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
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
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 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
The different methods in differentiating biliary atresia(BA)from non-BA-related cholestasis were evaluated in order to provide a practical basis for a rapid,early and accurate differential diagnosis of the diseases.39...The different methods in differentiating biliary atresia(BA)from non-BA-related cholestasis were evaluated in order to provide a practical basis for a rapid,early and accurate differential diagnosis of the diseases.396 infants with cholestatic jaundice were studied prospectively during the period of May 2007 to June 2011.The liver function in all subjects was tested.All cases underwent abdominal ultrasonography and duodenal fluid examination.Most cases were subjected to hepatobiliary scintigraphy,magnetic resonance cholangiopancreatography(MRCP)and a percutaneous liver biopsy.The diagnosis of BA was finally made by cholangiography or histopathologic examination.The accuracy,sensitivity,specificity and predictive values of these various methods were compared.178 patients(108 males and 70 females with a mean age of 58±30 days)were diagnosed as having BA.218 patients(136 males and 82 females with a mean age of 61±24 days)were diagnosed as having non-BA etiologies of cholestasis jaundice during the follow-up period in which jaundice faded after treatment with medical therapy.For diagnosis of BA,clinical evaluation,hepatomegaly,stool color,serum gamma-glutamyltranspeptidase(GGT),duodenal juice color,bile acid in duodenal juice,ultrasonography(gallbladder),ultrasonography(griangular cord or strip-apparent hyperechoic foci),hepatobiliary scintigraphy,MRCP,liver biopsy had an accuracy of 76.0%,51.8%,84.3%,70.0%,92.4%,98.0%,90.4%,67.2%,85.3%,83.2%and 96.6%,a sensitivity of 83.1%,87.6%,96.1%,73.7%,90.4%,100%,92.7%,27.5%,100%,89.0%and 97.4%,a specificity of 70.2%,77.5%,74.8%,67.0%,94.0%,96.3%,88.5%,99.5%,73.3%,75.4%and 94.3%,a positive predictive value of 69.0%,72.6%,75.7%,64.6%,92.5%,95.7%,86.8%,98.0%,75.4%,82.6%and 98.0%,and a negative predictive value of 83.6%,8.5%,95.9%,75.7%,92.3%,100%,84.2%,93.7%,100%,84.0%and 92.6%,respectively.It was concluded that all the differential diagnosis methods are useful.The test for duodenal drainage and elements is fast and accurate.It is helpful in the differential diagnosis of BA and non-BA etiologies of cholestasis.It shows good practical value clinically.展开更多
AIM: To investigate the clinical value of ultrasono-graphic diagnosis of biliary atresia (BA), a retrospective analysis of the sonogram of 20 children with BA was undertaken. METHODS: Ultrasonography (US) was performe...AIM: To investigate the clinical value of ultrasono-graphic diagnosis of biliary atresia (BA), a retrospective analysis of the sonogram of 20 children with BA was undertaken. METHODS: Ultrasonography (US) was performed in 20 neonates and infants with BA, which was confirmed with cholangiography by operation or abdominoscopy. The presence of triangular cord, the size and echo of liver, the changes in empty stomach gallbladder and postprandial gallbladder were observed and recorded. RESULTS: The triangular cord could be observed at the porta hepatis (thickness: 0.3-0.6 cm) in 10 cases. Smaller triangular cord (0.2-0.26 cm) can be observed in 3 cases. The gallbladder was not observed in 2 cases, and 1 case showed a streak gallbladder without capsular space. The gallbladders of 15 cases were flat and small. The gallbladders of 2 cases were of normal size and appearance, however, there was no postprandial contraction. The livers of all cases showed hepatomegaly and hetreogeneous echogenicity. Statistical analysis was performed to compare the hepatomegaly and hetreogeneous echogenicity and the stage of hepatic fibrosis. CONCLUSION: The presence of the triangular cord atthe porta hepatis is specific. However, it is not the only diagnostic criterion, since flat and small gallbladder and poor contraction are also of important diagnostic and differential diagnostic significance. The degree of hepatomegaly and hetreogeneous echogenicity is proportional with liver fibrosis, and able to indicate the duration of course and prognosis.展开更多
Validation of aspartate aminotransferase to platelet ratiofor diagnosis of liver fibrosis and prediction of postoperativeprognosis in infants with biliary atresia pathological Metavir fibrosis score of the liver wedge...Validation of aspartate aminotransferase to platelet ratiofor diagnosis of liver fibrosis and prediction of postoperativeprognosis in infants with biliary atresia pathological Metavir fibrosis score of the liver wedgespecimens of 91 BA infants. The prognostic value ofpreoperative APRI for jaundice persistence, liver injury,and occurrence of cholangitis within 6 mo after KP wasstudied based on the follow-up data of 48 BA infants.RESULTS： APRI was significantly correlated withMetavir scores （rs = 0.433; P 〈 0.05）. The mean APRIvalue was 0.76 in no/mild fibrosis group （Metavir scoreF0-F1）, 1.29 in significant fibrosis group （F2-F3）, and2.51 in cirrhosis group （F4） （P 〈 0.001）. The areaunder the ROC curve （AUC） of APRI for diagnosingsignificant fibrosis and cirrhosis was 0.75 （P 〈 0.001）and 0.81 （P = 0.001）, respectively. The APRI cut-offof 0.95 was 60.6% sensitive and 76.0% specific forsignificant fibrosis diagnosis, and a threshold of 1.66was 70.6% sensitive and 82.7% specific for cirrhosis.The preoperative APRI in infants who maintainedjaundice around 6 mo after KP was higher than thatin those who did not （1.86 ± 2.13 vs 0.87 ± 0.48, P 〈0.05）. The AUC of APRI for prediction of postoperativejaundice occurrence was 0.67. A cut-off value of0.60 showed a sensitivity of 66.7% and a specificityof 83.3% for the prediction of jaundice persistence.Preoperative APRI had no significant association withlater liver injury or occurrence of cholangitis.CONCLUSION： Our study demonstrated that APRIcould diagnose significant liver fibrosis, especiallycirrhosis in BA infants, and the elevated preoperativeAPRI predicts jaundice persistence after KP.展开更多
In order to evaluate the value of magnetic resonance cholangiography （MRC） and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia （EHBA） and infantile hepatiti...In order to evaluate the value of magnetic resonance cholangiography （MRC） and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia （EHBA） and infantile hepatitis syndrome （IHS）, 52 patients with infantile cholestatic jaundice were examined by MRC and duodenal fluid examination. Original interpretations were compared with clinical outcome. Calculated sensitivity of duodenal fluid examination in diagnosis of EHBA was 100 %, and specificity was 91.1%. Sensitivity of MRC in the diagnosis of EHBA was 94.4 % and specificity 88.24 %. The sensitivity of MRC and examination of duodenal fluid combined in diagnosis of EHBA was 94.4 % and specificity 97.06 %. We are led to conclude that MRC and dynamic examination of duodenal fluid are useful in the differential diagnosis between IHS and EHBA and the combined use of the two techniques yield better resutls.展开更多
BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development...BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrho- sis. Early diagnosis of BA is difficult, and there is no speci- fic therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and thera- peutic strategies. METHODS; Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS : The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19% ), 61-90 days in 14 (45% ), 91-120 days in 8 (26% ), and >120 days in 3 ( 10% ). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32. 3% (10/31). The early operative rate in China was 12.9% (30/ 232). One child received liver transplantation with a long- term survival rate of 31.9%. CONCLUSIONS; Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA pa- tients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplanta- tion for children with advanced BA, and development of other medical strategies.展开更多
AIM: To study the relation between collagen 1, α-smooth muscle actin (α-SMA) and CD34 expression and the most essential portoenterostomy (PE) outcomes.
BACKGROUND： Portal vein thrombosis （PVT） is one of the main vascular complications after liver transplantation （LT）, especially in pediatric patients with biliary atresia （BA）. This study aimed to assess the pr...BACKGROUND： Portal vein thrombosis （PVT） is one of the main vascular complications after liver transplantation （LT）, especially in pediatric patients with biliary atresia （BA）. This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT.METHODS： One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultra- sound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT （within 1 month following LT） and those without PVT. Receiver oper- ating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. RESULTS： Of the 128 transplant recipients, 41 （32.03%） had a hypoplastic portal vein （PV）, 52 （40.63%） had hepatofugal PV flow and 40 （31.25%） had a high hepatic artery resistance index （HARI） of 〉1. Nine cases （7.03%） experienced early PVT. A PV diameter 〈4 mm （sensitivity 88.89%, specificity 72.27%）, and a hepatofugal PV flow （sensitivity 77.78%, specificity 62.18%） with a high HARI ≥1 （sensitivity 77.78%, specificity 72.27%） were hepatic hemodynamic risk factors for early PVT.（ CONCLUSIONS： Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter （≤4 mm） and hepatofugal PV flow combined with high HARI （≥1） are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.展开更多
BACKGROUND Timely differentiation of biliary atresia(BA)from other infantile cholestatic diseases can impact patient outcomes.Additionally,non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not be...BACKGROUND Timely differentiation of biliary atresia(BA)from other infantile cholestatic diseases can impact patient outcomes.Additionally,non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not been widely standardized.Shear wave elastography is an ultrasound modality that detects changes in tissue stiffness.The authors propose that the utility of elastography in BA can be elucidated through meta-analysis of existing studies.AIM To assess the utility of elastography in:(1)BA diagnosis,and(2)post-Kasai fibrosis surveillance.METHODS A literature search identified articles that evaluated elastography for BA diagnosis and for post-Kasai follow-up.Twenty studies met criteria for meta-analysis:Eleven for diagnosis and nine for follow-up post-Kasai.Estimated diagnostic odds ratio(DOR),sensitivity,and specificity of elastography were calculated through a random-effects model using Meta-DiSc software.RESULTS Mean liver stiffness in BA infants at diagnosis was significantly higher than in non-BA,with overall DOR 24.61,sensitivity 83%,and specificity 79%.Post-Kasai,mean liver stiffness was significantly higher in BA patients with varices than in patients without,with DOR 16.36,sensitivity 85%,and specificity 76%.Elastography differentiated stage F4 fibrosis from F0-F3 with DOR of 70.03,sensitivity 96%,and specificity 89%.Elastography also differentiated F3-F4 fibrosis from F0-F2 with DOR of 24.68,sensitivity 85%,and specificity 81%.CONCLUSION Elastography has potential as a non-invasive modality for BA diagnosis and surveillance post-Kasai.This paper’s limitations include inter-study method heterogeneity and small sample sizes.Future,standardized,multi-center studies are recommended.展开更多
AIM: To describe the radiological findings of a macro-regenerative nodule (MRN) in the liver of pre-transplantation biliary atresia (BA) patients and to correlate it with histological findings. METHODS: Between August...AIM: To describe the radiological findings of a macro-regenerative nodule (MRN) in the liver of pre-transplantation biliary atresia (BA) patients and to correlate it with histological findings. METHODS: Between August 1990 and November 2007, 144 BA patients underwent liver transplantation (LT) at our institution. The pre-transplantation computer tomograghy (CT) and magnetic resonance imaging (MRI) findings were reviewed and correlated with the post-transplantation pathological findings. RESULTS: Nine tumor lesions in 7 patients were diagnosed in explanted livers. The post-transplantation pathological findings showed that all the lesions were MRNs without malignant features. No small nodule was detected by either MRI or CT. Of the 8 detectable lesions, 6 (75%) were in the central part of the liver, 5 (63%) were larger than 5 cm, 5 (63%) had intra- tumor tubular structures, 3 (38%) showed enhancing fibrous septa, 3 (38%) had arterial enhancement in CT, one (13%) showed enhancement in MRI, and one (13%) had internal calcifications. CONCLUSION: Although varied in radiological appearance, MRN can be differentiated from hepatocellular carcinoma (HCC) in most of BA patients awaiting LT. The presence of an arterial-enhancingnodule does not imply that LT is withheld solely on the basis of presumed malignancy by imaging studies. Liver biopsy may be required in aid of diagnostic imaging to exclude malignancy.展开更多
AIM To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia. METHODS A retrospective study was conducted first and then a validation ...AIM To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia. METHODS A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with nonhepatic conditions. Analysis was first made by two observers separately and finally, a consensus conclusion was achieved. Then, the diagnostic value of the HSST sign was validated in an independent cohort including 45 BA and 45 non-BA patients. RESULTS In the retrospective investigation, an ampli.ed HSST sign was found in all BA patients, while we were unable to detect the HSST sign in 98.1% of the 54 non-BA patients. There was no HSST sign in any of the control subjects. In the first review, the sensitivity and specificity from one reviewer were 100% and 98.1%, respectively, and the results from the other reviewer were both 100%. The consensus sensitivity and specificity were 100% and 98.1%, respectively. The HSST sign was defined as being composed of several enlarged tortuous spider-like vascular plexuses with two to eight branches distributed on all over the liver surface, which presented as either a concentrated type or a dispersed type. In the independent validation group, the sensitivity, specificity, positive predictive value and negative predictive value of the HSST sign were 100%, 97.8%, 97.8% and 100%, respectively. CONCLUSION The HSST sign is characteristic in BA, and laparoscopic exploration for the HSST sign is valuable in the diagnosis of BA.展开更多
AIM To examine the medical status of children with biliary atresia(BA) surviving with native livers.METHODS In this cross-sectional review,data collected included complications of chronic liver disease(CLD)(cholangiti...AIM To examine the medical status of children with biliary atresia(BA) surviving with native livers.METHODS In this cross-sectional review,data collected included complications of chronic liver disease(CLD)(cholangitis in the preceding 12 mo,portal hypertension,variceal bleeding,fractures,hepatopulmonary syndrome,portopulmonary hypertension) and laboratory indices(white cell and platelet counts,total bilirubin,albumin,international normalized ratio,alanine aminotransferase,aspartate aminotransferase,γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices. RESULTS Fifty-two children [females = 32,62%; median age 7.4 years,n = 35(67%) older than 5 years] with BA(median age at surgery 60 d,range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension(40%,n = 21; 2 younger than 5 years),cholangitis(36%) and bleeding varices(25%,n = 13; 1 younger than 5 years). Fifteen(29%) had no clinical complications of CLD and three(6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient(2%). CONCLUSION Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age,underscoring the importance of medical surveillance for complications of BA starting at a young age.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
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 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
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
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.
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.
文摘The different methods in differentiating biliary atresia(BA)from non-BA-related cholestasis were evaluated in order to provide a practical basis for a rapid,early and accurate differential diagnosis of the diseases.396 infants with cholestatic jaundice were studied prospectively during the period of May 2007 to June 2011.The liver function in all subjects was tested.All cases underwent abdominal ultrasonography and duodenal fluid examination.Most cases were subjected to hepatobiliary scintigraphy,magnetic resonance cholangiopancreatography(MRCP)and a percutaneous liver biopsy.The diagnosis of BA was finally made by cholangiography or histopathologic examination.The accuracy,sensitivity,specificity and predictive values of these various methods were compared.178 patients(108 males and 70 females with a mean age of 58±30 days)were diagnosed as having BA.218 patients(136 males and 82 females with a mean age of 61±24 days)were diagnosed as having non-BA etiologies of cholestasis jaundice during the follow-up period in which jaundice faded after treatment with medical therapy.For diagnosis of BA,clinical evaluation,hepatomegaly,stool color,serum gamma-glutamyltranspeptidase(GGT),duodenal juice color,bile acid in duodenal juice,ultrasonography(gallbladder),ultrasonography(griangular cord or strip-apparent hyperechoic foci),hepatobiliary scintigraphy,MRCP,liver biopsy had an accuracy of 76.0%,51.8%,84.3%,70.0%,92.4%,98.0%,90.4%,67.2%,85.3%,83.2%and 96.6%,a sensitivity of 83.1%,87.6%,96.1%,73.7%,90.4%,100%,92.7%,27.5%,100%,89.0%and 97.4%,a specificity of 70.2%,77.5%,74.8%,67.0%,94.0%,96.3%,88.5%,99.5%,73.3%,75.4%and 94.3%,a positive predictive value of 69.0%,72.6%,75.7%,64.6%,92.5%,95.7%,86.8%,98.0%,75.4%,82.6%and 98.0%,and a negative predictive value of 83.6%,8.5%,95.9%,75.7%,92.3%,100%,84.2%,93.7%,100%,84.0%and 92.6%,respectively.It was concluded that all the differential diagnosis methods are useful.The test for duodenal drainage and elements is fast and accurate.It is helpful in the differential diagnosis of BA and non-BA etiologies of cholestasis.It shows good practical value clinically.
文摘AIM: To investigate the clinical value of ultrasono-graphic diagnosis of biliary atresia (BA), a retrospective analysis of the sonogram of 20 children with BA was undertaken. METHODS: Ultrasonography (US) was performed in 20 neonates and infants with BA, which was confirmed with cholangiography by operation or abdominoscopy. The presence of triangular cord, the size and echo of liver, the changes in empty stomach gallbladder and postprandial gallbladder were observed and recorded. RESULTS: The triangular cord could be observed at the porta hepatis (thickness: 0.3-0.6 cm) in 10 cases. Smaller triangular cord (0.2-0.26 cm) can be observed in 3 cases. The gallbladder was not observed in 2 cases, and 1 case showed a streak gallbladder without capsular space. The gallbladders of 15 cases were flat and small. The gallbladders of 2 cases were of normal size and appearance, however, there was no postprandial contraction. The livers of all cases showed hepatomegaly and hetreogeneous echogenicity. Statistical analysis was performed to compare the hepatomegaly and hetreogeneous echogenicity and the stage of hepatic fibrosis. CONCLUSION: The presence of the triangular cord atthe porta hepatis is specific. However, it is not the only diagnostic criterion, since flat and small gallbladder and poor contraction are also of important diagnostic and differential diagnostic significance. The degree of hepatomegaly and hetreogeneous echogenicity is proportional with liver fibrosis, and able to indicate the duration of course and prognosis.
基金Supported by grants from General Medical Project of Guangzhou Municipal Health Bureau,No.20141A010025Western Medicine Key Project of Guangzhou Municipal Health Bureau,No.201102A212023
文摘Validation of aspartate aminotransferase to platelet ratiofor diagnosis of liver fibrosis and prediction of postoperativeprognosis in infants with biliary atresia pathological Metavir fibrosis score of the liver wedgespecimens of 91 BA infants. The prognostic value ofpreoperative APRI for jaundice persistence, liver injury,and occurrence of cholangitis within 6 mo after KP wasstudied based on the follow-up data of 48 BA infants.RESULTS： APRI was significantly correlated withMetavir scores （rs = 0.433; P 〈 0.05）. The mean APRIvalue was 0.76 in no/mild fibrosis group （Metavir scoreF0-F1）, 1.29 in significant fibrosis group （F2-F3）, and2.51 in cirrhosis group （F4） （P 〈 0.001）. The areaunder the ROC curve （AUC） of APRI for diagnosingsignificant fibrosis and cirrhosis was 0.75 （P 〈 0.001）and 0.81 （P = 0.001）, respectively. The APRI cut-offof 0.95 was 60.6% sensitive and 76.0% specific forsignificant fibrosis diagnosis, and a threshold of 1.66was 70.6% sensitive and 82.7% specific for cirrhosis.The preoperative APRI in infants who maintainedjaundice around 6 mo after KP was higher than thatin those who did not （1.86 ± 2.13 vs 0.87 ± 0.48, P 〈0.05）. The AUC of APRI for prediction of postoperativejaundice occurrence was 0.67. A cut-off value of0.60 showed a sensitivity of 66.7% and a specificityof 83.3% for the prediction of jaundice persistence.Preoperative APRI had no significant association withlater liver injury or occurrence of cholangitis.CONCLUSION： Our study demonstrated that APRIcould diagnose significant liver fibrosis, especiallycirrhosis in BA infants, and the elevated preoperativeAPRI predicts jaundice persistence after KP.
文摘In order to evaluate the value of magnetic resonance cholangiography （MRC） and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia （EHBA） and infantile hepatitis syndrome （IHS）, 52 patients with infantile cholestatic jaundice were examined by MRC and duodenal fluid examination. Original interpretations were compared with clinical outcome. Calculated sensitivity of duodenal fluid examination in diagnosis of EHBA was 100 %, and specificity was 91.1%. Sensitivity of MRC in the diagnosis of EHBA was 94.4 % and specificity 88.24 %. The sensitivity of MRC and examination of duodenal fluid combined in diagnosis of EHBA was 94.4 % and specificity 97.06 %. We are led to conclude that MRC and dynamic examination of duodenal fluid are useful in the differential diagnosis between IHS and EHBA and the combined use of the two techniques yield better resutls.
文摘BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrho- sis. Early diagnosis of BA is difficult, and there is no speci- fic therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and thera- peutic strategies. METHODS; Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS : The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19% ), 61-90 days in 14 (45% ), 91-120 days in 8 (26% ), and >120 days in 3 ( 10% ). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32. 3% (10/31). The early operative rate in China was 12.9% (30/ 232). One child received liver transplantation with a long- term survival rate of 31.9%. CONCLUSIONS; Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA pa- tients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplanta- tion for children with advanced BA, and development of other medical strategies.
基金Supported by Sigrid Juselius Foundationthe Finnish Pediatric Research Foundation
文摘AIM: To study the relation between collagen 1, α-smooth muscle actin (α-SMA) and CD34 expression and the most essential portoenterostomy (PE) outcomes.
基金supported by a grant from the Science and Research of Shanghai Municipal Health Bureau(20134Y019)
文摘BACKGROUND： Portal vein thrombosis （PVT） is one of the main vascular complications after liver transplantation （LT）, especially in pediatric patients with biliary atresia （BA）. This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT.METHODS： One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultra- sound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT （within 1 month following LT） and those without PVT. Receiver oper- ating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. RESULTS： Of the 128 transplant recipients, 41 （32.03%） had a hypoplastic portal vein （PV）, 52 （40.63%） had hepatofugal PV flow and 40 （31.25%） had a high hepatic artery resistance index （HARI） of 〉1. Nine cases （7.03%） experienced early PVT. A PV diameter 〈4 mm （sensitivity 88.89%, specificity 72.27%）, and a hepatofugal PV flow （sensitivity 77.78%, specificity 62.18%） with a high HARI ≥1 （sensitivity 77.78%, specificity 72.27%） were hepatic hemodynamic risk factors for early PVT.（ CONCLUSIONS： Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter （≤4 mm） and hepatofugal PV flow combined with high HARI （≥1） are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.
文摘BACKGROUND Timely differentiation of biliary atresia(BA)from other infantile cholestatic diseases can impact patient outcomes.Additionally,non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not been widely standardized.Shear wave elastography is an ultrasound modality that detects changes in tissue stiffness.The authors propose that the utility of elastography in BA can be elucidated through meta-analysis of existing studies.AIM To assess the utility of elastography in:(1)BA diagnosis,and(2)post-Kasai fibrosis surveillance.METHODS A literature search identified articles that evaluated elastography for BA diagnosis and for post-Kasai follow-up.Twenty studies met criteria for meta-analysis:Eleven for diagnosis and nine for follow-up post-Kasai.Estimated diagnostic odds ratio(DOR),sensitivity,and specificity of elastography were calculated through a random-effects model using Meta-DiSc software.RESULTS Mean liver stiffness in BA infants at diagnosis was significantly higher than in non-BA,with overall DOR 24.61,sensitivity 83%,and specificity 79%.Post-Kasai,mean liver stiffness was significantly higher in BA patients with varices than in patients without,with DOR 16.36,sensitivity 85%,and specificity 76%.Elastography differentiated stage F4 fibrosis from F0-F3 with DOR of 70.03,sensitivity 96%,and specificity 89%.Elastography also differentiated F3-F4 fibrosis from F0-F2 with DOR of 24.68,sensitivity 85%,and specificity 81%.CONCLUSION Elastography has potential as a non-invasive modality for BA diagnosis and surveillance post-Kasai.This paper’s limitations include inter-study method heterogeneity and small sample sizes.Future,standardized,multi-center studies are recommended.
文摘AIM: To describe the radiological findings of a macro-regenerative nodule (MRN) in the liver of pre-transplantation biliary atresia (BA) patients and to correlate it with histological findings. METHODS: Between August 1990 and November 2007, 144 BA patients underwent liver transplantation (LT) at our institution. The pre-transplantation computer tomograghy (CT) and magnetic resonance imaging (MRI) findings were reviewed and correlated with the post-transplantation pathological findings. RESULTS: Nine tumor lesions in 7 patients were diagnosed in explanted livers. The post-transplantation pathological findings showed that all the lesions were MRNs without malignant features. No small nodule was detected by either MRI or CT. Of the 8 detectable lesions, 6 (75%) were in the central part of the liver, 5 (63%) were larger than 5 cm, 5 (63%) had intra- tumor tubular structures, 3 (38%) showed enhancing fibrous septa, 3 (38%) had arterial enhancement in CT, one (13%) showed enhancement in MRI, and one (13%) had internal calcifications. CONCLUSION: Although varied in radiological appearance, MRN can be differentiated from hepatocellular carcinoma (HCC) in most of BA patients awaiting LT. The presence of an arterial-enhancingnodule does not imply that LT is withheld solely on the basis of presumed malignancy by imaging studies. Liver biopsy may be required in aid of diagnostic imaging to exclude malignancy.
基金Supported by the Public Welfare Research Special Fund of the National Health and Family Planning of China,No.201402007
文摘AIM To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia. METHODS A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with nonhepatic conditions. Analysis was first made by two observers separately and finally, a consensus conclusion was achieved. Then, the diagnostic value of the HSST sign was validated in an independent cohort including 45 BA and 45 non-BA patients. RESULTS In the retrospective investigation, an ampli.ed HSST sign was found in all BA patients, while we were unable to detect the HSST sign in 98.1% of the 54 non-BA patients. There was no HSST sign in any of the control subjects. In the first review, the sensitivity and specificity from one reviewer were 100% and 98.1%, respectively, and the results from the other reviewer were both 100%. The consensus sensitivity and specificity were 100% and 98.1%, respectively. The HSST sign was defined as being composed of several enlarged tortuous spider-like vascular plexuses with two to eight branches distributed on all over the liver surface, which presented as either a concentrated type or a dispersed type. In the independent validation group, the sensitivity, specificity, positive predictive value and negative predictive value of the HSST sign were 100%, 97.8%, 97.8% and 100%, respectively. CONCLUSION The HSST sign is characteristic in BA, and laparoscopic exploration for the HSST sign is valuable in the diagnosis of BA.
基金Supported by University Malaya High Impact Research(UM.C/625/HIR/MOHE/CHAN/13/1)from Ministry of Higher Education,Malaysia
文摘AIM To examine the medical status of children with biliary atresia(BA) surviving with native livers.METHODS In this cross-sectional review,data collected included complications of chronic liver disease(CLD)(cholangitis in the preceding 12 mo,portal hypertension,variceal bleeding,fractures,hepatopulmonary syndrome,portopulmonary hypertension) and laboratory indices(white cell and platelet counts,total bilirubin,albumin,international normalized ratio,alanine aminotransferase,aspartate aminotransferase,γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices. RESULTS Fifty-two children [females = 32,62%; median age 7.4 years,n = 35(67%) older than 5 years] with BA(median age at surgery 60 d,range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension(40%,n = 21; 2 younger than 5 years),cholangitis(36%) and bleeding varices(25%,n = 13; 1 younger than 5 years). Fifteen(29%) had no clinical complications of CLD and three(6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient(2%). CONCLUSION Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age,underscoring the importance of medical surveillance for complications of BA starting at a young age.
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 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 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.
