Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and ...Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still contro-versial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.展开更多
AIM:To explore differences in biochemical indices between neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and that with other etiologies. METHODS:Patients under 6 mo of age who were referred for ...AIM:To explore differences in biochemical indices between neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and that with other etiologies. METHODS:Patients under 6 mo of age who were referred for investigation of conjugated hyperbiliru-binaemia from June 2003 to December 2010 were eligible for this study. After excluding diseases affecting the extrahepatic biliary system, all patients were screened for the two most common SLC25A13 mutations; the coding exons of the entire SLC25A13 gene was sequenced and Western blotting of citrin protein performed in selected cases. Patients in whom homo-zygous or compound heterozygous SLC25A13 mutation and/or absence of normal citrin protein was detected were defined as having NICCD. Cases in which no specific etiological factor could be ascertained after a com-prehensive conjugated hyperbilirubinaemia work-up were defined as idiopathic neonatal cholestasis (INC). Thirty-two NICCD patients, 250 INC patients, and 39 infants with cholangiography-confirmed biliary atresia (BA) were enrolled. Laboratory values at their first visit were abstracted from medical files and compared. RESULTS:Compared with BA and INC patients, the NICCD patients had significantly higher levels of total bile acid (TBA) [all measures are expressed as median (inter-quartile range):178.0 (111.2-236.4) μmol/L in NICCD vs 112.0 (84.9-153.9) μmol/L in BA and 103.0 (70.9-135.3) μmol/L in INC, P = 0.0001]. The NICCD patients had significantly lower direct bilirubin [D-Bil 59.6 (43.1-90.9) μmol/L in NICCD vs 134.0 (115.9-151.2) μmol/L in BA and 87.3 (63.0-123.6) μmol/L in INC, P = 0.0001]; alanine aminotransferase [ALT 34.0 (23.0-55.0) U/L in NICCD vs 108.0 (62.0-199.0) U/L in BA and 84.5 (46.0-166.0) U/L in INC, P = 0.0001]; aspartate aminotransferase [AST 74.0 (53.5-150.0) U/L in NICCD vs 153.0 (115.0-239.0) U/L in BA and 130.5 (81.0-223.0) U/L in INC, P = 0.0006]; albumin [34.9 (30.7-38.2) g/L in NICCD vs 38.4 (36.3-42.2) g/L in BA and 39.9 (37.0-42.3) g/L in INC, P = 0.0001]; glucose 展开更多
Background Biliary atresia (BA) is the most common cause of obstructive jaundice in infants.Although the Kasai procedure has greatly improved the progno sis,most patients still need liver transplantation (LT) for long...Background Biliary atresia (BA) is the most common cause of obstructive jaundice in infants.Although the Kasai procedure has greatly improved the progno sis,most patients still need liver transplantation (LT) for long-term survival.The pathogenesis of BA has not been fully clarified,and liver fibrosis in BA is far beyond biliary obstructive cirrhosis.Data sources Literature reviews were underwent through PubMed.Persistent inflammation,immune response,biliary epithelial-mesenchymal transition,matrix deposition,decompensated angiogenesis,and unique biliary structure development all contribute to the fibrosis process.Observed evidences in such fields have been collected and form the backbone of this review.Results Interactions of the multiple pathways accelerate this process.Conclusions Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.展开更多
It is well known that during mammalian ovarian follicular development, the majority of follicles undergo atresia at various stages of their development. However, the mechanisms controlling this selection process remai...It is well known that during mammalian ovarian follicular development, the majority of follicles undergo atresia at various stages of their development. However, the mechanisms controlling this selection process remain unknown. In this study, we investigated apoptosis in granulosa cells during goat follicular atresia by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The changes in the levels of steroids, insulin-like growth factors (IGFs) and IGF receptors were studied by radioimmunoassay (RIA) and semi-quantitative reverse transcrip-tion-PCR. We found that the percentage of apoptotic granulosa cells in the atretic (A) follicles was significantly higher than that in the slightly atretic (SA) and healthy (H) follicles. The level of estradiol and the ratio of estradiol to progesterone in H follicles were significantly higher than those in A follicles. On the other hand, the level of progesterone was not significantly different among these follicle types. We also found that the level of IGF-Ⅰ in H follicles was higher than in SA and A follicles, whereas the amount of IGF-Ⅱ did not vary significantly. The expression of IGF receptor also decreased in A follicles as compared to that in H and SA follicles. These results suggested that estradiol and IGF-Ⅰ might be involved in controlling apoptosis in granulosa cells during follicular atresia.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
In 1953, the pioneer of human orthotopic liver transplantation(LT), Thomas E Starzl, was the first to attempt an orthotopic liver transplant into a 3 years old patient suffering from biliary atresia. Thus, the first L...In 1953, the pioneer of human orthotopic liver transplantation(LT), Thomas E Starzl, was the first to attempt an orthotopic liver transplant into a 3 years old patient suffering from biliary atresia. Thus, the first LT in humans was attempted in a disease, which, up until today, remains the main indication for pediatric LT(p LT). During the last sixty years, refinements in diagnostics and surgical technique, the introduction of new immunosuppressive medications and improvements in perioperative pediatric care have established LT as routine procedure for childhood acute and chronic liver failure as well as inherited liver diseases. In contrast to adult recipients, p LT differs greatly in indications for LT, allocation practice, surgical technique, immunosuppression and postoperative life-long aftercare. Many aspects are focus of ongoing preclinical and clinical research. The present review gives an overview of current developments and the clinical outcome of p LT, with a focus on alternatives to full-size deceased-donor organ transplantation.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 different methods in differentiating idiopathic neonatal hepatitis from biliary atresia. METHODS: Sixty-five infants with cholestatic jaundice and final diagnosis of idiopathic neonatal hepatitis and ...AIM: To evaluate different methods in differentiating idiopathic neonatal hepatitis from biliary atresia. METHODS: Sixty-five infants with cholestatic jaundice and final diagnosis of idiopathic neonatal hepatitis and biliary atresia were studied prospectively from September 2003 to March 2006. A thorough history and physical examination were undertaken and the liver enzymes were examined. All cases underwent abdominal ultrasonography, hepatobiliary scintigraphy, and percutaneous liver biopsy. The accuracy, sensitivity, specificity and predictive values of these various methods were compared. RESULTS: There were 34 girls and 31 boys, among them 46 subjects had idiopathic neonatal hepatitis (age, 61 ± 17 d) and 19 had biliary atresia (age, 64 ± 18 d). The mean age at onset of jaundice was significantly lower in cases of biliary atresia when compared to idiopathic neonatal hepatitis cases (9 ± 13 d vs 20 ± 21 d; P = 0.032). The diagnostic accuracy of different methods was as follows: liver biopsy, 96.9%; clinical evaluation, 70.8%; ultrasonography, 69.2%; hepatobiliary scintigraphy, 58.5%; and liver enzymes, 50.8%.CONCLUSION: Our results indicate that clinical evaluation by an experienced pediatric hepatologist and a biopsy of the liver are considered as the most reliable methods to differentiate idiopathic neonatal hepatitis and biliary atresia.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
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 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 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
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%, r展开更多
Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic...Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscularthickening, tracheobronchial remnants(TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography(EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.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
基金Supported by Fundao de Amparo à Pesquisa do Estado de Minas Gerais, BrazilConselho Nacional de Desenvolvimento Científico e Tecnológico, Brazil+1 种基金FAPEMIG: CBB-APQ-00075-09/ CNPq 573646/2008-2Programa de Grupos de ExcelênciaFINEP, Brazil
文摘Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still contro-versial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.
基金Supported by National Science Foundation of China, No. 30973230 and No. 81070281
文摘AIM:To explore differences in biochemical indices between neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and that with other etiologies. METHODS:Patients under 6 mo of age who were referred for investigation of conjugated hyperbiliru-binaemia from June 2003 to December 2010 were eligible for this study. After excluding diseases affecting the extrahepatic biliary system, all patients were screened for the two most common SLC25A13 mutations; the coding exons of the entire SLC25A13 gene was sequenced and Western blotting of citrin protein performed in selected cases. Patients in whom homo-zygous or compound heterozygous SLC25A13 mutation and/or absence of normal citrin protein was detected were defined as having NICCD. Cases in which no specific etiological factor could be ascertained after a com-prehensive conjugated hyperbilirubinaemia work-up were defined as idiopathic neonatal cholestasis (INC). Thirty-two NICCD patients, 250 INC patients, and 39 infants with cholangiography-confirmed biliary atresia (BA) were enrolled. Laboratory values at their first visit were abstracted from medical files and compared. RESULTS:Compared with BA and INC patients, the NICCD patients had significantly higher levels of total bile acid (TBA) [all measures are expressed as median (inter-quartile range):178.0 (111.2-236.4) μmol/L in NICCD vs 112.0 (84.9-153.9) μmol/L in BA and 103.0 (70.9-135.3) μmol/L in INC, P = 0.0001]. The NICCD patients had significantly lower direct bilirubin [D-Bil 59.6 (43.1-90.9) μmol/L in NICCD vs 134.0 (115.9-151.2) μmol/L in BA and 87.3 (63.0-123.6) μmol/L in INC, P = 0.0001]; alanine aminotransferase [ALT 34.0 (23.0-55.0) U/L in NICCD vs 108.0 (62.0-199.0) U/L in BA and 84.5 (46.0-166.0) U/L in INC, P = 0.0001]; aspartate aminotransferase [AST 74.0 (53.5-150.0) U/L in NICCD vs 153.0 (115.0-239.0) U/L in BA and 130.5 (81.0-223.0) U/L in INC, P = 0.0006]; albumin [34.9 (30.7-38.2) g/L in NICCD vs 38.4 (36.3-42.2) g/L in BA and 39.9 (37.0-42.3) g/L in INC, P = 0.0001]; glucose
基金the Natural Science Foundation of China(81400576).
文摘Background Biliary atresia (BA) is the most common cause of obstructive jaundice in infants.Although the Kasai procedure has greatly improved the progno sis,most patients still need liver transplantation (LT) for long-term survival.The pathogenesis of BA has not been fully clarified,and liver fibrosis in BA is far beyond biliary obstructive cirrhosis.Data sources Literature reviews were underwent through PubMed.Persistent inflammation,immune response,biliary epithelial-mesenchymal transition,matrix deposition,decompensated angiogenesis,and unique biliary structure development all contribute to the fibrosis process.Observed evidences in such fields have been collected and form the backbone of this review.Results Interactions of the multiple pathways accelerate this process.Conclusions Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.
基金supported by the National Natural Science Foundation of China(No.30070556)the Major State Basic Research Development Program of China(No.G200016107).
文摘It is well known that during mammalian ovarian follicular development, the majority of follicles undergo atresia at various stages of their development. However, the mechanisms controlling this selection process remain unknown. In this study, we investigated apoptosis in granulosa cells during goat follicular atresia by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The changes in the levels of steroids, insulin-like growth factors (IGFs) and IGF receptors were studied by radioimmunoassay (RIA) and semi-quantitative reverse transcrip-tion-PCR. We found that the percentage of apoptotic granulosa cells in the atretic (A) follicles was significantly higher than that in the slightly atretic (SA) and healthy (H) follicles. The level of estradiol and the ratio of estradiol to progesterone in H follicles were significantly higher than those in A follicles. On the other hand, the level of progesterone was not significantly different among these follicle types. We also found that the level of IGF-Ⅰ in H follicles was higher than in SA and A follicles, whereas the amount of IGF-Ⅱ did not vary significantly. The expression of IGF receptor also decreased in A follicles as compared to that in H and SA follicles. These results suggested that estradiol and IGF-Ⅰ might be involved in controlling apoptosis in granulosa cells during follicular atresia.
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
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.
文摘In 1953, the pioneer of human orthotopic liver transplantation(LT), Thomas E Starzl, was the first to attempt an orthotopic liver transplant into a 3 years old patient suffering from biliary atresia. Thus, the first LT in humans was attempted in a disease, which, up until today, remains the main indication for pediatric LT(p LT). During the last sixty years, refinements in diagnostics and surgical technique, the introduction of new immunosuppressive medications and improvements in perioperative pediatric care have established LT as routine procedure for childhood acute and chronic liver failure as well as inherited liver diseases. In contrast to adult recipients, p LT differs greatly in indications for LT, allocation practice, surgical technique, immunosuppression and postoperative life-long aftercare. Many aspects are focus of ongoing preclinical and clinical research. The present review gives an overview of current developments and the clinical outcome of p LT, with a focus on alternatives to full-size deceased-donor organ transplantation.
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 different methods in differentiating idiopathic neonatal hepatitis from biliary atresia. METHODS: Sixty-five infants with cholestatic jaundice and final diagnosis of idiopathic neonatal hepatitis and biliary atresia were studied prospectively from September 2003 to March 2006. A thorough history and physical examination were undertaken and the liver enzymes were examined. All cases underwent abdominal ultrasonography, hepatobiliary scintigraphy, and percutaneous liver biopsy. The accuracy, sensitivity, specificity and predictive values of these various methods were compared. RESULTS: There were 34 girls and 31 boys, among them 46 subjects had idiopathic neonatal hepatitis (age, 61 ± 17 d) and 19 had biliary atresia (age, 64 ± 18 d). The mean age at onset of jaundice was significantly lower in cases of biliary atresia when compared to idiopathic neonatal hepatitis cases (9 ± 13 d vs 20 ± 21 d; P = 0.032). The diagnostic accuracy of different methods was as follows: liver biopsy, 96.9%; clinical evaluation, 70.8%; ultrasonography, 69.2%; hepatobiliary scintigraphy, 58.5%; and liver enzymes, 50.8%.CONCLUSION: Our results indicate that clinical evaluation by an experienced pediatric hepatologist and a biopsy of the liver are considered as the most reliable methods to differentiate idiopathic neonatal hepatitis and biliary atresia.
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 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 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.
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.
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%, r
文摘Congenital esophageal stenosis(CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscularthickening, tracheobronchial remnants(TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography(EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.
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.
