Medicine › Surgery

Pediatric Hepatobiliary Diseases and Treatments

Works Count: 49893

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Description

This cluster of papers focuses on the pathogenesis, diagnosis, management, and outcomes of biliary atresia, including related conditions such as Alagille syndrome and choledochal cysts. It covers topics such as the Kasai operation, liver transplantation, inflammation, and the etiology of these conditions.

Keywords

Biliary Atresia; Alagille Syndrome; Kasai Operation; Cholestasis; Hepaticojejunostomy; Choledochal Cysts; Liver Transplantation; Inflammation; Pancreaticobiliary Maljunction; Etiology

[Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb].

1969-01-01

Babbitt Dp

Congenital bile duct cysts

1977-08-01

Takuji Todani , Yasuhiro Watanabe , Mitsuo Narusue +2 more

Congenital choledochal cyst

1980-11-01

Muneyuki Yamaguchi

Diseases of the Liver and Biliary System

2001-12-17

Sheila Sherlock , James Dooley

Diseases of the liver and biliary system , Diseases of the liver and biliary system , کتابخانه دیجیتال جندی شاپور اهواز Diseases of the liver and biliary system , Diseases of the liver and biliary system , کتابخانه دیجیتال جندی شاپور اهواز

Hepatogastroenterology

2011-09-26

Mingwei Zheng , Wang Cai , Mingfang Qin

To summarize our experience and results of combined endoscopic and laparoscopic treatment for Mirizzi syndrome (MS) retrospectively, and to evaluate the effect of the procedures of minimally invasive strategy.Fifty-four patients … To summarize our experience and results of combined endoscopic and laparoscopic treatment for Mirizzi syndrome (MS) retrospectively, and to evaluate the effect of the procedures of minimally invasive strategy.Fifty-four patients with Mirizzi syndrome were admitted to our centre. Endoscopic procedures were performed firstly to identify the Csendes Types and alleviate jaundice. Different laparoscopic surgeries were adopted according to Csendes Types. The clinical data about operation and recovery of the two groups was recorded and compared.Total 63 endoscopic procedures were performed successfully in 46 cases and failed in 3 cases. Five patients were found to have MS intraoperatively. Endoscopic complication rate was 31.7% (20/63). Forty-three patients underwent laparoscopic surgeries successfully and 7 cases were converted to open surgeries. The average laparoscopic operation time was 83.2±34.7min and postoperative hospital stay was 9.5±2.4 days. Postoperative complication rate was 16.3%. Median follow-up period was 38.6 (range, 16-83) months and 4 stone recurrence occurred.Combined endoscopic and laparoscopic approaches in treating patients with MS are technically feasible and minimally invasive. However, laparoscopic primary suture of the defect on the wall of common bile duct is difficult; hence it is recommended it be performed by experienced hands.

Molecular Pathogenesis of Cholestasis

2004-01-01

Michael Trauner , Peter L. M. Jansen

Mutations in the human Jagged1 gene are responsible for Alagille syndrome

1997-07-01

Takaya Oda , Abdel Elkahloun , Brian L. Pike +7 more

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Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1

1997-07-01

Linheng Li , Ian D. Krantz , Yu Deng +7 more

CONGENITAL ANOMALIES OF THE GALLBLADDER

1936-01-01

Robert E. Gross

Congenital anomalies of the gallbladder are rare, but it is desirable for the roentgenologist and surgeon to be familiar with the malformations of this organ which they so commonly examine … Congenital anomalies of the gallbladder are rare, but it is desirable for the roentgenologist and surgeon to be familiar with the malformations of this organ which they so commonly examine and operate on. Malformations of the bile ducts and their associated blood vessels have been admirably emphasized by Eisendrath, Haberland, Holmes, Howard and Wolbach, Kehr, Ladd, Mentzer and others, but such treatises have not included any comprehensive study of the gallbladder. It is my purpose in this presentation to collect from the literature the isolated reports relevant to the subject of anomalies of the gallbladder and to present this material in a unified manner. The review has been extensive but not exhaustive, for many articles have appeared in periodicals that were not available, and other examples have doubtless been overlooked. Numerous cases of the types under discussion were recorded before 1900, and while many of these were well described, only

Guideline for the Evaluation of Cholestatic Jaundice in Infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

2004-07-20

Virginia A. Moyer , Deborah K. Freese , Peter F. Whitington +4 more

For the primary care provider, cholestatic jaundice in infancy, defined as jaundice caused by an elevated conjugated bilirubin, is an uncommon but potentially serious problem that indicates hepatobiliary dysfunction. Early … For the primary care provider, cholestatic jaundice in infancy, defined as jaundice caused by an elevated conjugated bilirubin, is an uncommon but potentially serious problem that indicates hepatobiliary dysfunction. Early detection of cholestatic jaundice by the primary care physician and timely, accurate diagnosis by the pediatric gastroenterologist are important for successful treatment and a favorable prognosis. The Cholestasis Guideline Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnostic evaluation of cholestatic jaundice in the infant. The Cholestasis Guideline Committee, consisting of a primary care pediatrician, a clinical epidemiologist (who also practices primary care pediatrics), and five pediatric gastroenterologists, based its recommendations on a comprehensive and systematic review of the medical literature integrated with expert opinion. Consensus was achieved through the Nominal Group Technique, a structured quantitative method. The Committee examined the value of diagnostic tests commonly used for the evaluation of cholestatic jaundice and how those interventions can be applied to clinical situations in the infant. The guideline provides recommendations for management by the primary care provider, indications for consultation by a pediatric gastroenterologist, and recommendations for management by the pediatric gastroenterologist. The Cholestasis Guideline Committee recommends that any infant noted to be jaundiced at 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin. However, breast-fed infants who can be reliably monitored and who have an otherwise normal history (no dark urine or light stools) and physical examination may be asked to return at 3 weeks of age and, if jaundice persists, have measurement of total and direct serum bilirubin at that time. This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition on the evaluation of cholestatic jaundice in infants. The American Academy of Pediatrics has also endorsed these recommendations. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all patients with this problem.

Palliative Bile Duct Drainage - A New Endoscopic Method of Introducing a Transpapillary Drain

1980-01-01

N. Soehendra , V. Reynders-Frederix

An endoscopic method for placing an internal drain in the bile-duct is described. Compared to the nasobiliary suction-tube this method has the advantage that it guarantees the physiological flow of … An endoscopic method for placing an internal drain in the bile-duct is described. Compared to the nasobiliary suction-tube this method has the advantage that it guarantees the physiological flow of the bile into the duodenum, and that the patient is in no way inconvenienced. For high-risk or inoperable cases the method can be considered as an alternative to choledochoduodenostomy.

Wirsung and Santorini: The men behind the ducts

2002-01-01

G Flati , Åke Andrén‐Sandberg

Congenital diseases of intrahepatic bile ducts: Variations on the theme “ductal plate malformation”

1992-10-01

Valeer Desmet

Congenital diseases of intrahepatic bile ducts (IHBDs) can be divided into two main groups: diseases characterized by necroinflammatory destruction of IHBDs and diseases characterized by a variable degree of ectasia … Congenital diseases of intrahepatic bile ducts (IHBDs) can be divided into two main groups: diseases characterized by necroinflammatory destruction of IHBDs and diseases characterized by a variable degree of ectasia of IHBDs and associated with a variable degree of fibrosis.This review will emphasize that in mostif not allof these conditions, persistence or lack of remodeling of the embryonic ductal plate (so-called "ductal plate malformation" [DPM]) is an essential precursor of the lesions (1). EMBRYOLOGY OF IHBDs AND DPMDuring the fourth week of human gestation, the liver arises as a bud of cells (the hepatic diverticulum) from the ventral wall of the most cephalad portion of the foregut, near the junction with the yolk sac (2).In the human, as in the mouse, the hepatic diverticulum comprises two parts: a pars cranialis or pars hepatica and a pars caudalis or pars cystica.The former gives rise to liver precursor cells that grow into the mesenchyme of the septum transversum, which consists of a mass of loosely arranged mesodermal cells separating the pericardial and peritoneal cavities.The caudal part of the hepatic diverticulum develops into the gallbladder and the common bile duct.The cranial or hepatic part of the diverticulum acquires a T-shaped cephalic end, with more pronounced growth of epithelial sprouts from the lateral walls, creating the early outline of the right and left lobes of the liver.The liver precursor cells grow between small endothelium-lined spaces in the septum transversum that connect with the capillary plexus of the vitelline (or omphalomesenteric) veins, thus establishing the basic architecture of liver parenchyma: parenchymal cords and plates alternating with hepatic sinusoids.During the first 7 wk or so of embryonic life, no intrahepatic bile duct system exists in the developing liver.Its formation begins around the eighth gestational week.

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Choledochal Cyst Disease A Changing Pattern of Presentation

1994-11-01

Pamela A. Lipsett , Henry A. Pitt , Paul M. Colombani +2 more

Objective The authors compared the presentation, treatment, and long-term outcome of children and adults with choledochal cysts. Summary Background Data The typical patient with choledochal cyst disease has been the … Objective The authors compared the presentation, treatment, and long-term outcome of children and adults with choledochal cysts. Summary Background Data The typical patient with choledochal cyst disease has been the female infant with the triad of jaundice, an abdominal mass, and pain. However, the recent experience of the authors suggested that the disease currently is recognized more commonly in adults. Methods Forty-two patients (11 children, 32 adults) with choledochal cyst disease were treated primarily at this institution between 1976 and 1993. Patient presentation, clinical evaluation, and operative treatment were obtained from existing records. Long-term follow-up was obtained by records, physician, or direct patient contact. Results One child—but no adults—had the classic triad of jaundice, abdominal mass, and pain. Children were more likely to have two of the three signs or symptoms (82% vs. 25%; p = < 0.05). Adult patients most commonly had abdominal pain and were thought to have pancreatitis (23%) or acute biliary tract symptoms, prompting cholecystectomy (50%). The type of choledochal cyst seen in children and adults was similar; the fusiform extrahepatic (Type I) was most common (50%), and the combined intrahepatic and extrahepatic (Type IVA) was the next most prominent (33%). For both children and adults, treatment consisted of excision of the cyst and biliary reconstruction with a hepaticojejunostomy. There was no surgical mortality. Gallbladder or cholangiocarcinoma was identified in three adults (9.7%), two of which were manifest on presentation. Long-term follow-up revealed one patient with a biliary stricture and three patients with Type IVA cysts who had intrahepatic stones. Conclusions Children and adults differ in presentation of choledochal cysts, with adults commonly having acute biliary tract or pancreatic symptoms. Surgical treatment with cyst excision and biliary bypass is safe and effective in children and adults with excellent long-term results that minimize the development of malignancy.

A New Xenobiotic-Induced Mouse Model of Sclerosing Cholangitis and Biliary Fibrosis

2007-06-29

Peter Fickert , Ulrike Stöger , Andrea Fuchsbichler +7 more

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Alagille syndrome: pathogenesis, diagnosis and management

2011-09-21

Peter D. Turnpenny , Sian Ellard

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A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

2006-04-01

Benjamin L. Shneider , Morton B. Brown , Barbara Haber +7 more

Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur

1975-01-01

D Alagille , M Odièvre , M Gautier +1 more

Fibroblast growth factor 15 functions as an enterohepatic signal to regulate bile acid homeostasis

2005-10-01

Takeshi Inagaki , Mihwa Choi , Antonio Moschetta +7 more

NOTCH2 Mutations Cause Alagille Syndrome, a Heterogeneous Disorder of the Notch Signaling Pathway

2006-06-07

Ryan M. McDaniell , Daniel M. Warthen , Pedro A. Sanchez‐Lara +4 more

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Endoskopische Sphinkterotomie der Papilla Vateri und Steinextraktion aus dem Ductus choledochus

1974-03-01

M. Claßen , L Demling

Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): Review of 80 cases

1987-02-01

D Alagille , Andrea Estrada , Michelle Hadchouel +3 more

Intrahepatic cholestasis of pregnancy: A retrospective case-control study of perinatal outcome

1994-03-01

Alonso Rioseco , Milenko Ivankovic , Alejandro Manzur +4 more

Cholesterol Solubility in Bile. EVIDENCE THAT SUPERSATURATED BILE IS FREQUENT IN HEALTHY MAN

1973-06-01

Rüdiger Holzbach , Mitsuko Marsh , Monica Olszewski +1 more

The development and validation of a direct method for measuring maximum cholesterol solubility in bile is described. Application of this method to five large mammalian species, including man, produced a … The development and validation of a direct method for measuring maximum cholesterol solubility in bile is described. Application of this method to five large mammalian species, including man, produced a micellar zone significantly smaller than that previously reported. Further studies on in vitro model solutions patterned after bile confirmed this new micellar zone. Thus, direct evidence demonstrates that the micellar zone boundary derived in vitro from model solutions is applicable to human gallbladder bile. Using the present criteria, normal human bile, in contrast to bile from other mammalian species, is commonly supersaturated with cholesterol. A male-female difference in bile composition is not demonstrable despite the well-established female preponderance of cholelithiasis. Bile from patients with cholesterol cholelithiasis has a micellar zone similar to normals but differs compositionally in that there is a greater excess of cholesterol above saturation. We conclude that cholesterol supersaturation may be a necessary but not solely sufficient cause for gallstone formation.

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Liver Anatomy: Portal (and Suprahepatic) or Biliary Segmentation

1999-01-01

C Couinaud

In liver anatomy and surgery, is portal and hepatic vein segmentation (French segmentation) to be preferred over arteriobiliary segmentation (Healey and Schroy, North American segmentation)?Several embryological arguments and an analysis … In liver anatomy and surgery, is portal and hepatic vein segmentation (French segmentation) to be preferred over arteriobiliary segmentation (Healey and Schroy, North American segmentation)?Several embryological arguments and an analysis of anatomical data from a personal collection of 110 vasculobiliary casts were made.Embryological arguments: Portal vein branching appears first, arteriobiliary branching secondly follows the portal vein distribution. Segment II (the left lateral sector) is the development of the right lateral embryological lobe. The umbilical vein enters the left portion of the middle embryological lobe, forming segment IV on the right and segment III on the left: this is the left paramedian sector. So the left portal fissure (between left and middle lobes) transversally crosses the classical left lobe, which is not a portal unit. Segment VI is a late secondary prominence of segment VII, reaching the anterior margin of the liver only in man. Anatomical arguments: hepatic vein segmentation must be added to portal segmentation; the academic left lobe is the left hepatic vein sector, and the left hepatic fissure separates the classical right and left lobes. Portal vein segmentation must be preferred: portal vein duplication of branches of first order occurs only in 23.5% of the cases, while arteriobiliary duplication of first-order branches is noted in 50% of the livers, portal segmentation being much simpler.Portal and hepatic vein segmentation seems to be much more accurate.

Safety and Efficacy of a Fish-Oil–Based Fat Emulsion in the Treatment of Parenteral Nutrition–Associated Liver Disease

2008-02-29

Kathleen M. Gura , Sang Hong Lee , Clarissa Valim +7 more

Parenteral nutrition-associated liver disease can be a progressive and fatal entity in children with short-bowel syndrome. Soybean-fat emulsions provided as part of standard parenteral nutrition may contribute to its pathophysiology.We … Parenteral nutrition-associated liver disease can be a progressive and fatal entity in children with short-bowel syndrome. Soybean-fat emulsions provided as part of standard parenteral nutrition may contribute to its pathophysiology.We compared safety and efficacy outcomes of a fish-oil-based fat emulsion in 18 infants with short-bowel syndrome who developed cholestasis (serum direct bilirubin level of > 2 mg/dL) while receiving soybean emulsions with those from a historical cohort of 21 infants with short-bowel syndrome who also developed cholestasis while receiving soybean emulsions. The primary end point was time to reversal of cholestasis (3 consecutive measurements of serum direct bilirubin level of < or = 2 mg/dL).Among survivors, the median time to reversal of cholestasis was 9.4 and 44.1 weeks in the fish-oil and historical cohorts, respectively. Subjects who received fish-oil-based emulsion experienced reversal of cholestasis 4.8 times faster than those who received soybean emulsions and 6.8 times faster in analysis adjusted for baseline bilirubin concentration, gestational age, and the diagnosis of necrotizing enterocolitis. A total of 2 deaths and 0 liver transplantations were recorded in the fish-oil cohort and 7 deaths and 2 transplantations in the historical cohort. The provision of fish-oil-based fat emulsion was not associated with essential fatty acid deficiency, hypertriglyceridemia, coagulopathy, infections, or growth delay.Parenteral fish-oil-based fat emulsions are safe and may be effective in the treatment of parenteral nutrition-associated liver disease.

Transcription factors in liver development, differentiation, and regeneration

2003-11-25

Robert H. Costa , Vladimir V. Kalinichenko , Ai‐Xuan L. Holterman +1 more

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Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis.

1980-02-01

Peter B. Cotton

Pancreas divisum occurs when the embryological ventral and dorsal parts of the pancreas fail to fuse, so that pancreatic drainage is mainly through the accessory papilla. In 169 patients with … Pancreas divisum occurs when the embryological ventral and dorsal parts of the pancreas fail to fuse, so that pancreatic drainage is mainly through the accessory papilla. In 169 patients with primary biliary tract disease who underwent pancreatography incidental to endoscopic cholangiography, the incidence of pancreas divisum was 3.6%. Among 78 patients with unexplained recurrent pancreatitis, the incidence was 25.6%. The hypothesis that the duct anomaly can cause obstructive pain and pancreatitis is presented, and supported by a detailed analysis of 47 patients with the condition.

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Surgical treatment of biliary atresia

1968-12-01

Morio Kasai , Shigeru Kimura , Yoshihiro Asakura +3 more

Surgical Pathology of the GI Tract, Liver, Biliary Tract, and Pancreas

2009-01-01

edited by Robert D. Odze , John R. Goldblum

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Impact of Age at Kasai Operation on Its Results in Late Childhood and Adolescence: A Rational Basis for Biliary Atresia Screening

2009-04-27

Marie‐Odile Serinet , Barbara E. Wildhaber , Pierre Broué +5 more

Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists … Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up. In this study we examined the relationship between increased age at surgery and its results in adolescence.All patients with biliary atresia who were living in France and born between 1986 and 2002 were included. Median follow-up in survivors was 7 years.Included in the study were 743 patients with biliary atresia, 695 of whom underwent a Kasai operation; 2-, 5-, 10-, and 15-year survival rates with native liver were 57.1%, 37.9%, 32.4%, and 28.5%, respectively. Median age at Kasai operation was 60 days and was stable over the study period. Whatever the follow-up (2, 5, 10, or 15 years), survival rates with native liver decreased when age at surgery increased (< or =30, 31-45, 46-60, 61-75, and 76-90 days). Accordingly, we estimated that if every patient with biliary atresia underwent the Kasai operation before 46 days of age, 5.7% of all liver transplantations performed annually in France in patients younger than 16 years could be spared.Increased age at surgery had a progressive and sustained deleterious effect on the results of the Kasai operation until adolescence. These findings indicate a rational basis for biliary atresia screening to reduce the need for liver transplantations in infancy and childhood.

The frequency and outcome of biliary atresia in the UK and Ireland

2000-01-01

Patrick McKiernan , Alastair Baker , Déirdre Kelly

Notch signaling controls liver development by regulating biliary differentiation

2009-04-16

Yiwei Zong , Archana Panikkar , Jie Xu +4 more

In the mammalian liver, bile is transported to the intestine through an intricate network of bile ducts. Notch signaling is required for normal duct formation, but its mode of action … In the mammalian liver, bile is transported to the intestine through an intricate network of bile ducts. Notch signaling is required for normal duct formation, but its mode of action has been unclear. Here, we show in mice that bile ducts arise through a novel mechanism of tubulogenesis involving sequential radial differentiation. Notch signaling is activated in a subset of liver progenitor cells fated to become ductal cells, and pathway activation is necessary for biliary fate. Notch signals are also required for bile duct morphogenesis, and activation of Notch signaling in the hepatic lobule promotes ectopic biliary differentiation and tubule formation in a dose-dependent manner. Remarkably, activation of Notch signaling in postnatal hepatocytes causes them to adopt a biliary fate through a process of reprogramming that recapitulates normal bile duct development. These results reconcile previous conflicting reports about the role of Notch during liver development and suggest that Notch acts by coordinating biliary differentiation and morphogenesis.

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Expression of tissue inhibitor of metalloproteinases 1 and 2 is increased in fibrotic human liver

1996-03-01

RC Benyon , John P. Iredale , S Goddard +2 more

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The cholangiopathies: Disorders of biliary epithelia

2004-11-01

Konstantinos N. Lazaridis , Mario Strazzabosco , Nicholas F. LaRusso

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A mouse model of Alagille syndrome:Notch2as a genetic modifier ofJag1haploinsufficiency

2002-02-15

Brent McCright , Julie Lozier , Thomas Gridley

Alagille syndrome is a human autosomal dominant developmental disorder characterized by liver, heart, eye, skeletal, craniofacial and kidney abnormalities. Alagille syndrome is caused by mutations in the Jagged 1 (JAG1) … Alagille syndrome is a human autosomal dominant developmental disorder characterized by liver, heart, eye, skeletal, craniofacial and kidney abnormalities. Alagille syndrome is caused by mutations in the Jagged 1 (JAG1) gene, which encodes a ligand for Notch family receptors. The majority of JAG1 mutations seen in Alagille syndrome patients are null alleles, suggesting JAG1 haploinsufficiency as a primary cause of this disorder. Mice homozygous for a Jag1 null mutation die during embryogenesis and Jag1/+ heterozygous mice exhibit eye defects but do not exhibit other phenotypes characteristic of Alagille syndrome patients (Xue, Y., Gao, X., Lindsell, C. E., Norton, C. R., Chang, B., Hicks, C., Gendron-Maguire, M., Rand, E. B., Weinmaster, G. and Gridley, T. (1999) Hum. Mol. Genet.8, 723-730). Here we report that mice doubly heterozygous for the Jag1 null allele and a Notch2 hypomorphic allele exhibit developmental abnormalities characteristic of Alagille syndrome. Double heterozygous mice exhibit jaundice, growth retardation, impaired differentiation of intrahepatic bile ducts and defects in heart, eye and kidney development. The defects in bile duct epithelial cell differentiation and morphogenesis in the double heterozygous mice are similar to defects in epithelial morphogenesis of Notch pathway mutants in Drosophila, suggesting that a role for the Notch signaling pathway in regulating epithelial morphogenesis has been conserved between insects and mammals. This work also demonstrates that the Notch2 and Jag1 mutations interact to create a more representative mouse model of Alagille syndrome and provides a possible explanation of the variable phenotypic expression observed in Alagille syndrome patients.

Features of Alagille Syndrome in 92 Patients: Frequency and Relation to Prognosis

1999-03-01

Karan M. Emerick , Elizabeth B. Rand , Elizabeth Goldmuntz +3 more

We have studied 92 patients with Alagille syndrome (AGS) to determine the frequency of clinical manifestations and to correlate the clinical findings with outcome. Liver biopsy specimens showed paucity of … We have studied 92 patients with Alagille syndrome (AGS) to determine the frequency of clinical manifestations and to correlate the clinical findings with outcome. Liver biopsy specimens showed paucity of the interlobular ducts in 85% of patients. Cholestasis was seen in 96%, cardiac murmur in 97%, butterfly vertebrae in 51%, posterior embryotoxon in 78%, and characteristic facies in 96% of patients. Renal disease was present in 40% and intracranial bleeding or stroke occurred in 14% of patients. The presence of intracardiac congenital heart disease was the only clinical feature statistically associated with increased mortality ( P < .001). Initial measures of hepatic function in infancy including absence of scintiscan excretion were not predictive of risk for transplantation or increased mortality. The hepatic histology of these AGS patients showed a significant increase in the prevalence of bile duct paucity ( P = .002) and fibrosis ( P < .001) with increasing age. Liver transplantation for hepatic decompensation was necessary in 21% (19 of 92) of patients with 79% survival 1–year posttransplantation. Current mortality is 17% (16 of 92). The factors that contributed significantly to mortality were complex congenital heart disease (15%), intracranial bleeding (25%), and hepatic disease or hepatic transplantation (25%). The 20–year predicted life expectancy is 75% for all patients, 80% for those not requiring liver transplantation, and 60% for those who required liver transplantation.

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The onecut transcription factor HNF6 is required for normal development of the biliary tract

2002-04-15

Frédéric Clotman , Vincent Lannoy , Michaël Reber +6 more

During liver development, hepatoblasts differentiate into hepatocytes or biliary epithelial cells (BEC). The BEC delineate the intrahepatic and extrahepatic bile ducts, and the gallbladder. The transcription factors that control the … During liver development, hepatoblasts differentiate into hepatocytes or biliary epithelial cells (BEC). The BEC delineate the intrahepatic and extrahepatic bile ducts, and the gallbladder. The transcription factors that control the development of the biliary tract are unknown. Previous work has shown that the onecut transcription factor HNF6 is expressed in hepatoblasts and in the gallbladder primordium. We now show that HNF6 is also expressed in the BEC of the developing intrahepatic bile ducts, and investigate its involvement in biliary tract development by analyzing the phenotype of Hnf6–/– mice. In these mice, the gallbladder was absent, the extrahepatic bile ducts were abnormal and the development of the intrahepatic bile ducts was perturbed in the prenatal period. The morphology of the intrahepatic bile ducts was identical to that seen in mice whose Hnf1β gene has been conditionally inactivated in the liver. HNF1β expression was downregulated in the intrahepatic bile ducts of Hnf6–/– mice during development. Furthermore, we found that HNF6 can stimulate the Hnf1β promoter. We conclude that HNF6 is essential for differentiation and morphogenesis of the biliary tract and that intrahepatic bile duct development is controlled by a HNF6→HNF1β cascade.

Embryonic Lethality and Vascular Defects in Mice Lacking the Notch Ligand Jagged1

1999-05-01

Yun Xue , Xiang Gao , C.E. Lindsell +7 more

The Notch signaling pathway is an evolutionarily conserved intercellular signaling mechanism essential for embryonic development in mammals. Mutations in the human JAGGED1 (JAG1) gene, which encodes a ligand for the … The Notch signaling pathway is an evolutionarily conserved intercellular signaling mechanism essential for embryonic development in mammals. Mutations in the human JAGGED1 (JAG1) gene, which encodes a ligand for the Notch family of transmembrane receptors, cause the autosomal dominant disorder Alagille syndrome. We have examined the in vivo role of the mouse Jag1 gene by creating a null allele through gene targeting. Mice homozygous for the Jag1 mutation die from hemorrhage early during embryogenesis, exhibiting defects in remodeling of the embryonic and yolk sac vasculature. We mapped the Jag1 gene to mouse chromosome 2, in the vicinity of the Coloboma (Cm) deletion. Molecular and complementation analyses revealed that the Jag1 gene is functionally deleted in the Cm mutant allele. Mice heterozygous for the Jag1 null allele exhibit an eye dysmorphology similar to that of Cm/+ heterozygotes, but do not exhibit other phenotypes characteristic of Cm/+ mice or of humans with Alagille syndrome. These results establish the phenotype of Cm/+ mice as a contiguous gene deletion syndrome and demonstrate that Jag1 is essential for remodeling of the embryonic vasculature.

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Vascular Anomalies in Alagille Syndrome

2004-03-02

Binita M. Kamath , Nancy B. Spinner , Karan M. Emerick +4 more

Background— Alagille syndrome (AGS) is a dominantly inherited multisystem disorder involving the liver, heart, eyes, face, and skeleton, caused by mutations in Jagged1 . Intracranial bleeding is a recognized complication … Background— Alagille syndrome (AGS) is a dominantly inherited multisystem disorder involving the liver, heart, eyes, face, and skeleton, caused by mutations in Jagged1 . Intracranial bleeding is a recognized complication and cause of mortality in AGS. There are multiple case reports of intracranial vessel abnormalities and other vascular anomalies in AGS. The objective of this study was to characterize the nature and spectrum of vascular anomalies in AGS. Methods and Results— Retrospective chart review of 268 individuals with AGS was performed. Twenty-five patients (9%) had noncardiac vascular anomalies or events. Sixteen patients had documented structural vascular abnormalities. Two had basilar artery aneurysms, 7 had internal carotid artery anomalies, and another had a middle cerebral artery aneurysm. Moyamoya disease was described in 1 patient. Three of the 16 patients had aortic aneurysms, and 2 had aortic coarctations. One of the patients with a basilar artery aneurysm also had coarctation of the aorta. One of the individuals with an internal carotid artery anomaly also had renal artery stenosis. Nine more patients had intracranial events without documented vessel abnormalities. Vascular accidents accounted for 34% of the mortality in this cohort. Conclusions— The vascular anomalies described in our cohort of AGS individuals identify an underrecognized and potentially devastating complication of this disorder. It is a major cause of morbidity and mortality in this population, accounting for 34% of the mortality. We have also reviewed the body of evidence supporting a role for Jagged1 and the Notch signaling pathway in vascular development.

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Association of severe intrahepatic cholestasis of pregnancy with adverse pregnancy outcomes: A prospective population‐based case‐control study

2013-07-15

Victoria Geenes , Lucy C. Chappell , Paul T. Seed +3 more

Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disease, characterized by maternal pruritus and raised serum bile acids. Our objectives were to describe the epidemiology and pregnancy complications associated … Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disease, characterized by maternal pruritus and raised serum bile acids. Our objectives were to describe the epidemiology and pregnancy complications associated with severe ICP and to test the hypothesis that adverse perinatal outcomes are increased in these women. A prospective population-based case-control study with national coverage was undertaken using the UK Obstetric Surveillance System (UKOSS). Control data for comparison were obtained from women with healthy pregnancy outcome through UKOSS (n = 2,232), St Mary's Maternity Information System (n = 554,319), and Office for National Statistics (n = 668,195). The main outcome measures investigated were preterm delivery, stillbirth, and neonatal unit admission. In all, 713 confirmed cases of severe ICP were identified, giving an estimated incidence of 9.2 per 10,000 maternities. Women with severe ICP and a singleton pregnancy (n = 669) had increased risks of preterm delivery (164/664; 25% versus 144/2200; 6.5%; adjusted odds ratio [OR] 5.39, 95% confidence interval [CI] 4.17 to 6.98), neonatal unit admission (80/654; 12% versus 123/2192; 5.6%; adjusted OR 2.68, 95% CI 1.97 to 3.65), and stillbirth (10/664; 1.5% versus 11/2205; 0.5%; adjusted OR 2.58, 95% CI 1.03 to 6.49) compared to controls. Seven of 10 stillbirths in ICP cases were associated with coexisting pregnancy complications. These differences remained significant against national data. Risks of preterm delivery, meconium-stained amniotic fluid, and stillbirth rose with increasing maternal serum bile acid concentrations. In the largest prospective cohort study in severe ICP to date, we demonstrate significant increased risks of adverse perinatal outcomes, including stillbirth. Our findings support the case for close antenatal monitoring of pregnancies affected by severe ICP.

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Dilated biliary tract: evaluation with MR cholangiography with a T2-weighted contrast-enhanced fast sequence.

1991-12-01

Bernd Wallner , K Schumacher , W. Weidenmaier +1 more

A heavily T2-weighted gradient-echo sequence was used for magnetic resonance (MR) imaging of the biliary system in five healthy volunteers and 13 patients with obstructive jaundice. Images were obtained in … A heavily T2-weighted gradient-echo sequence was used for magnetic resonance (MR) imaging of the biliary system in five healthy volunteers and 13 patients with obstructive jaundice. Images were obtained in the sagittal and coronal planes during sequential breath-hold intervals and were post-processed by using a maximum-intensity projection algorithm. The extrahepatic and intrahepatic bile ducts were well visualized in 11 patients. The level of obstruction and the grade of dilatation were depicted with MR cholangiography. However, the cause of obstruction could be determined with MR cholangiography in only eight cases. The part of the biliary system below the obstruction could not be visualized with MR cholangiography. In the volunteers, MR cholangiography could demonstrate the anatomy of the biliary tract in only two subjects. Possible causes for this phenomenon are the limited spatial resolution of MR imaging, partial volume effects, or flow within the bile ducts. MR cholangiography may be a useful adjunctive tool for noninvasive evaluation of patients with obstructive jaundice. However, further technical advances are necessary to improve image quality.

Cholangiocytes derived from human induced pluripotent stem cells for disease modeling and drug validation

2015-07-13

Fotios Sampaziotis , Miguel Cardoso de Brito , Pedro Madrigal +7 more

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Mechanisms of bile formation, hepatic uptake, and biliary excretion.

1984-03-01

Curtis D. Klaassen , J. B. Watkins

Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases.

1959-01-01

F Alonso-Lej , Rever Wb , Pessagno Dj

Histochemistry of Hepatic Phosphatases at a Physiologic pH: With Special Reference to the Demonstration of Bile Canaliculi

1957-01-01

M. Wachstein , E. Meisel

Journal Article Histochemistry of Hepatic Phosphatases at a Physiologic pH: With Special Reference to the Demonstration of Bile Canaliculi Get access Max Wachstein, M.D., Max Wachstein, M.D. Department of Pathology, … Journal Article Histochemistry of Hepatic Phosphatases at a Physiologic pH: With Special Reference to the Demonstration of Bile Canaliculi Get access Max Wachstein, M.D., Max Wachstein, M.D. Department of Pathology, St. Catherine's Hospital, Brooklyn 6, New York Search for other works by this author on: Oxford Academic Google Scholar Elizabeth Meisel, Ph.D. Elizabeth Meisel, Ph.D. Department of Pathology, St. Catherine's Hospital, Brooklyn 6, New York Search for other works by this author on: Oxford Academic Google Scholar American Journal of Clinical Pathology, Volume 27, Issue 1, 1 January 1957, Pages 13–23, https://doi.org/10.1093/ajcp/27.1.13 Published: 01 January 1957 Article history Received: 08 June 1956 Accepted: 18 September 1956 Published: 01 January 1957

Liver Disease in Alpha1-Antitrypsin Deficiency Detected by Screening of 200,000 Infants

1976-06-10

Tomas Sveger

We prosepctively studied 200,000 newborns to determine the frequency and clinical characteristics of alpha1-antitrypsin deficiency. One hundred and twenty Pi Z, 48 Pi SZ, two PI Z-and one Pi S-infants … We prosepctively studied 200,000 newborns to determine the frequency and clinical characteristics of alpha1-antitrypsin deficiency. One hundred and twenty Pi Z, 48 Pi SZ, two PI Z-and one Pi S-infants were identified and followed to the age of six months. Fourteen of 120 Pi Z infants had prolonged obstructive jaundice, nine with severe clinical and laboratory evidence of liver disease. Five had only laboratory evidence of liver disease. Eight other Pi Z infants had minimal abnormalities in serum bilirubin and hepatic enzyme activity and variable hepatosplenomegaly. All 22 Pi Z infants with hepatic abnormalities, two thirds of whom were made, appeared healthy at six months of age. Ninety-eight Pi Z infants did not have clinical liver disease, but liver-function tests gave abnormal results in 44 of 84 at three months, and in 36 of 60 at six months of age. The number of small-for-gestational-age infants was greater (P less than 0.001) among those with clinical liver disease. None of the 48 Pi SZ infants had clinical liver disease, but 10 of 42 at three months and one of 22 at six months of age had abnormal liver function. The Pi Z and Pi SZ phenotypes are associated with covert or readily apparent hepatic dysfunction in the first three months of life.

Guideline for the Evaluation of Cholestatic Jaundice in Infants

2016-07-16

Rima Fawaz , Ulrich Baumann , Udeme D. Ekong +7 more

Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic … Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans. Thus, these guidelines can have an essential role for the evaluation of neonatal cholestasis to optimize care. The recommendations from this clinical practice guideline are based upon review and analysis of published literature and the combined experience of the authors. The committee recommends that any infant noted to be jaundiced after 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin, and that an elevated serum direct bilirubin level (direct bilirubin levels >1.0 mg/dL or >17 μmol/L) warrants timely consideration for evaluation and referral to a pediatric gastroenterologist or hepatologist. Of note, current differential diagnostic plans now incorporate consideration of modern broad-based next-generation DNA sequencing technologies in the proper clinical context. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all infants with cholestasis. Broad implementation of these recommendations is expected to reduce the time to the diagnosis of pediatric liver diseases, including biliary atresia, leading to improved outcomes.

Cholangiocyte pathobiology

2019-03-08

Jesús M. Bañales , Robert C. Huebert , Tom H. Karlsen +3 more

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Biliary atresia

2009-11-01

Jane Hartley , Mark Davenport , Déirdre Kelly

Correction for: “Anatomic Variants of the Biliary Tree: MR Cholangiographic Findings and Clinical Applications”

2025-06-25

| American Journal of Roentgenology

A 29-Year-Old Woman with Jaundice

2025-06-24

Nadia Eden , Victoria Appleby | NEJM Evidence

AbstractMorning Report is a time-honored tradition in which physicians-in-training present cases to their colleagues and clinical experts to collaboratively explore a patient's presentation. The Morning Report section seeks to carry … AbstractMorning Report is a time-honored tradition in which physicians-in-training present cases to their colleagues and clinical experts to collaboratively explore a patient's presentation. The Morning Report section seeks to carry on this tradition by presenting a patient's chief concern and story, inviting the reader to develop a differential diagnosis and discover the diagnosis alongside the authors of this case. This report examines the story of a 29-year-old woman who presented to the emergency department with jaundice and oral mucosal bleeding, 2 days after delivering an infant at home at 36 weeks and 2 days of gestation. Using targeted questions, history-taking, physical examination, and diagnostic testing, an illness script emerges. As the case unfolds, the differential diagnosis is refined until a final diagnosis is reached.

Carcinoma sequence inferred from increasing age at choledochal cyst excision

2025-06-24

Derek A Riffert , Heidi Chen , James L. Rogers +7 more | Surgery

Choledochal cysts pose risk for biliary carcinoma, although age-related progression of biliary epithelial transformation remains uncharacterized. This study aimed to elucidate the carcinoma sequence within choledochal cysts across all patient … Choledochal cysts pose risk for biliary carcinoma, although age-related progression of biliary epithelial transformation remains uncharacterized. This study aimed to elucidate the carcinoma sequence within choledochal cysts across all patient ages to inform cancer risk and screening. Patients treated either for choledochal cysts or biliary carcinoma at 1 institution (1988-2023) were analyzed. Choledochal cyst pathology was reviewed for biliary epithelial changes, whereas patients with biliary carcinoma were assessed for history of choledochal cysts. Descriptive statistics and logistic regression of age at epithelial change were performed. Among 130 patients with choledochal cysts, median age at resection was 12 years, female patients predominated (73%), and Asian race was 2.5-fold increased. Patients commonly presented with biliary obstruction, pancreatitis, and type I choledochal cysts. Cyst pathology revealed chronic inflammation (56.1%) and progressive epithelial transformation (6.9%): 3 metaplasia, 1 hyperplasia, 3 dysplasia, and 3 synchronous biliary carcinoma. Logistic regression showed positive association between increasing age at excision and progressive epithelial transformation (P = .038). One 6-year-old patient with choledochal cysts developed metachronous biliary carcinoma at age 37 years. Among 886 nonampullary patients with biliary carcinoma, 8 (0.9%) had choledochal cysts: 7 synchronous and the same metachronous. Resection of choledochal cysts after age 17 years showed increased odds for epithelial transformation (odds ratio, 15.0; P = .0093). Cumulatively, median age (years) increased from premalignant biliary epithelial transformation (34 [16-35]), to biliary carcinoma with choledochal cysts (51 [37-78]), to biliary carcinoma without choledochal cysts (65 [56-72]; P < .001). These data infer sequential epithelial transformation to biliary carcinoma correlating with increasing age at resection of choledochal cysts. Coupled with 1 case of metachronous biliary carcinoma, this study underscores the need to screen patients with previous excision of choledochal cysts for cancer beginning at age 30 years.

Long-term Efficacy and Safety of Selective PPARδ Agonist Seladelpar in Primary Biliary Cholangitis: ASSURE Interim Study Results

2025-06-24

Cynthia Levy , Palak Trivedi , Kris V. Kowdley +7 more | The American Journal of Gastroenterology

Evaluate interim data from the ongoing, open-label, long-term efficacy and safety ASSURE study of seladelpar, a selective peroxisome proliferator-activated receptor δ agonist, in primary biliary cholangitis (PBC). Patients rolling over … Evaluate interim data from the ongoing, open-label, long-term efficacy and safety ASSURE study of seladelpar, a selective peroxisome proliferator-activated receptor δ agonist, in primary biliary cholangitis (PBC). Patients rolling over from the phase 3, randomized, placebo-controlled, 12-month RESPONSE study or with previous participation in earlier legacy seladelpar studies were enrolled. Interim evaluations included composite biochemical response (alkaline phosphatase [ALP] <1.67×upper limit of normal [ULN], total bilirubin ≤ULN, and ALP decrease ≥15%), pruritus numerical rating scale (NRS) change among patients with a baseline score ≥4, and safety. At interim cutoff, 337 patients were enrolled and received ≥1 seladelpar 10-mg dose; 54 placebo-treated and 104 seladelpar-treated from RESPONSE and 179 from legacy studies. The composite response rate at RESPONSE completion was 62% (79/128) with seladelpar and 20% (13/65) with placebo. After 12 months in ASSURE, response rates were 72% (21/29) in patients continuing seladelpar and 94% (15/16) in crossover seladelpar patients. In legacy trial patients, response rates were 73% (120/164) and 70% (69/99) after 12 and 24 months of treatment in ASSURE, respectively. The NRS decrease at RESPONSE completion in seladelpar-treated patients with baseline NRS ≥4 (-3.4) was maintained after 6 additional months of treatment (-3.8); changes were similar in crossover seladelpar (-3.8) and legacy patients (-3.5) after 6 months of treatment in ASSURE. No seladelpar-related serious adverse events were reported. Seladelpar demonstrated durable improvements in cholestatic biomarkers and pruritus in patients with PBC with up to 2 years of treatment and remained overall safe with long-term use.

A comparative study of childhood versus adult-onset biliary complications following pediatric choledochal cyst surgery

2025-06-23

Wei‐Jen Chen , Long Li , Xianhua Hao +5 more | Pediatric Surgery International

Development and validation of a SOTA-based system for biliopancreatic segmentation and station recognition system in EUS

2025-06-23

Jingjie Zhang , Junqian Zhang , Hongsuo Chen +4 more | Surgical Endoscopy

Endoscopic ultrasound (EUS) is a vital tool for diagnosing biliopancreatic disease, offering detailed imaging to identify key abnormalities. Its interpretation demands expertise, which limits its accessibility for less trained practitioners. … Endoscopic ultrasound (EUS) is a vital tool for diagnosing biliopancreatic disease, offering detailed imaging to identify key abnormalities. Its interpretation demands expertise, which limits its accessibility for less trained practitioners. Thus, the creation of tools or systems to assist in interpreting EUS images is crucial for improving diagnostic accuracy and efficiency. To develop an AI-assisted EUS system for accurate pancreatic and biliopancreatic duct segmentation, and evaluate its impact on endoscopists' ability to identify biliary-pancreatic diseases during segmentation and anatomical localization. The EUS-AI system was designed to perform station positioning and anatomical structure segmentation. A total of 45,737 EUS images from 1852 patients were used for model training. Among them, 2881 images were for internal testing, and 2747 images from 208 patients were for external validation. Additionally, 340 images formed a man-machine competition test set. During the research process, various newer state-of-the-art (SOTA) deep learning algorithms were also compared. In classification, in the station recognition task, compared to the ResNet-50 and YOLOv8-CLS algorithms, the Mean Teacher algorithm achieved the highest accuracy, with an average of 95.60% (92.07%-99.12%) in the internal test set and 92.72% (88.30%-97.15%) in the external test set. For segmentation, compared to the UNet ++ and YOLOv8 algorithms, the U-Net v2 algorithm was optimal. Ultimately, the EUS-AI system was constructed using the optimal models from two tasks, and a man-machine competition experiment was conducted. The results demonstrated that the performance of the EUS-AI system significantly outperformed that of mid-level endoscopists, both in terms of position recognition (p < 0.001) and pancreas and biliopancreatic duct segmentation tasks (p < 0.001, p = 0.004). The EUS-AI system is expected to significantly shorten the learning curve for the pancreatic EUS examination and enhance procedural standardization.

Three cases in which plasmapheresis was effective for persistent obstructive jaundice due to malignant biliary obstruction after biliary drainage

2025-06-22

Yuhei Iwasa , Keisuke Iwata , Ryuichi Tezuka +6 more | Clinical Journal of Gastroenterology

Choledochal Cysts in Children: A Single-Center Study in South India

2025-06-21

Venkat Kumar Raju Cherukuri , S. Radhakrishnan , RADHIKA PILLAI | Cureus

Commentary on “The causal effects of primary biliary cholangitis on irritable bowel syndrome: a Mendelian randomization study”

2025-06-20

Chuanyi Zhang , Longbi Liu , Zhen Wang +1 more | International Journal of Surgery

Yinchenhao Decoction Mitigates Cholestatic Liver Injury in Mice via Gut Microbiota Regulation and Activation of FXR-FGF15 Pathway

2025-06-20

Weiwei Li , Doudou Huang , Zichen Luo +2 more | Pharmaceuticals

Objective: Yinchenhao decoction (YCHD), a classical herbal formula comprising Artemisia capillaris, Gardenia jasminoides, and Rheum palmatum, has been clinically used for over 1000 years to treat cholestasis. However, its mechanism … Objective: Yinchenhao decoction (YCHD), a classical herbal formula comprising Artemisia capillaris, Gardenia jasminoides, and Rheum palmatum, has been clinically used for over 1000 years to treat cholestasis. However, its mechanism of action remains undefined. This study aimed to elucidate YCHD’s therapeutic mechanisms against cholestasis, with a focus on the gut microbiota-mediated regulation of the farnesoid X receptor (FXR)–fibroblast growth factor 15 (FGF15) pathway. Methods: An alpha-naphthyl isothiocyanate (ANIT)-induced cholestasis mouse model was established. Mice received YCHD (3/9 g/kg) for 7 days. 16S rRNA sequencing, targeted LC/MS (bile acid (BA) quantification), untargeted GC/MS (fecal metabolite detection), qPCR/Western blot (FXR pathway analysis), fecal microbiota transplantation (FMT), and antibiotic depletion were employed to dissect the gut–liver axis interactions. Results: YCHD alleviated cholestatic liver injury by reducing serum biomarkers, restoring BA homeostasis via FXR-FGF15 activation, and suppressing hepatic Cyp7a1-mediated BA synthesis. It remodeled gut microbiota, enriched FXR-activating secondary BAs (CDCA, DCA, CA), and restored the intestinal barrier integrity. Antibiotic cocktail abolished YCHD’s efficacy, while FMT from YCHD-treated mice enhanced its therapeutic effects, confirming microbiota dependency. Conclusions: YCHD mitigates cholestasis through gut microbiota-driven FXR activation and direct hepatobiliary regulation. These findings bridge traditional medicine and modern pharmacology, highlighting microbiome modulation as a therapeutic strategy for cholestatic liver diseases.

Letter to the editor: a commentary on “The causal effects of primary biliary cholangitis on irritable bowel syndrome a mendelian randomization study”

2025-06-20

Xintao Zeng , Wei He , Xi Chen +2 more | International Journal of Surgery

Analysis of risk factors affecting psycho-behavioural development in children with short bowel syndrome

2025-06-20

Yue Wang , Yu Li , Ruifei Zheng +4 more | Pediatric Surgery International

Acute-Onset Left Upper Quadrant Pain in a Young Woman

2025-06-19

Esat Kaba , Merve Solak , Yusuf Çubukçu +2 more | Annals of Emergency Medicine

Obstructive Icteric Syndrome in a Patient with a History of Burkitt's Lymphoma

2025-06-19

L Alvarez | Deleted Journal

The patient was a 16-year-old male with a history of Burkitt's lymphoma, stage III, diagnosed at 3 years of age and treated with chemotherapy, who presented with fever, productive cough, … The patient was a 16-year-old male with a history of Burkitt's lymphoma, stage III, diagnosed at 3 years of age and treated with chemotherapy, who presented with fever, productive cough, dyspnea not associated with exertion and altered state of consciousness. On physical examination, patient in regular clinical conditions, generalized icteric tinge. He presented paraclinical findings suggestive of icteric syndrome and an abdominal tomography that reported dilatation of intrahepatic and extrahepatic ducts, so the diagnosis of obstructive icteric syndrome was made. In the case presented, the etiopathogenesis of this patient's diagnosis can be explained by an extrinsic compression of the biliary tract due to a probable recurrence of the underlying pathology 13 years after his diagnosis.

Corrigendum: Surgical treatment for biliary obstruction in patients with painful chronic pancreatitis and a dilated duct: choledochojejunostomy or biliopancreatic tunneling?

2025-06-18

Đại Ngà , Quoc Vinh Nguyen , Minh Trí Phan +1 more | Y HOC TP HO CHI MINH

Expansion of CD103+CD69+CD8+ cytotoxic liver tissue resident memory T cells and inflammatory monocytes in advanced biliary atresia

2025-06-18

Freya Sibbertsen , Regine J. Dress , Sören Weidemann +7 more | Frontiers in Immunology

Introduction The pathogenesis of biliary atresia (BA) is unclear to date and no therapies targeting immune regulatory pathways exist. Here we characterized potent effector liver tissue resident memory CD8 + … Introduction The pathogenesis of biliary atresia (BA) is unclear to date and no therapies targeting immune regulatory pathways exist. Here we characterized potent effector liver tissue resident memory CD8 + T cells (Trm) and monocytic cells in children with advanced BA and an age-matched control group to gain insight into BA pathogenesis and immunologic regulation. Methods Liver explants from 18 children with biliary atresia and 10 with metabolic disease and normal histology were analyzed ex vivo by multicolor flow-cytometry and immunohistochemistry. Cytokines and cytotoxic mediators were quantified by intracellular staining and bead-based arrays in culture supernatant. Results The frequency of CD103 + CD69 + CD8 + Trm cells and CD14 + CD16 + monocytes was significantly higher in BA than in the control group. In BA, T cells showed elevated expression of CD103, CD69, CD39 and production of TNF-α and Granzyme-B ex vivo , which could be reproduced in vitro by allowing cell-contact with monocytes. Conclusions Cytotoxic CD8 + Trm cells and intrahepatic monocytes might contribute to tissue destruction in BA. Therapies targeting Trm cells or the TNF-α signaling pathway could be explored to delay progression to cirrhosis in BA.

Reverse sphincterotomy assisted accessory pancreatic duct cannulation

2025-06-18

Fan Fan , Jin‐Hui Yi , Qian Tong +1 more | Endoscopy

A two-hit model in biliary atresia: cooperative viral-bacterial activation of MMP7 via TLR4/NF-κB signaling

2025-06-18

Khaled Saad , Mostafa Embaby , Thamer Alruwaili +5 more | Pediatric Research

Unraveling the Yellow Mystery: Severe Jaundice and Multisystem Collapse in a Returning Traveler

2025-06-17

Bharadwaj Adithya Sateesh , Natalie Shammas , Ahmed Abdelfattah +4 more | Cureus

Cholestase

2025-06-17

A. Pariente | EMC - Hépatologie

Lemmel Syndrome Detected on 99mTc-Mebrofenin Hepatobiliary Scintigraphy

2025-06-17

Dhananjaya Saraswat , Pareena Malik , Suraj Kumar +5 more | Clinical Nuclear Medicine

Lemmel syndrome is a rare clinical condition characterized by cholestatic jaundice caused by compression of the common bile duct (CBD) by a periampullary duodenal diverticulum. The authors present a patient … Lemmel syndrome is a rare clinical condition characterized by cholestatic jaundice caused by compression of the common bile duct (CBD) by a periampullary duodenal diverticulum. The authors present a patient with post-cholecystectomy jaundice, in whom 99m Tc-Mebrofenin hepatobiliary scintigraphy showed pooling of tracer activity in the proximal CBD with delayed bilioenteric drainage. Single photon emission computed tomography/computed tomography detected a duodenal diverticulum medially displacing and compressing the distal CBD, which was corroborated by magnetic resonance cholangiopancreatography.

Corrigendum: partial quadrate lobectomy improves early outcomes of laparoscopic Kasai surgery in type III biliary atresia

2025-06-17

Chunhui Gu , Jian Sun , Lihong Ding +3 more | Frontiers in Pediatrics

Multimodal model for the diagnosis of biliary atresia based on sonographic images and clinical parameters

2025-06-17

Wenying Zhou , Run Lin , Yuanhang Zheng +7 more | npj Digital Medicine

Engineering a Human‐Sized Common Bile Duct Prototype with Regenerative Potential: In Vitro Evaluation of Mechanics, Function, Degradation, and Immune Modulation

2025-06-16

Mattia Pasqua , Marianna Barbuto , Matteo Calligaris +7 more | Advanced Healthcare Materials

Abstract Tissue engineering offers new hope for treating biliary defects. Several scaffolds have been proposed, but their properties have not been fully investigated. In this study, the design, fabrication, and … Abstract Tissue engineering offers new hope for treating biliary defects. Several scaffolds have been proposed, but their properties have not been fully investigated. In this study, the design, fabrication, and characterization of a novel common bile duct (CBD)‐like prototype are described. This prototype combines two biocompatible biomaterials, methacrylated type I collagen (CollMA) and poly(ε‐caprolactone) (PCL), along with biliary epithelial cells. CollMA supports the organization of biliary epithelial cells into a functional biliary‐like epithelium, as shown by histological, functional, and proteomic assays, while PCL provides mechanical strength. The biological and mechanical phases of the prototype are integrated into a multiphasic tubular scaffold using molding and electrospinning techniques. The final CBD‐like prototype consists of three interpenetrating phases: from innermost to outermost, these are biliary epithelial cell‐laden CollMA, PCL, and CollMA. This design overcomes challenges seen in multilayer constructs, such as interlayer delamination and lack of homogeneity. The prototype remains stable under physiological conditions, enables bile flow without leakage, and exhibits bile acid transport and modification activities, warranting future in vivo preclinical evaluation. In an ex vivo human blood assay, the acellular prototype elicited a favorable immune response, limiting inflammation and promoting sustained release of epidermal growth factor (EGF), indicating its potential to support regenerative processes.

Abnormal Pancreaticobiliary Junction in Children

2025-06-16

Khuld A Saeedi , Govind B. Chavhan , Tanja Gonska +3 more | Investigative Radiology

Background and Aims: There is scarcity of data on the prevalence of abnormal pancreaticobiliary junction (APBJ) in children. This study aimed to determine the frequency and clinical significance of APBJ … Background and Aims: There is scarcity of data on the prevalence of abnormal pancreaticobiliary junction (APBJ) in children. This study aimed to determine the frequency and clinical significance of APBJ including pancreas divisum (PD) and common channel (CC) using magnetic resonance cholangiopancreatography (MRCP). Materials and Methods: Single-center, retrospective study of MRCPs in children aged 0 to 18 years from 2012 to 2022. Two independent readers assessed PBJ visibility, abnormalities (PD, CC, other), and CC length. Findings were correlated with presenting diagnoses of choledochal cyst, biliary lithiasis, and pancreatitis. Results: A total of 631 MRCPs were included (46.8% females; mean age: 12 ± 5 y). The PBJ was visible in 85.7% of cases. APBJ was observed in 114/631 (18.1%) children, with PD in 47 (7.4%) cases and CC in 61 (9.7%) cases, with an average length of 9 mm (range, 3 to 22 mm). There was a significant inverse association between PD and biliary lithiasis ( P = 0.02). There was no association between PD and pancreatitis. CC was significantly associated with choledochal cyst ( P < 0.0001), pancreatitis ( P = 0.004) and biliary lithiasis ( P < 0.0001), with 21/61 (34.4%) of CC demonstrating stones within (median age: 3.8 y). The CC length was also significantly associated with complications ( P = 0.014), with complication-free cases having a median length of 7 mm (range, 4 to 8) compared with 11 mm (range, 3 to 25) in complicated cases. Conclusion: APBJ is a frequent finding on pediatric MRCP. CC is significantly associated with choledochal cyst, pancreatitis and lithiasis, and may show stones within them, particularly in small children. PD is inversely associated with biliary lithiasis. Careful PBJ assessment is important in children.

Cholecystoduodenostomy in Cats: indicate or not?

2025-06-15

Kátia Barão Corgozinho , Amanda Chaves de Jesus , Simone Carvalho dos Santos Cunha +2 more | ACTA SCIENTIAE VETERINARIAE

Background: Cholecystoduodenostomy is a surgical procedure recommended for cats suffering from extrahepatic biliary tract obstruction (EHBTO) when biliary tract patency cannot be reestablished. However, this procedure is associated with high … Background: Cholecystoduodenostomy is a surgical procedure recommended for cats suffering from extrahepatic biliary tract obstruction (EHBTO) when biliary tract patency cannot be reestablished. However, this procedure is associated with high perioperative morbidity and mortality. Cats diagnosed with EHBTO are considered to be at high risk for anesthetic and surgical complications. This study reports the case of a cat with severe jaundice that underwent cholecystoduodenostomy and successfully recovered, sparking a discussion on whether this procedure should be recommended to treat EHBTO.Case: A 15-year-old neutered male domestic shorthair cat presented with progressive weight loss, abdominal distension, and jaundice. Serum biochemical analyses showed high alkaline phosphatase, alanine aminotransferase, γ-glutamyl trans-peptidase, and total bilirubin levels Abdominal ultrasonography revealed several abnormalities, such as enlargement of the common bile duct, distension of gallbladder, gallbladder wall thickening, hyperechoic hepatomegaly, large hepatic mass (5.5 x 8.7 cm in left medial lobe), and small irregular kidneys, while the results of serum biochemical analyses indicated liver dysfunction. The cat received supportive care treatment and was later subjected to hepatic mass removal surgery and cholecystoduodenostomy. Complications included postoperative hypotension and anemia, which were treated with a blood transfusion to correct the low packed cell volume (PCV). The cat’s hypotension improved after the transfusion, and there were no complications related to the biliary enteric anastomosis. Unfortunately, 3 months after the surgery, the cat fell ill, exhibiting symptoms of anemia and jaundice, and was therefore euthanized. Necropsy revealed pancreatic carcinoma. Discussion: The most common clinical signs of EHBTO in cats are vomiting, anorexia, lethargy, weakness, and weight loss. The cat of this report showed weight loss and abdominal distension. Vomiting and anorexia may not be present in cats with EHBTO. Abdominal ultrasonography is the primary imaging modality for biliary tract disease. The examination revealed an enlarged gallbladder with a thickened wall, moderately enlarged cystic duct, and dilated common bile duct, consistent with other reports in cats with EHBTO. The diagnosis of complete versus partial biliary obstruction is challenging, as animals with this condition may respond to medical treatment instead of requiring immediate surgery. In this case report, the decision was made to prioritize medical management before considering surgery, in an attempt to stabilize the clinical condition and deal with the hepatic mass. The animal was elderly, but its owner insisted on surgery despite its clinical condition and age. The survival outcome of a cat with EHBTO is influenced by various factors such as age, clinical condition, hepatic impairment, anesthesia, surgical expertise, and the medical expertise of the veterinary team. The cat of this report survived for 3 months after surgery. The veterinary team was initially hesitant to perform the surgery because the cat was in poor clinical condition and they doubted it would survive. However, the owner insisted on the operation. The question raised is: were the three months of survival worth it? For the owner, the 3 months of survival were worth it. When a veterinary team recommends surgery, it is important to consider the animal’s clinical condition, the owner’s perspective, and the surgical qualifications of the veterinary team.Keywords: jaundice, extrahepatic biliary obstruction, cholecystoduodenostomy, pancreatic carcinoma.

From dogma to individualized care: the potential of 6-mm fully covered self-expandable metal stent in unresectable malignant distal biliary obstruction

2025-06-15

Hyung Ku Chon | Clinical Endoscopy

Current-generation sequencing: Has the next generation of malignant biliary stricture identification arrived?

2025-06-14

Omeed Alipour | Gastrointestinal Endoscopy

[Digital cholangioscopy assisted endoscopic retrograde cholangiopancreatography for pediatric common bile duct stones in 2 cases].

2025-06-14

Zhihua Zhang , Yingmo Shen , Kun Yan +4 more | PubMed

CXCL6 Is a Novel Biliary Marker and a Downstream Target of MMP7 in Biliary Atresia

2025-06-14

Fanyang Kong , Jingying Jiang , Min Du +4 more | Hepatology Research

ABSTRACT Aim MMP7 has been identified as a potential biomarker for biliary atresia (BA) diagnosis. However, the mechanism of MMP7 and its downstream signaling pathway remain unknown in the pathogenesis … ABSTRACT Aim MMP7 has been identified as a potential biomarker for biliary atresia (BA) diagnosis. However, the mechanism of MMP7 and its downstream signaling pathway remain unknown in the pathogenesis of BA. Herein, this study was performed to figure out MMP7's downstream target. Methods Single‐cell RNA sequencing (scRNA‐seq) was performed to screen out MMP7's downstream molecule CXCL6. QRT‐PCR, immunohistochemistry, western blot, and ELISA were used to determine the expressions of MMP7 and CXCL6 in the liver and serum of BA and controls. Immunofluorescence was conducted to validate the hepatic cellular expressions of MMP7 and CXCL6. In vitro, overexpression and knockdown of MMP7 in biliary epithelial cells (BECs) were established to verify the MMP7's regulation on CXCL6. Results ScRNA‐seq demonstrated that MMP7 and CXCL6 were exclusively expressed on cholangiocytes and up‐regulated in BA when compared with normal controls (NC). QRT‐PCR, immunohistochemistry, western blot and ELISA validated the higher expressions of MMP7 and CXCL6 in the liver and serum of BA when compared with non‐BA cholestasis (CS) and NC. Immunofluorescence further verified the biliary localization of MMP7 and CXCL6 in BA. Hepatic and serum CXCL6 expressions were positively correlated with MMP7 expressions, and both expressions were correlated with the stages of fibrosis in BA. Overexpression of MMP7 promoted CXCL6 expression, while knocking down MMP7 inhibited CXCL6 expression in BECs. Conclusion CXCL6 is a downstream target of MMP7, and is identified as a novel biliary marker in BA. The production of CXCL6 by MMP7 may exert pathological roles in the liver fibrogenesis of BA.

Double common bile duct - A rare case report and review of literature

2025-06-14

Tu Trong Doan , Duc Minh Pham , Cuong V. Nguyen +2 more | International Journal of Surgery Case Reports

Extrahepatic bile duct duplication is an extremely rare congenital anomaly. There are five different types of this malformation. Due to unusual anatomy, the bile duct can be damaged during surgery. … Extrahepatic bile duct duplication is an extremely rare congenital anomaly. There are five different types of this malformation. Due to unusual anatomy, the bile duct can be damaged during surgery. We herein report a case of double common bile duct and review the literature about this uncommon anatomical variation. A 72-year-old female was diagnosed with hepatic flexure colon cancer cT4bN1M0. The tumor invaded the pancreatic head and D2 duodenum in abdomen CT-scan. She underwent an en-bloc extended right hemicolectomy with duodenopancreatectomy and lymph node dissection. During surgery, we discovered that the cystic duct did not open into the common bile duct and there two parallel extrahepatic bile ducts merging into a single duct before draining into the D2 duodenum. Bile duct duplication is an extremely rare congenital variation and has only been reported in the literature as clinical cases. According to Choi, there were five types of these anomalies, our clinical case was classified as type V of this classification. We presented a case of double common bile duct discovered incidentally during surgery. The surgeons need to carefully expose the extrahepatic biliary tract to avoid bile duct injury.

A case of bile duct stones after surgery for congenital biliary dilatation successfully treated by hybrid endoscopic treatment with surgery

2025-06-13

Takumi Wada , Daisuke Ochi , Aya Osone +7 more | Progress of Digestive Endoscopy

Reoperation related to Roux-en-Y jejunal limb after hepaticojejunostomy of choledochal cyst

2025-06-12

Anxiao Ming , Shao yifeng , Mei Diao +3 more | Pediatric Surgery International

[Gallstone biliary and pancreatic disorders during pregnancy].

2025-06-11

Ouassila Benkhelifa , Virginia H R Monteil , Christian Toso +1 more | PubMed

Gallstone biliopancreatic disorders are frequent in pregnant women, given that these conditions are exacerbated by the hormonal changes of pregnancy. They present a clinical challenge with diagnostic and treatment complexities … Gallstone biliopancreatic disorders are frequent in pregnant women, given that these conditions are exacerbated by the hormonal changes of pregnancy. They present a clinical challenge with diagnostic and treatment complexities that require an individualized approach, ranging from conservative treatment to minimally invasive surgical interventions, favoring techniques without radiation exposure. Close multidisciplinary collaboration and open communication with the patient are essential to optimize care. This article highlights the «red flags» to identify to ensure timely patient management and prevent complications.