BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic pati...BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.展开更多
Background Follicular atresia significantly impairs female fertility and hastens reproductive senescence.Apoptosis of granulosa cells is the primary cause of follicular atresia.Pyroptosis and necroptosis,as additional...Background Follicular atresia significantly impairs female fertility and hastens reproductive senescence.Apoptosis of granulosa cells is the primary cause of follicular atresia.Pyroptosis and necroptosis,as additional forms of pro-grammed cell death,have been reported in mammalian cells.However,the understanding of pyroptosis and necrop-tosis pathways in granulosa cells during follicular atresia remains unclear.This study explored the effects of pro-grammed cell death in granulosa cells on follicular atresia and the underlying mechanisms.Results The results revealed that granulosa cells undergo programmed cell death including apoptosis,pyroptosis,and necroptosis during follicular atresia.For the first time,we identified the formation of a PANoptosome com-plex in porcine granulosa cells.This complex was initially identified as being composed of ZBP1,RIPK3,and RIPK1,and is recruited through the RHIM domain.Additionally,we demonstrated that caspase-6 is activated and cleaved,interacting with RIPK3 as a component of the PANoptosome.Heat stress may exacerbate the activation of the PANop-tosome,leading to programmed cell death in granulosa cells.Conclusions Our data identified the formation of a PANoptosome complex that promoted programmed cell death in granulosa cells during the process of follicular atresia.These findings provide new insights into the molecular mechanisms underlying follicular atresia.展开更多
Programmed cell death(PCD),including autophagy,apoptosis,and ferroptosis,is a fundamental biological process that plays a critical role in follicular development and atresia in livestock.In ovaries,the vast majority o...Programmed cell death(PCD),including autophagy,apoptosis,and ferroptosis,is a fundamental biological process that plays a critical role in follicular development and atresia in livestock.In ovaries,the vast majority of follicles undergo atresia,while only a small fraction reach ovulation.Emerging evidence suggests that these three forms of PCD are intricately involved in regulating follicular fate through distinct yet interconnected molecular mechanisms.This review summarizes recent advances in understanding the roles of autophagy,apoptosis,and ferroptosis in follicular development and atresia,with a focus on their molecular mechanisms and interactions.By elucidating the complex regulatory networks of PCD in ovarian physiology,this review aims to provide new insights into improving reproductive efficiency in livestock through targeted modulation of these pathways.展开更多
BACKGROUND Air embolism(AE)is a rare but potentially fatal complication of intestinal endoscopy(IE).CASE SUMMARY Herein,we report the case of an 18-year-old woman who underwent a successful Kasai portoenterostomy(KPE)...BACKGROUND Air embolism(AE)is a rare but potentially fatal complication of intestinal endoscopy(IE).CASE SUMMARY Herein,we report the case of an 18-year-old woman who underwent a successful Kasai portoenterostomy(KPE)for biliary atresia but died of AE during intraoperative IE for stone removal at the portoenterostomy site.Our review of the English literature identified only four similar cases of fatal AE during IE in patients undergoing KPE.The common clinical setting in the five patients,including our case,was high-pressure air insufflation into the blind closed afferent loop of the KPE to secure visibility.We hypothesize that the highly pressurized air injected into the closed loop entered the bile canaliculi—previously opened by KPE for bile drainage—passed through the tiny,microscopic pores of the fenestrated liver sinusoid endothelial cells,and finally entered the bloodstream with ease,resulting in fatal AE.CONCLUSION Meticulous performance of IE,especially on the KPE blind loop,is warranted owing to the risk of AE.展开更多
BACKGROUND Congenital intestinal atresia(CIA)is a common intestinal malformation in the neonatal period,and surgery is currently the main treatment method.The choice of postoperative feeding is crucial for the recover...BACKGROUND Congenital intestinal atresia(CIA)is a common intestinal malformation in the neonatal period,and surgery is currently the main treatment method.The choice of postoperative feeding is crucial for the recovery of gastrointestinal function in children.AIM To compare and analyze the effects of different postoperative feeding methods on gastrointestinal function reconstruction in newborns with CIA.METHODS Twenty-six children diagnosed with neonatal CIA,treated with minimally invasive surgery at Shijiazhuang Maternal and Child Health Hospital between January 2021 and May 2024,were selected for this single-center prospective randomized controlled study.They were divided into two groups using envelope randomization:Enteral nutrition(EN)group(n=13)and parenteral nutrition(PN)group(n=13).Baseline and clinical characteristics were collected,and recovery time of bowel sounds and time to first defecation were used as evaluation indices for gastrointestinal functional reconstruction.Differences between the groups were analyzed using t-test,χ2 test,and Fisher’s exact test.Spearman’s correlation tests and linear regression models were employed to analyze factors influencing time to first defecation.RESULTS The time to bowel sound recovery(51.54 vs 65.85,P=0.013)and first defecation(58.15 vs 76.62,P<0.001)was shorter in the EN group compared to the PN group.Clinical improvements in the EN group,including discharge weight(P=0.044),hospital stay(P=0.027),white blood cell count(P=0.023),albumin content(P=0.013),and direct bilirubin content(P=0.018),were also better than those in the PN group.No substantial differences in postoperative complications were found between the groups.Correlation analysis indicated that abdominal infection and operation time may relate to time to first defecation.Linear regression analysis demonstrated a considerable association between EN feeding and shorter time to first defecation.Abdominal infection and an operation time>2 hours may be risk factors for prolonged time to first defecation.CONCLUSION EN substantially promotes the recovery of gastrointestinal function after CIA in neonates and can improve clinical outcomes in children.Future research should explore optimal EN practices to enhance clinical application and child health.展开更多
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
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
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 describe the ages at diagnosis and operation of biliary atresia(BA) and its incidence over a 15-year period in Taiwan.METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan...AIM: To describe the ages at diagnosis and operation of biliary atresia(BA) and its incidence over a 15-year period in Taiwan.METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases,Ninth Revision(ICD-9) code of BA 751.61 plus Kasai operation(ICD-9 procedure code 51.37) or liver transplantation(LT,ICD-9 procedure code 50.5). The patients' characteristics including sex,age at diagnosis,age at receiving Kasai operation and age at receiving LT were compared among three birth cohorts:(1) 1997 to 2001;(2) 2002 to 2006; and(3) 2007 to 2011.RESULTS: There were a total of 540 BA cases(275 females) with an incidence of 1.62 per 10000 live births. No seasonality of BA was noted. The mean ages at diagnosis of three cohorts were 57.9,55.6 and 52.6 d.A linear regression model demonstrated a decreasing trend of the mean age at diagnosis(1.27 d per year). The proportion of BA cases that received the Kasai operation within 60 d of age increased from 76% to 81%. A total of 189(35%) BA patients underwent LT. The mean age at LT was reduced from 3-year-old to 1-year-old. The rates of LT were 25.6% and 32.3% in patients who received the Kasai operation within 60 d or after 60 d of age,respectively. All patients who did not undergo a Kasai operation eventually required LT.CONCLUSION: The ages at diagnosis and operation in BA cases have decreased over time. Kasai operation performed at younger age reduces the need for LT. The incidence of BA in Taiwan fluctuates,but without certain trend.展开更多
AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia.METHODS: Resected small bowel specimens from affected patients (n = 7) we...AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia.METHODS: Resected small bowel specimens from affected patients (n = 7) were divided into three parts (proximal, atretic, distal). Standard histology and enzyme immunohistochemistry anti-S100, anti-protein gene product (PGP) 9.5, anti-neurofilament (NF), antic-kit-receptor (CD117) was carried out on conventional paraffin sections of the proximal and distal part. RESULTS: The neuronal and glial markers (PGP 9.5, NF, S-100) were expressed in hypertrophied ganglia and nerve fibres within the myenteric and submucosal plexuses. Furthermore, the submucous plexus contained typical giant ganglia. The innervation pattern of the proximal bowel resembled intestinal neuronal dysplasia. The density of myenteric ICCs was clearly reduced in the proximal bowel, whereas a moderate number of muscular ICCs were found. The anti-CD117 immunore- action revealed additional numerous mast cells. The distal bowel demonstrated normal morphology and density of the ENS, the ICCs and the mast cells.CONCLUSION: The proximal and distal bowel in small bowel atresia revealed clear changes in morphology and density of the ENS and ICCs.展开更多
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 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
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
AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA ...AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.展开更多
AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA contr...AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies(ANAs), by line-blot assay; ANA and antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA(i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient.RESULTS The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls(14.8% vs 2.2%, P < 0.05). Accordingly, 32(25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(P < 0.05), with 6/92(8.6%) of patients with other liver diseases and 2/48(4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls(3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs(ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls(37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis(r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis.CONCLUSION High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.展开更多
BACKGROUND: 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 analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule(CD56) in patients with biliary atresia(BA).METHODS: Established clinical laboratory markers of hepatic funct...AIM: To analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule(CD56) in patients with biliary atresia(BA).METHODS: Established clinical laboratory markers of hepatic function, including enzyme activity, protein synthesis, and bilirubin metabolism, were evaluated in patients with BA and compared with those in patients with choledochal cysts and neonatal hepatitis. Pathological changes in tissue morphology and fibrosis were examined by histological and tissue collagen staining. Immunohistochemical staining for the biliary epithelial cell markers CD56 and CK19 together with the Notch signaling related molecules Notch1 and Notch2 was performed in the context of alterations in the structure of intrahepatic biliary ducts.RESULTS: Differences in some clinical laboratoryparameters among the three diseases examined were observed, but they did not correlate with the pathological classification of fibrosis in BA. Immunohistochemical staining showed the presence of CD56-positive immature bile ducts in most patients(74.5%) with BA but not in patients with choledochal cysts or neonatal hepatitis. The number of CD56-expressing cells correlated with disease severity, with more positive cells present in the later stages of liver damage(81.8% vs 18.2%). Furthermore, bile plugs were mainly found in CD56-positive immature biliary ducts. Notch signaling was a key regulatory pathway in biliary duct formation and played a role in tissue fibrosis. Notch1 was co-expressed in CD56-positive cells, whereas Notch2 was found exclusively in blood vessels in the portal area of patients with BA. CONCLUSION: The maturation of biliary epithelial cells and the expression of Notch may play a role in the pathogenesis of BA.展开更多
AIM To analyze the expression and function of the notchsignaling target gene Hes1 in a rhesus rotavirusinduced mouse biliary atresia model. METHODS The morphologies of biliary epithelial cells in biliary atresia patie...AIM To analyze the expression and function of the notchsignaling target gene Hes1 in a rhesus rotavirusinduced mouse biliary atresia model. METHODS The morphologies of biliary epithelial cells in biliary atresia patients and in a mouse model were examined by immunohistochemical staining. Then, the differential expression of Notch signaling pathway-related molecules was investigated. Further, the effects of the si RNAmediated inhibition of Hes1 expression were examined using a biliary epithelial cell 3 D culture system.RESULTS Both immature(Ep CAM+) and mature(CK19+) biliary epithelial cells were detected in the livers of biliary atresia patients without a ductile structure and in the mouse model with a distorted bile duct structure. The hepatic expression of transcripts for most Notch signaling molecules were significantly reduced on day 7 but recovered to normal levels by day 14, except for the target molecule Hes1, which still exhibited lower m RNA and protein levels. Expression of the Hes1 transcriptional co-regulator, RBP-Jκ was also reduced. A 3 D gel culture system promoted the maturation of immature biliary epithelial cells, with increased expression of CK19+ cells and the formation of a duct-like structure. The administration of Hes1 si RNA blocked this process. As a result, the cells remained in an immature state, and no duct-like structure was observed.CONCLUSION Our data indicated that Hes1 might contribute to the maturation and the cellular structure organization of biliary epithelial cells, which provides new insight into understanding the pathology of biliary atresia.展开更多
BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death...BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.展开更多
AIM: To investigate the 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
基金Supported by the Beijing Municipal Science and Technology Commission,No.Z191100006619002Haiyou Health High-Caliber Talent Project,No.202412the Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment,Chinese Academy of Medical Sciences,No.2021RU015.
文摘BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.
基金National Key Research and Development Program of China(2022YFD1300405)National Natural Science Foundation of China(32202671)Open project of State Key Laboratory of Animal Biotech Breeding(Grant No.2024SKLAB6-5).
文摘Background Follicular atresia significantly impairs female fertility and hastens reproductive senescence.Apoptosis of granulosa cells is the primary cause of follicular atresia.Pyroptosis and necroptosis,as additional forms of pro-grammed cell death,have been reported in mammalian cells.However,the understanding of pyroptosis and necrop-tosis pathways in granulosa cells during follicular atresia remains unclear.This study explored the effects of pro-grammed cell death in granulosa cells on follicular atresia and the underlying mechanisms.Results The results revealed that granulosa cells undergo programmed cell death including apoptosis,pyroptosis,and necroptosis during follicular atresia.For the first time,we identified the formation of a PANoptosome com-plex in porcine granulosa cells.This complex was initially identified as being composed of ZBP1,RIPK3,and RIPK1,and is recruited through the RHIM domain.Additionally,we demonstrated that caspase-6 is activated and cleaved,interacting with RIPK3 as a component of the PANoptosome.Heat stress may exacerbate the activation of the PANop-tosome,leading to programmed cell death in granulosa cells.Conclusions Our data identified the formation of a PANoptosome complex that promoted programmed cell death in granulosa cells during the process of follicular atresia.These findings provide new insights into the molecular mechanisms underlying follicular atresia.
基金funded by The National Key Research and Development Program of China,grant number 2022YFF1000202National Natural Science Foundation of China Grants(32402745).Sichuan Science and Technology Program,grant number 2023NSFSC1940,2021YFYZ0007,and 2021YFYZ0031China Agriculture Research System of MOF and MARA,grant number CARS-40.
文摘Programmed cell death(PCD),including autophagy,apoptosis,and ferroptosis,is a fundamental biological process that plays a critical role in follicular development and atresia in livestock.In ovaries,the vast majority of follicles undergo atresia,while only a small fraction reach ovulation.Emerging evidence suggests that these three forms of PCD are intricately involved in regulating follicular fate through distinct yet interconnected molecular mechanisms.This review summarizes recent advances in understanding the roles of autophagy,apoptosis,and ferroptosis in follicular development and atresia,with a focus on their molecular mechanisms and interactions.By elucidating the complex regulatory networks of PCD in ovarian physiology,this review aims to provide new insights into improving reproductive efficiency in livestock through targeted modulation of these pathways.
文摘BACKGROUND Air embolism(AE)is a rare but potentially fatal complication of intestinal endoscopy(IE).CASE SUMMARY Herein,we report the case of an 18-year-old woman who underwent a successful Kasai portoenterostomy(KPE)for biliary atresia but died of AE during intraoperative IE for stone removal at the portoenterostomy site.Our review of the English literature identified only four similar cases of fatal AE during IE in patients undergoing KPE.The common clinical setting in the five patients,including our case,was high-pressure air insufflation into the blind closed afferent loop of the KPE to secure visibility.We hypothesize that the highly pressurized air injected into the closed loop entered the bile canaliculi—previously opened by KPE for bile drainage—passed through the tiny,microscopic pores of the fenestrated liver sinusoid endothelial cells,and finally entered the bloodstream with ease,resulting in fatal AE.CONCLUSION Meticulous performance of IE,especially on the KPE blind loop,is warranted owing to the risk of AE.
文摘BACKGROUND Congenital intestinal atresia(CIA)is a common intestinal malformation in the neonatal period,and surgery is currently the main treatment method.The choice of postoperative feeding is crucial for the recovery of gastrointestinal function in children.AIM To compare and analyze the effects of different postoperative feeding methods on gastrointestinal function reconstruction in newborns with CIA.METHODS Twenty-six children diagnosed with neonatal CIA,treated with minimally invasive surgery at Shijiazhuang Maternal and Child Health Hospital between January 2021 and May 2024,were selected for this single-center prospective randomized controlled study.They were divided into two groups using envelope randomization:Enteral nutrition(EN)group(n=13)and parenteral nutrition(PN)group(n=13).Baseline and clinical characteristics were collected,and recovery time of bowel sounds and time to first defecation were used as evaluation indices for gastrointestinal functional reconstruction.Differences between the groups were analyzed using t-test,χ2 test,and Fisher’s exact test.Spearman’s correlation tests and linear regression models were employed to analyze factors influencing time to first defecation.RESULTS The time to bowel sound recovery(51.54 vs 65.85,P=0.013)and first defecation(58.15 vs 76.62,P<0.001)was shorter in the EN group compared to the PN group.Clinical improvements in the EN group,including discharge weight(P=0.044),hospital stay(P=0.027),white blood cell count(P=0.023),albumin content(P=0.013),and direct bilirubin content(P=0.018),were also better than those in the PN group.No substantial differences in postoperative complications were found between the groups.Correlation analysis indicated that abdominal infection and operation time may relate to time to first defecation.Linear regression analysis demonstrated a considerable association between EN feeding and shorter time to first defecation.Abdominal infection and an operation time>2 hours may be risk factors for prolonged time to first defecation.CONCLUSION EN substantially promotes the recovery of gastrointestinal function after CIA in neonates and can improve clinical outcomes in children.Future research should explore optimal EN practices to enhance clinical application and child health.
文摘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 The Ditmanson Medical Foundation Chia-Yi Christian Hospital Research ProgramNo.R102-11
文摘AIM: To describe the ages at diagnosis and operation of biliary atresia(BA) and its incidence over a 15-year period in Taiwan.METHODS: This was a population-based cohort study. BA cases were identified from the Taiwan National Health Insurance Research Database based on the International Classification of Diseases,Ninth Revision(ICD-9) code of BA 751.61 plus Kasai operation(ICD-9 procedure code 51.37) or liver transplantation(LT,ICD-9 procedure code 50.5). The patients' characteristics including sex,age at diagnosis,age at receiving Kasai operation and age at receiving LT were compared among three birth cohorts:(1) 1997 to 2001;(2) 2002 to 2006; and(3) 2007 to 2011.RESULTS: There were a total of 540 BA cases(275 females) with an incidence of 1.62 per 10000 live births. No seasonality of BA was noted. The mean ages at diagnosis of three cohorts were 57.9,55.6 and 52.6 d.A linear regression model demonstrated a decreasing trend of the mean age at diagnosis(1.27 d per year). The proportion of BA cases that received the Kasai operation within 60 d of age increased from 76% to 81%. A total of 189(35%) BA patients underwent LT. The mean age at LT was reduced from 3-year-old to 1-year-old. The rates of LT were 25.6% and 32.3% in patients who received the Kasai operation within 60 d or after 60 d of age,respectively. All patients who did not undergo a Kasai operation eventually required LT.CONCLUSION: The ages at diagnosis and operation in BA cases have decreased over time. Kasai operation performed at younger age reduces the need for LT. The incidence of BA in Taiwan fluctuates,but without certain trend.
文摘AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel atresia.METHODS: Resected small bowel specimens from affected patients (n = 7) were divided into three parts (proximal, atretic, distal). Standard histology and enzyme immunohistochemistry anti-S100, anti-protein gene product (PGP) 9.5, anti-neurofilament (NF), antic-kit-receptor (CD117) was carried out on conventional paraffin sections of the proximal and distal part. RESULTS: The neuronal and glial markers (PGP 9.5, NF, S-100) were expressed in hypertrophied ganglia and nerve fibres within the myenteric and submucosal plexuses. Furthermore, the submucous plexus contained typical giant ganglia. The innervation pattern of the proximal bowel resembled intestinal neuronal dysplasia. The density of myenteric ICCs was clearly reduced in the proximal bowel, whereas a moderate number of muscular ICCs were found. The anti-CD117 immunore- action revealed additional numerous mast cells. The distal bowel demonstrated normal morphology and density of the ENS, the ICCs and the mast cells.CONCLUSION: The proximal and distal bowel in small bowel atresia revealed clear changes in morphology and density of the ENS and ICCs.
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 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 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.
基金Supported by Ratchadapiseksompotch Fund, Faculty of Medicine, Chulalongkorn University, Thailand Research Fund, and the Commission on Higher Education
文摘AIM: To analyze plasma osteopontin levels and liver stiffness using transient elastography in postoperative biliary atresia (BA) children compared with healthy controls. METHODS: Thirty children with postoperative BA and 10 normal controls were enrolled. The patients were categorized into two groups according to their jaundicestatus. Plasma levels of osteopontin were determined using commercially available enzyme-linked immunosorbent assay. Liver stiffness was measured by using transient elastography (Fibroscan). Ten validated Fibroscan measurements were performed in each patient and control with the result expressed in kilopascals (kPa). RESULTS: Plasma osteopontin was significantly elevated in BA children compared with that of healthy controls (47.0 ± 56.4 ng/mL vs 15.1 ± 15.0 ng/mL, P = 0.01). The liver stiffness measurement was markedly elevated in the patients with BA compared with that of controls (26.9 ± 24.6 kPa vs 3.9 ± 0.7 kPa, P = 0.001). Subgroup analysis showed that the BA patients with jaundice had more pronounced plasma osteopontin levels than those without jaundice (87.1 ± 61.6 ng/mL vs 11.9 ± 6.1 ng/mL, P = 0.001). Furthermore, the mean liver stiffness was significantly greater in the jaundiced BA patients compared with non-jaundiced patients (47.7 ± 21.8 kPa vs 8.7 ± 3.0 kPa, P = 0.001). Additionally, plasma osteopontin was positively related to serum total bilirubin (r = 0.64, P < 0.001). There was also a correlation between plasma osteopontin and liver stiffness values (r = 0.60, P < 0.001). CONCLUSION: High plasma osteopontin positively correlated with degree of hepatic fibrosis and could be used as a biochemical parameter reflecting disease severity in postoperative BA children.
基金the Guangdong Provincial Science and Technology Planning Project,No.2014A020212520the Guangzhou Science and Technology Project,No.201707010014
文摘AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies(ANAs), by line-blot assay; ANA and antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA(i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient.RESULTS The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls(14.8% vs 2.2%, P < 0.05). Accordingly, 32(25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(P < 0.05), with 6/92(8.6%) of patients with other liver diseases and 2/48(4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls(3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs(ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls(37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis(r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis.CONCLUSION High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.
基金supported by 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Supported by The grant of State Clinical Key Specialty Construction Project(Pediatric Surgery)2013No.GJLCZD1301+1 种基金Guangdong Provincial Science and Technology Plan Projects 2014No.2014A020212373
文摘AIM: To analyze the clinical and pathological parameters and expression of the neural cell adhesion molecule(CD56) in patients with biliary atresia(BA).METHODS: Established clinical laboratory markers of hepatic function, including enzyme activity, protein synthesis, and bilirubin metabolism, were evaluated in patients with BA and compared with those in patients with choledochal cysts and neonatal hepatitis. Pathological changes in tissue morphology and fibrosis were examined by histological and tissue collagen staining. Immunohistochemical staining for the biliary epithelial cell markers CD56 and CK19 together with the Notch signaling related molecules Notch1 and Notch2 was performed in the context of alterations in the structure of intrahepatic biliary ducts.RESULTS: Differences in some clinical laboratoryparameters among the three diseases examined were observed, but they did not correlate with the pathological classification of fibrosis in BA. Immunohistochemical staining showed the presence of CD56-positive immature bile ducts in most patients(74.5%) with BA but not in patients with choledochal cysts or neonatal hepatitis. The number of CD56-expressing cells correlated with disease severity, with more positive cells present in the later stages of liver damage(81.8% vs 18.2%). Furthermore, bile plugs were mainly found in CD56-positive immature biliary ducts. Notch signaling was a key regulatory pathway in biliary duct formation and played a role in tissue fibrosis. Notch1 was co-expressed in CD56-positive cells, whereas Notch2 was found exclusively in blood vessels in the portal area of patients with BA. CONCLUSION: The maturation of biliary epithelial cells and the expression of Notch may play a role in the pathogenesis of BA.
基金Supported by the Science and Technology Project of Guangzhou,No.201707010014the National Natural Science Foundation of China,No.81600399 and No.81671498
文摘AIM To analyze the expression and function of the notchsignaling target gene Hes1 in a rhesus rotavirusinduced mouse biliary atresia model. METHODS The morphologies of biliary epithelial cells in biliary atresia patients and in a mouse model were examined by immunohistochemical staining. Then, the differential expression of Notch signaling pathway-related molecules was investigated. Further, the effects of the si RNAmediated inhibition of Hes1 expression were examined using a biliary epithelial cell 3 D culture system.RESULTS Both immature(Ep CAM+) and mature(CK19+) biliary epithelial cells were detected in the livers of biliary atresia patients without a ductile structure and in the mouse model with a distorted bile duct structure. The hepatic expression of transcripts for most Notch signaling molecules were significantly reduced on day 7 but recovered to normal levels by day 14, except for the target molecule Hes1, which still exhibited lower m RNA and protein levels. Expression of the Hes1 transcriptional co-regulator, RBP-Jκ was also reduced. A 3 D gel culture system promoted the maturation of immature biliary epithelial cells, with increased expression of CK19+ cells and the formation of a duct-like structure. The administration of Hes1 si RNA blocked this process. As a result, the cells remained in an immature state, and no duct-like structure was observed.CONCLUSION Our data indicated that Hes1 might contribute to the maturation and the cellular structure organization of biliary epithelial cells, which provides new insight into understanding the pathology of biliary atresia.
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.
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.
