275 females) with an incidence of 1.62 per 10000 live births. No seasonality of BA was noted. The mean ages at diagnosis of three cohorts were 57.9,55.6 and 52.6 d.A linear regression model demonstrated a decreasing trend of the mean age at diagnosis(1.27 d per year). The proportion of BA cases that received the Kasai operation within 60 d of age increased from 76% to 81%. A total of 189(35%) BA patients underwent LT. The mean age at LT was reduced from 3-year-old to 1-year-old. The rates of LT were 25.6% and 32.3% in patients who received the Kasai operation within 60 d or after 60 d of age,respectively. All patients who did not undergo a Kasai operation eventually required LT.CONCLUSION: The ages at diagnosis and operation in BA cases have decreased over time. Kasai operation performed at younger age reduces the need for LT. The incidence of BA in Taiwan fluctuates,but without certain trend.展开更多
AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia.METHODS: Resected small bowel specimens from affected patients (n = 7) we...AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia.METHODS: Resected small bowel specimens from affected patients (n = 7) were divided into three parts (proximal, atretic, distal). Standard histology and enzyme immunohistochemistry anti-S100, anti-protein gene product (PGP) 9.5, anti-neurofilament (NF), antic-kit-receptor (CD117) was carried out on conventional paraffin sections of the proximal and distal part. RESULTS: The neuronal and glial markers (PGP 9.5, NF, S-100) were expressed in hypertrophied ganglia and nerve fibres within the myenteric and submucosal plexuses. Furthermore, the submucous plexus contained typical giant ganglia. The innervation pattern of the proximal bowel resembled intestinal neuronal dysplasia. The density of myenteric ICCs was clearly reduced in the proximal bowel, whereas a moderate number of muscular ICCs were found. The anti-CD117 immunore- action revealed additional numerous mast cells. The distal bowel demonstrated normal morphology and density of the ENS, the ICCs and the mast cells.CONCLUSION: The proximal and distal bowel in small bowel atresia revealed clear changes in morphology and density of the ENS and ICCs.展开更多
Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the ...Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.展开更多
BACKGROUND: It is a globally challenging problem to differentially diagnose biliary atresia (BA) from other disease processes causing infantile cholestatic jaundice. The high frequency ultrasonography (HUS) yields muc...BACKGROUND: It is a globally challenging problem to differentially diagnose biliary atresia (BA) from other disease processes causing infantile cholestatic jaundice. The high frequency ultrasonography (HUS) yields much improved spatial resolution and therefore, might show better image in BA diagnostic examination. The present study was to evaluate the HUS on the diagnosis of BA in infants with jaundice. METHODS: Fifty-one infants with neonatal jaundice were scanned with ultrasonography. Images included gallbladder, bile duct, right hepatic artery (RHA), portal vein (PV) and triangular cord (TC) sign, magnetic resonance imaging and additionally laboratory tests and histopathology reports were assessed. RESULTS: Twenty-three BA and 28 non-BA cases were con firmed. The sensitivity, specificity, and accuracy of HUS were 91.3%, 92.9%, and 92.2%, respectively. All of these indices were significantly higher than those of conventional ultrasonography (P<0.01) and MR cholangiopancreatography (P<0.05). The HUS features, included a positive TC sign, an increased RHA diameter and RHA-diameter to portal-vein-diameter ratio (RHA/PV) and abnormal gallbladder, were important in the diagnosis of BA. CONCLUSION: HUS provided better imaging of BA and should be considered as a primary modality in the differential diagnosis of infantile jaundice.展开更多
AIM: To evaluate liver stiffness measurement(LSM)using non-invasive transient elastography(Fibroscan)in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia(BA).METHODS: Thirt...AIM: To evaluate liver stiffness measurement(LSM)using non-invasive transient elastography(Fibroscan)in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia(BA).METHODS: Thirty-one children with BA admitted to the Department of Pediatric Surgery of Beijing Children's Hospital from March 2012 to February 2013 were included in this study. Their preoperative LSM, liver biopsy findings, and laboratory results were studied retrospectively.RESULTS: The grade of liver fibrosis in all 31 patients was evaluated according to the METAVIR scoring system, which showed that 4 cases were in group F2, 20 in group F3 and 7 in group F4. There were 24non-cirrhosis cases(F2-F3) and 7 cirrhosis cases(F4).In groups F2, F3 and F4, the mean LSM was 9.10 ±3.30 kPa, 11.02 ± 3.31 kPa and 22.86 ± 12.43 kPa,respectively. LSM was statistically different between groups F2 and F4(P = 0.002), and between groups F3 and F4(P = 0.000), however, there was no statistical difference between groups F2 and F3(P = 0.593). The area under the receiver operating characteristic curve of LSM for ≥ F4 was 0.866. The cut-off value of LSM was 15.15 kPa for ≥ F4, with a sensitivity, specificity,positive predictive value and negative predictive value of 0.857, 0.917, 0.750 and 0.957, respectively.CONCLUSION: Fibroscan can be used as a noninvasive technique to assess liver fibrosis in children with BA. The cut-off value of LSM(15.15 kPa) can distinguish cirrhotic patients from non-cirrhotic patients.展开更多
AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA ...AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.展开更多
AIM: To analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule(CD56) in patients with biliary atresia(BA).METHODS: Established clinical laboratory markers of hepatic funct...AIM: To analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule(CD56) in patients with biliary atresia(BA).METHODS: Established clinical laboratory markers of hepatic function, including enzyme activity, protein synthesis, and bilirubin metabolism, were evaluated in patients with BA and compared with those in patients with choledochal cysts and neonatal hepatitis. Pathological changes in tissue morphology and fibrosis were examined by histological and tissue collagen staining. Immunohistochemical staining for the biliary epithelial cell markers CD56 and CK19 together with the Notch signaling related molecules Notch1 and Notch2 was performed in the context of alterations in the structure of intrahepatic biliary ducts.RESULTS: Differences in some clinical laboratoryparameters among the three diseases examined were observed, but they did not correlate with the pathological classification of fibrosis in BA. Immunohistochemical staining showed the presence of CD56-positive immature bile ducts in most patients(74.5%) with BA but not in patients with choledochal cysts or neonatal hepatitis. The number of CD56-expressing cells correlated with disease severity, with more positive cells present in the later stages of liver damage(81.8% vs 18.2%). Furthermore, bile plugs were mainly found in CD56-positive immature biliary ducts. Notch signaling was a key regulatory pathway in biliary duct formation and played a role in tissue fibrosis. Notch1 was co-expressed in CD56-positive cells, whereas Notch2 was found exclusively in blood vessels in the portal area of patients with BA. CONCLUSION: The maturation of biliary epithelial cells and the expression of Notch may play a role in the pathogenesis of BA.展开更多
AIM: To summarize our single-center experience with liver transplantation(LT) for biliary atresia(BA).METHODS: From October 2006 to December 2012, 188 children with BA were analyzed retrospectively. The stage?Ⅰ?group...AIM: To summarize our single-center experience with liver transplantation(LT) for biliary atresia(BA).METHODS: From October 2006 to December 2012, 188 children with BA were analyzed retrospectively. The stage?Ⅰ?group(from October 2006 to December 2010) comprised the first 74 patients, and the stage Ⅱ group(from January 2011 to December 2012) comprised the remaining 114 patients. Finally, 123 liver transplants were performed in 122(64.9%) patients, whereas 66 patients did not undergo LT due to denial by their parents or lack of suitable liver grafts. The selection of graft types depended on the patients' clinical status and whether a suitable living donor was available. The characteristics of patients in stages?Ⅰ?and Ⅱ were described, and the surgical outcomes of LT recipients were compared between the two stages. The KaplanMeier method was used to estimate the cumulative patient and graft survival rates, and the equality of survival distributions was evaluated using the log-rank test.RESULTS: The 188 children consisted of 102 boysand 86 girls. Their ages ranged from 3 to 144 mo with a median of 8 mo. One hundred and fifteen(61.2%) patients were born in rural areas. Comparing stage?Ⅰ?and stage Ⅱ patients, the proportion of patients referred by pediatricians(43.2% vs 71.1%, respectively; P < 0.001) and the proportion of patients who previously received a Kasai procedure(KP)(32.4% vs 44.7%, respectively; P = 0.092) obviously increased, and significantly more parents were willing to treat their children with LT(73% vs 86%, respectively; P = 0.027). Grafts from living donors(102/122, 83.6%) were the most commonly used graft type. Surgical complications(16/25, 64.0%) were the main reason for posttransplant mortality. Among the living donor liver transplantation recipients(n = 102), the incidence of surgical complications was significantly reduced(34.1% vs 15.5%, respectively; P = 0.029) and survival rates of patients and grafts were greatly improved(81.8% vs 89.7%, respectively, at 1 year; 75.0% vs 87.8%, respectively, at 3 years; P = 0.107) from stage?Ⅰ?to stage Ⅱ.CONCLUSION: The status of surgical treatments for BA has been changing in China's Mainland. Favorable midterm outcomes after LT were achieved as centers gained greater technical experience.展开更多
BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death...BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.展开更多
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 contr...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 antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, 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-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(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 autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.展开更多
BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT)...BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT) in comparison with the traditional first-line treatment,the Kasai procedure.METHODS:We assessed 28 children with BA at age of less than two years(3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011.Eighteen children who had had primary LDLT were included in a primary LDLT group,and ten children who had undergone the Kasai operation in a pre-Kasai group.All patients were followed up after discharge from the hospital.The records of the BA patients and donors were reviewed.RESULTS:The time of follow-up ranged 12-44.5 months with a median of 31 months.The 30-day and 1-year survival rates were 85.7% and 78.6%,respectively.There was no significant difference in the 30-day or 1-year survival between the two groups(83.3% vs 90% and 77.8% vs 80%,P>0.05).The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group(8,80%) than in the primary LDLT group(9,50%)(P=0.226).But no significant differences were observed in operating time(9.3 vs 8.9 hours,P=0.77),intraoperative blood loss(208.6 vs 197.0 mL,P=0.84) and blood transfusion(105.6 vs 100.0 mL,P=0.91) between the two groups.The durations of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours(P=0.18) and 27 vs 29 days(P=0.29),respectively.CONCLUSIONS:Primary LDLT is a safe and efficient management for young pediatric patients with BA.Compared with the outcome of LDLT for patients receiving a previous Kasai operation,a similar survival rate and a low rate of re-operation and intensive medical care for patients with BA can be obtained.展开更多
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.
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BACKGROUND Congenital biliary atresia(CBA)is a serious hepatobiliary disease in children with unknown etiology.Its outcome is often liver transplantation or death.Clarifying the etiology of CBA is of great significance for prognosis,treatment,and genetic counseling.CASE SUMMARY A male Chinese infant at an age of 6 mo and 24 d was hospitalized because of"yellow skin for more than 6 mo".Soon after birth,the patient developed jaundice,which then progressively intensified.A"laparoscopic exploration"indicated"biliary atresia".After coming to our hospital,genetic testing suggested a GPC1mutation[loss 1(exons 6-7)].The patient recovered and was discharged after living donor liver transplantation.After discharge,the patient was followed up.The condition was controlled by oral drugs,and the patient’s condition was stable.CONCLUSION CBA is a complex disease with a complex etiology.Clarifying the etiology is of great clinical importance for treatment and prognosis.This case reports CBA caused by a GPC1 mutation,which enriches the genetic etiology of biliary atresia.However,its specific mechanism needs to be confirmed by further research.
基金This study was approved by the Institutional Ethics Committee of West China Hospital,Sichuan University (Chengdu, Sichuan, ChinaNo. 14-163)+1 种基金This study has received funding by Sichuan Science and Technology Program(2020YJ0229)1⋅3⋅5 Project for Disciplines of Excellence,West China Hospital,Sichuan University(ZYGD18013)。
文摘Background:Pulmonary atresia(PA)is a group of heterogeneous complex congenital heart disease.Only one study modality might not get a correct diagnosis.This study aims to investigate the diagnostic power of dualsource computed tomography(DSCT)for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography(TTE).Materials and Methods:This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis.All associated malformations and clinical information,including treatments,were recorded and compared among the three groups.The diagnostic power of DSCT and TTE on all associated malformations were compared.The surgical index(McGoon ratio,pulmonary arterials index(PAI),and total neopulmonary arterial index)and radiation dose were calculated on the basis of DSCT.Results:Of the patients,32,30,and 17 were divided into the groups of PA with ventricular septal defect(VSD),PA with VSD and major aortopulmonary collateral arteries,and PA with other major malformations,respectively.Consequently,182,162,and 13 intracardiac,extracardiac,and other major malformations were diagnosed,respectively.Moreover,DSCT showed a better diagnostic performance in extracardiac deformities(154 vs.117,p<0.001),whereas TTE could diagnose intracardiac deformities better(159 vs.139,p=0.001).The McGoon ratio,PAI,and treatment methods were significantly different among the three groups(p=0.014,p=0.008,and p=0.018,respectively).Conclusion:More than one imaging modality should be used to make a correct diagnosis when clinically suspecting PA.DSCT is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.
文摘Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.
文摘BACKGROUND Female anorectal malformation is a correctable congenital defect.Delayed manifestations in patients with anal deformities are uncommon,especially after adolescence.CASE SUMMARY The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed.Diagnosis was made based on the patient's clinical symptoms,signs,imaging showing the fistula,X-ray and magnetic resonance imaging results.The preoperative examination was improved.Anorectoplasty was performed.The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up.CONCLUSION Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.
文摘BACKGROUND Esophageal atresia(EA)is a life-threatening congenital malformation in newborns,and the traditional repair approaches pose technical challenges and are extremely invasive.Therefore,surgeons have been actively investigating new minimally invasive techniques to address this issue.Magnetic compression anastomosis has been reported in several studies for its potential in repairing EA.In this paper,the primary repair of EA with magnetic compression anastomosis under thoracoscopy was reported.CASE SUMMARY A full-term male weighing 3500 g was diagnosed with EA gross type C.The magnetic devices used in this procedure consisted of two magnetic rings and several catheters.Tracheoesophageal fistula ligation and two purse strings were performed.The magnetic compression anastomosis was then completed thoracoscopically.After the primary repair,no additional operation was conducted.A patent anastomosis was observed on the 15th day postoperatively,and the magnets were removed on the 23rd day.No leakage existed when the transoral feeding started.CONCLUSION Thoracoscopic magnetic compression anastomosis may be a promising minimally invasive approach for repairing EA.
文摘BACKGROUND Esophageal replacement(ER)with gastric pull-up(GPU)or jejunal interposition(JI)used to be the standard treatment for long-gap esophageal atresia(LGEA).Changes of the ER grafts on a macro-and microscopic level however,are unknown.AIM To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA.METHODS A cohort study was conducted including all LGEA patients≥16 years who had undergone GPU or JI between 1985-2003 at two tertiary referral centers in the Netherlands.Patients underwent clinical assessment,contrast study and endoscopy with biopsy.Data was collected prospectively.Group differences between JI and GPU patients,and associations between different outcome measures were assessed using the Fisher’s exact test for bivariate variables and the Mann-Whitney U-test for continuous variables.Differences with a P-value<0.05 were considered statistically significant.RESULTS Nine GPU patients and eleven JI patients were included.Median age at follow-up was 21.5 years and 24.4 years,respectively.Reflux was reported in six GPU patients(67%)vs four JI patients(36%)(P=0.37).Dysphagia symptoms were reported in 64%of JI patients,compared to 22%of GPU patients(P=0.09).Contrast studies showed dilatation of the jejunal graft in six patients(55%)and graft lengthening in four of these six patients.Endoscopy revealed columnar-lined esophagus in three GPU patients(33%)and intestinal metaplasia was histologically confirmed in two patients(22%).No association was found between reflux symptoms and macroscopic anomalies or intestinal metaplasia.Three GPU patients(33%)experienced severe feeding problems vs none in the JI group.The median body mass index of JI patients was 20.9 kg/m^(2) vs 19.5 kg/m^(2) in GPU patients(P=0.08).CONCLUSION The majority of GPU patients had reflux and intestinal metaplasia in 22%.The majority of JI patients had dysphagia and a dilated graft.Follow-up after ER for LGEA is essential.
文摘Unilateral pulmonary vein atresia(UPVA)in children is a rare disease that is characterized by a recurrent pulmonary infection and hemoptysis in childhood.This paper is a report of a case of pulmonary venous atresia treated by a right sub-axillary incision.Hopefully,more literature can be produced to improve the awareness and treatment level of pulmonary vein atresia.
基金Supported by The Ditmanson Medical Foundation Chia-Yi Christian Hospital Research ProgramNo.R102-11
文摘AIM: To describe the ages at diagnosis and operation of biliary atresia(BA) and its incidence over a 15-year period in Taiwan.METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases,Ninth Revision(ICD-9) code of BA 751.61 plus Kasai operation(ICD-9 procedure code 51.37) or liver transplantation(LT,ICD-9 procedure code 50.5). The patients' characteristics including sex,age at diagnosis,age at receiving Kasai operation and age at receiving LT were compared among three birth cohorts:(1) 1997 to 2001;(2) 2002 to 2006; and(3) 2007 to 2011.RESULTS: There were a total of 540 BA cases(275 females) with an incidence of 1.62 per 10000 live births. No seasonality of BA was noted. The mean ages at diagnosis of three cohorts were 57.9,55.6 and 52.6 d.A linear regression model demonstrated a decreasing trend of the mean age at diagnosis(1.27 d per year). The proportion of BA cases that received the Kasai operation within 60 d of age increased from 76% to 81%. A total of 189(35%) BA patients underwent LT. The mean age at LT was reduced from 3-year-old to 1-year-old. The rates of LT were 25.6% and 32.3% in patients who received the Kasai operation within 60 d or after 60 d of age,respectively. All patients who did not undergo a Kasai operation eventually required LT.CONCLUSION: The ages at diagnosis and operation in BA cases have decreased over time. Kasai operation performed at younger age reduces the need for LT. The incidence of BA in Taiwan fluctuates,but without certain trend.
文摘AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia.METHODS: Resected small bowel specimens from affected patients (n = 7) were divided into three parts (proximal, atretic, distal). Standard histology and enzyme immunohistochemistry anti-S100, anti-protein gene product (PGP) 9.5, anti-neurofilament (NF), antic-kit-receptor (CD117) was carried out on conventional paraffin sections of the proximal and distal part. RESULTS: The neuronal and glial markers (PGP 9.5, NF, S-100) were expressed in hypertrophied ganglia and nerve fibres within the myenteric and submucosal plexuses. Furthermore, the submucous plexus contained typical giant ganglia. The innervation pattern of the proximal bowel resembled intestinal neuronal dysplasia. The density of myenteric ICCs was clearly reduced in the proximal bowel, whereas a moderate number of muscular ICCs were found. The anti-CD117 immunore- action revealed additional numerous mast cells. The distal bowel demonstrated normal morphology and density of the ENS, the ICCs and the mast cells.CONCLUSION: The proximal and distal bowel in small bowel atresia revealed clear changes in morphology and density of the ENS and ICCs.
文摘Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.
基金supported by agrant from the New Technology and Service Project of Tongji Hospital(2008057)
文摘BACKGROUND: It is a globally challenging problem to differentially diagnose biliary atresia (BA) from other disease processes causing infantile cholestatic jaundice. The high frequency ultrasonography (HUS) yields much improved spatial resolution and therefore, might show better image in BA diagnostic examination. The present study was to evaluate the HUS on the diagnosis of BA in infants with jaundice. METHODS: Fifty-one infants with neonatal jaundice were scanned with ultrasonography. Images included gallbladder, bile duct, right hepatic artery (RHA), portal vein (PV) and triangular cord (TC) sign, magnetic resonance imaging and additionally laboratory tests and histopathology reports were assessed. RESULTS: Twenty-three BA and 28 non-BA cases were con firmed. The sensitivity, specificity, and accuracy of HUS were 91.3%, 92.9%, and 92.2%, respectively. All of these indices were significantly higher than those of conventional ultrasonography (P<0.01) and MR cholangiopancreatography (P<0.05). The HUS features, included a positive TC sign, an increased RHA diameter and RHA-diameter to portal-vein-diameter ratio (RHA/PV) and abnormal gallbladder, were important in the diagnosis of BA. CONCLUSION: HUS provided better imaging of BA and should be considered as a primary modality in the differential diagnosis of infantile jaundice.
文摘AIM: To evaluate liver stiffness measurement(LSM)using non-invasive transient elastography(Fibroscan)in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia(BA).METHODS: Thirty-one children with BA admitted to the Department of Pediatric Surgery of Beijing Children's Hospital from March 2012 to February 2013 were included in this study. Their preoperative LSM, liver biopsy findings, and laboratory results were studied retrospectively.RESULTS: The grade of liver fibrosis in all 31 patients was evaluated according to the METAVIR scoring system, which showed that 4 cases were in group F2, 20 in group F3 and 7 in group F4. There were 24non-cirrhosis cases(F2-F3) and 7 cirrhosis cases(F4).In groups F2, F3 and F4, the mean LSM was 9.10 ±3.30 kPa, 11.02 ± 3.31 kPa and 22.86 ± 12.43 kPa,respectively. LSM was statistically different between groups F2 and F4(P = 0.002), and between groups F3 and F4(P = 0.000), however, there was no statistical difference between groups F2 and F3(P = 0.593). The area under the receiver operating characteristic curve of LSM for ≥ F4 was 0.866. The cut-off value of LSM was 15.15 kPa for ≥ F4, with a sensitivity, specificity,positive predictive value and negative predictive value of 0.857, 0.917, 0.750 and 0.957, respectively.CONCLUSION: Fibroscan can be used as a noninvasive technique to assess liver fibrosis in children with BA. The cut-off value of LSM(15.15 kPa) can distinguish cirrhotic patients from non-cirrhotic patients.
基金Supported by Ratchadapiseksompotch Fund, Faculty of Medicine, Chulalongkorn University, Thailand Research Fund, and the Commission on Higher Education
文摘AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.
基金Supported by The grant of State Clinical Key Specialty Construction Project(Pediatric Surgery)2013No.GJLCZD1301+1 种基金Guangdong Provincial Science and Technology Plan Projects 2014No.2014A020212373
文摘AIM: To analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule(CD56) in patients with biliary atresia(BA).METHODS: Established clinical laboratory markers of hepatic function, including enzyme activity, protein synthesis, and bilirubin metabolism, were evaluated in patients with BA and compared with those in patients with choledochal cysts and neonatal hepatitis. Pathological changes in tissue morphology and fibrosis were examined by histological and tissue collagen staining. Immunohistochemical staining for the biliary epithelial cell markers CD56 and CK19 together with the Notch signaling related molecules Notch1 and Notch2 was performed in the context of alterations in the structure of intrahepatic biliary ducts.RESULTS: Differences in some clinical laboratoryparameters among the three diseases examined were observed, but they did not correlate with the pathological classification of fibrosis in BA. Immunohistochemical staining showed the presence of CD56-positive immature bile ducts in most patients(74.5%) with BA but not in patients with choledochal cysts or neonatal hepatitis. The number of CD56-expressing cells correlated with disease severity, with more positive cells present in the later stages of liver damage(81.8% vs 18.2%). Furthermore, bile plugs were mainly found in CD56-positive immature biliary ducts. Notch signaling was a key regulatory pathway in biliary duct formation and played a role in tissue fibrosis. Notch1 was co-expressed in CD56-positive cells, whereas Notch2 was found exclusively in blood vessels in the portal area of patients with BA. CONCLUSION: The maturation of biliary epithelial cells and the expression of Notch may play a role in the pathogenesis of BA.
基金Supported by Key Joint Research Program of Shanghai Health Bureau,No.2013ZYJB0001a subtopic of Scientific and Technological Innovation and Action Project of Shanghai Science and Technology Commission,No.14411950404
文摘AIM: To summarize our single-center experience with liver transplantation(LT) for biliary atresia(BA).METHODS: From October 2006 to December 2012, 188 children with BA were analyzed retrospectively. The stage?Ⅰ?group(from October 2006 to December 2010) comprised the first 74 patients, and the stage Ⅱ group(from January 2011 to December 2012) comprised the remaining 114 patients. Finally, 123 liver transplants were performed in 122(64.9%) patients, whereas 66 patients did not undergo LT due to denial by their parents or lack of suitable liver grafts. The selection of graft types depended on the patients' clinical status and whether a suitable living donor was available. The characteristics of patients in stages?Ⅰ?and Ⅱ were described, and the surgical outcomes of LT recipients were compared between the two stages. The KaplanMeier method was used to estimate the cumulative patient and graft survival rates, and the equality of survival distributions was evaluated using the log-rank test.RESULTS: The 188 children consisted of 102 boysand 86 girls. Their ages ranged from 3 to 144 mo with a median of 8 mo. One hundred and fifteen(61.2%) patients were born in rural areas. Comparing stage?Ⅰ?and stage Ⅱ patients, the proportion of patients referred by pediatricians(43.2% vs 71.1%, respectively; P < 0.001) and the proportion of patients who previously received a Kasai procedure(KP)(32.4% vs 44.7%, respectively; P = 0.092) obviously increased, and significantly more parents were willing to treat their children with LT(73% vs 86%, respectively; P = 0.027). Grafts from living donors(102/122, 83.6%) were the most commonly used graft type. Surgical complications(16/25, 64.0%) were the main reason for posttransplant mortality. Among the living donor liver transplantation recipients(n = 102), the incidence of surgical complications was significantly reduced(34.1% vs 15.5%, respectively; P = 0.029) and survival rates of patients and grafts were greatly improved(81.8% vs 89.7%, respectively, at 1 year; 75.0% vs 87.8%, respectively, at 3 years; P = 0.107) from stage?Ⅰ?to stage Ⅱ.CONCLUSION: The status of surgical treatments for BA has been changing in China's Mainland. Favorable midterm outcomes after LT were achieved as centers gained greater technical experience.
基金supported by a grant from the Tianjin Bureau of Public Health Project (11KG103)
文摘BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.
基金the Guangdong Provincial Science and Technology Planning Project,No.2014A020212520the Guangzhou Science and Technology Project,No.201707010014
文摘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 antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, 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-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(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 autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.
基金supported by a grant from the National Science and Technology Major Project of China (2008ZX10002-025& 2008ZX10002-026)
文摘BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT) in comparison with the traditional first-line treatment,the Kasai procedure.METHODS:We assessed 28 children with BA at age of less than two years(3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011.Eighteen children who had had primary LDLT were included in a primary LDLT group,and ten children who had undergone the Kasai operation in a pre-Kasai group.All patients were followed up after discharge from the hospital.The records of the BA patients and donors were reviewed.RESULTS:The time of follow-up ranged 12-44.5 months with a median of 31 months.The 30-day and 1-year survival rates were 85.7% and 78.6%,respectively.There was no significant difference in the 30-day or 1-year survival between the two groups(83.3% vs 90% and 77.8% vs 80%,P>0.05).The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group(8,80%) than in the primary LDLT group(9,50%)(P=0.226).But no significant differences were observed in operating time(9.3 vs 8.9 hours,P=0.77),intraoperative blood loss(208.6 vs 197.0 mL,P=0.84) and blood transfusion(105.6 vs 100.0 mL,P=0.91) between the two groups.The durations of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours(P=0.18) and 27 vs 29 days(P=0.29),respectively.CONCLUSIONS:Primary LDLT is a safe and efficient management for young pediatric patients with BA.Compared with the outcome of LDLT for patients receiving a previous Kasai operation,a similar survival rate and a low rate of re-operation and intensive medical care for patients with BA can be obtained.
