Biliary Atresia Research Papers - Academia.edu (original) (raw)

Background/purpose: Persistent inflammatory response secondary to congenital or acquired biliary choleastasis plays an important role in the pathophysiology of hepatic tissue damage. The polyunsaturated fatty acids (PUFA) have been shown to suppress the inflammatory reactions in vivo and in vitro. PUFA has been shown also to protect againts various types of experimental liver damage in animal models and isolated hepatocytes.

- by
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- Pediatrics, Stress, Medicine, Prevention

Purpose: This retrospective study reviews the long-term outcome of type I biliary atresia (BA). Methods: Three hundred twenty-three patients with BA, including 50 with type I, underwent corrective surgery. The surgical results, role of cholangiograms during the corrective surgery, late complications, and current statuses were evaluated. Results: The overall survival rate of the nontransplant type I patients was better than that of the type II/ III patients (52% vs 33%, P = .0009). Cholangiograms of 32 patients were classified into 3 types: cloudy (48%), treelike (13%), and mixed (39%). Of 26 patients who underwent corrective surgery in 1972 or later, 7 (50%), 7 (78%), and 3 (100%) patients of the cloudy type, mixed type, and treelike type, respectively, have survived without liver transplantation (LTx). Of 18 type I patients who survived more than 20 years without LTx, 7 developed severe late complications. Two of them eventually required LTx after 20 years old. Conclusions: Use of cholangiograms during corrective surgery might have a long-term prognostic value. The overall survival rate of type I BA was better than that of type II/III. The incidence of late complications was, however, considerably high in the type I survivors. All patients required careful long-term follow-up. D

- by Masaki Nio
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- Survival Analysis, Treatment Outcome, Pediatric Surgery, Pediatric
- by N. Kvist and +2
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- Survival Analysis, International Cooperation, Treatment Outcome, Europe

Biliary atresia (BA) remains an enigmatic disease with a degree of etiologic heterogeneity. A number of variants can be defined clinically, and these include the syndromic group (typically BA splenic malformation), cystic BA, and cytomegalovirus (CMV) IgM ϩve associated BA. The remainder, and still the largest group, may be termed isolated BA. There is a wide variation in incidence across the globe from 1 in 5000 in Taiwan to 1 in 20,000 live births in Northern Europe, although the reasons for such a disparity remain obscure. Management remains primarily surgical with an attempt to restore bile flow by resection of extrahepatic biliary remnants and a reconstruction portoenterostomy (the Kasai procedure), reserving liver transplantation for those where this fails or complications of chronic liver disease supervene. Clearance of jaundice to normal values has been achieved in 40%-55% of cases in large series from around the world, with an expectation of 5-year native liver survival of similar proportions.

- by Mark Davenport
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- Spleen, Outcome, Liver Transplantation, Prognosis

Purpose: We reviewed post-portoenterostomy (PE) biliary atresia (BA) patients who became "jaundicefree" (JF; total bilirubin (T-bil) ≤ 1.2 mg/dL) to determine factors associated with survival with the native liver (SNL). Methods: We reviewed 76 BA patients treated by PE at our institute between 1989, when liver transplantation (LTx) became available in Japan, and 2012, prospectively. Of these, 60 who became JF and remained JF were divided into two groups, SNL (n = 44) and LTx (n = 16). Age and weight at PE, pre-and post-PE T-bil, AST, γ-GT, time taken to become JF, corticosteroid requirements, incidence of cholangitis, and micro-bile duct size were compared between the two groups. Results: The SNL patients became JF significantly earlier: 58 vs. 115 days (p b .05). Corticosteroid requirement, cholangitis, and postoperative AST/γ-GT were significantly lower in the SNL patients (p b .05). SNL was significantly higher if patients became JF ≤ 60 days post-PE (p b .01). LTx was performed from 0.5 to 11 years post-PE (mean = 3.4). All patients who had cholangitis within 3 months of PE eventually required LTx (p b .05). Conclusions: Becoming JF ≤ 60 days post-PE would appear to be a factor associated with SNL, while cholangitis within 3 months of PE would appear to be associated with LTx. Elevation of AST and γ-GT would also appear to be early indicators of risk for LTx during follow-up of JF patients after successful PE.

- by Manabu Okawada
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- Treatment Outcome, Pediatric Surgery, Follow-up studies, Liver Transplantation

We report on a girl with biliary atresia (BA) who also suffered with anorectal agenesis without fistula and complicated urogenital malformation. The outcome of patients with these severe anomalies is poor, but she has survived without liver and/or renal transplantation for more than 3 years. A careful treatment plan for each anomaly in addition to prevention of cholangitis and urinary tract infection is indispensable for managing these complicated anomalies.

- by Masaki Nio
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- Pediatric Surgery, Urinary Tract, Treatment planning, Biliary Atresia

Sistem empedu terdiri dari organ-organ dan saluran (saluran empedu, kandung empedu, dan struktur terkait) yang terlibat dalam produksi dan transportasi empedu. Ketika sel-sel hati mengeluarkan empedu, yang dikumpulkan oleh sistem saluran yang mengalir dari hati melalui duktus hepatika kanan dan kiri. Saluran ini akhirnya mengalir ke duktus hepatik umum. Duktus hepatika kemudian bergabung dengan duktus sistikus dari kantong empedu untuk membentuk saluran empedu umum, yang berlangsung dari hati ke duodenum (bagian pertama dari usus kecil). Namun, tidak semua berjalan empedu langsung ke duodenum. Sekitar 50 persen dari empedu yang dihasilkan oleh hati adalah pertama disimpan di kantong empedu, organ berbentuk buah pir yang terletak tepat di bawah hati. Kemudian, ketika makanan dimakan, kontrak kandung empedu dan melepaskan empedu ke duodenum disimpan untuk membantu memecah lemak. Fungsi utama system bilier yang meliputi: • Untuk mengeringkan produk limbah dari hati ke duodenum • Untuk membantu dalam pencernaan dengan pelepasan terkontrol empedu Empedu merupakan cairan kehijauan-kuning (terdiri dari produk-produk limbah, kolesterol, dan garam empedu) yang disekresikan oleh sel-sel hati untuk melakukan dua fungsi utama, termasuk yang berikut: • Untuk membawa pergi limbah • Untuk memecah lemak selama pencernaan Garam empedu adalah komponen aktual yang membantu memecah dan menyerap lemak. Empedu, yang dikeluarkan dari tubuh dalam bentuk kotoran, adalah apa yang memberikan kotoran warna gelapnya coklat (Tim Ohio State University.2011.Sistem Bilier.Columbus:Medical center). 2.2 Definisi Atresia bilier Atresia bilier (biliary atresia) adalah suatu penghambatan di dalam pipa/saluran-saluran yang membawa cairan empedu (bile) dari liver menuju ke kantung empedu (gallbladder). Ini merupakan kondisi congenital, yang berarti terjadi saat kelahiran (Lavanilate.2010.Askep Atresia Bilier).

- by yuni mayuni
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- Biliary Atresia

Two patients presented with neonatal cholestasis and acholic stools as first manifestations of McCune-Albright syndrome. Both went through an extensive evaluation including an exploratory laparotomy with peroperative cholangiography which ruled out biliary atresia. One patient presented from the fourth month of life with the classical cafe-au-lait spots following Blaschko's lines, while less classical cafe-au-lait spots were seen in the second patient at the age of 4 years. Bone lesions were seen in one patient at the age of 2.5 years and in the other at the age of 4 years. Despite the severity of presentation, both patients cleared their jaundice within 6 months, but still had mild abnormalities of liver function tests. Both patients T"

- by Charles Sultan
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- Hepatology, DNA, Liver, Mutation

In Taiwan, a screening system using an infant stool color card to promote the early diagnosis of biliary atresia (BA) was established in 2002. This study aimed to investigate the 5-year outcome of BA before and after using the screening program. BA patients were divided into three cohorts according to their birth dates. The patients in cohort A (n 5 89) were born before the stool card screening program (1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999)(2000); those in cohort B (n 5 28) were screened by the stool card regional screening program (2002)(2003); and those in cohort C (n 5 74) were screened by the stool card universal screening program (2004)(2005). The relative odds ratios were computed using logistic regression to compare the different factors affecting survival time. The rate of age at Kasai operation <60 days was 49.4% and 65.7% in cohorts A and B1C, respectively (P 5 0.02). The jaundice-free (total serum bilirubin <2.0 mg/dL) rate 3 months after surgery was 34.8% and 60.8% in cohorts A and B1C, respectively (P < 0.001). The 3-year jaundice-free survival rate with native liver was 31.5% in cohort A and 56.9% in cohort B1C (P < 0.001), whereas the 3-year overall survival rates were 64.0% and 89.2%, respectively (P < 0.001). The 5-year jaundice-free survival rate with native liver was 27.3% in cohort A and 64.3% in cohort B (P < 0.001), and the 5-year overall survival rates were 55.7% and 89.3%, respectively (P < 0.001). Conclusion: The stool color card screening program for BA allows for earlier Kasai operation, which increases the jaundice-free rate at 3 months postsurgery. With higher surgical success rates, the 3-and 5-year outcome of BA patients in Taiwan improves remarkably. (HEPATOLOGY 2011;53:202-208) Abbreviations: BA, biliary atresia; CI, confidence interval; OR, odds ratio. From the

- by Hung-chang Lee and +1
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- Gastroenterology, Treatment Outcome, Hepatology, Color

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.

- by Paul Colombani
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- Surgery, Evaluation, Treatment, Infant

Patients with Alagille syndrome (AGS), a genetic disorder of Notch signaling, suffer from severe ductopenia and cholestasis, but progression to biliary cirrhosis is rare. Instead, in biliary atresia (BA) severe cholestasis is associated with a pronounced "ductular reaction" and rapid progression to biliary cirrhosis. Given the role of Notch in biliary development, we hypothesized that defective Notch signaling would influence the reparative mechanisms in cholestatic cholangiopathies. Thus we compared phenotype and relative abundance of the epithelial components of the hepatic reparative complex in AGS (n ‫؍‬ 10) and BA (n ‫؍‬ 30) using immunohistochemistry and computer-assisted morphometry.

- by Aurelio Sonzogni
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- Immunohistochemistry, Adolescent, Signal Transduction, Child

Systemic disorders in pediatric patients, such as congenital biliary atresia, acute liver failure, and biliary hypoplasia, may be the indications for a need of liver transplantation. One of the manifestations of these disorders is the elevated serum levels of bilirubin (hyperbiliru-binemia), a product of hemoglobin degradation, which is deposited in different tissues, including mineralized and soft tissues. When hyperbilirubinemia occurs during the period of dental development, these teeth can develop a green coloration, which remains permanently, because, after maturation, these tissues loose their metabolic activity. This case report describes a 9-year-old girl who required a liver transplant due to biliary atresia when she was three years old. Some of her pigmented teeth needed extraction and afterwards were submitted for histological analysis and compared with sound teeth.

- by CAROLINA GUERRA
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- Dentistry, Case Report, Child, Teeth
- by Mohamed Ibrahim
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- Surgery, Quality of life, Treatment, Evolution

Background The molecular basis of clinical cholestasis is a subject of intense investigation. Villin is an actin binding, bundling, and severing protein needed for maintenance of structural integrity of canalicular microvilli, in which membrane transporters required for bile secretion are located. We aimed to investigate the role of canalicular cytoskeletal proteins in three genetically unrelated children with a biliary atresia-like clinical disorder, each of whom developed liver failure requiring liver transplantation.

- by Fred Pluthero
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- Electron Microscopy, Gene expression, Pathophysiology, Liver

We carried out a retrospective review of infants with biliary atresia splenic malformation (BASM). We found that 56 infants (10.2%) met the criteria for inclusion from a series of 548 infants (from January 1977 to December 2004). Syndromic infants were more likely to be female (P ‫؍‬ .04) and to have a higher incidence of antenatal pathology (specifically maternal diabetes; 12.5% vs 1.2%; P < .0001). Situs inversus was noted in 21 (37%) and cardiac abnormalities in 25 (45%) infants. There were no differences in liver histology (eg, degree of liver fibrosis) or in the HLA genotype between BASM and nonsyndromic infants. Five-year and 10-year estimated native liver survival were 46% and 32%, respectively. There were 7 long-term survivors with their native liver and a follow-up of more than 10 years; all were anicteric. BASM is a distinct subgroup, with an implied onset during the embryological phase of organ development. (J Pediatr 2006;149:393-400)

- by Mark Davenport
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- Pediatrics, Treatment Outcome, Public Health, Spleen

Biliary atresia (BA) constitutes about one third of all neonatal cholestasis (NC) and the most common indication (up to 50%) of liver transplantation (LTx) in children. Despite extensive studies, its etiopathogenesis has not been clearly revealed. Treatment is primarily surgical based on reinstitution of bile flow by Kasai portoenterostomy, the success of which is largely dependent on the early diagnosis before 60 days of age. If portoenterostomy is not successful or not performed, LTx is the only life-saving alternative. Accurate diagnosis of BA, particularly distinguishing it from other causes of liver injury in the neonatal period, is challenging as there is a high degree of overlap in clinical, biochemical, imaging, and histological characteristics. There is no single preoperative investigation that enables the diagnosis of BA to be made with certainty. Liver biochemistry assessment, biliary radionuclide excretion scanning, magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), percutaneous needle liver biopsy, and laparoscopy can all be helpful, but their results are not individually diagnostic. The current review presents an overview of BA with emphasis on the recent diagnostic modalities.

- by Mostafa Sira
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- Biochemistry, Immunology, Ultrasound, Medicine

There are variety serious diseases of the children liver. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. Early diagnosis is the greatest help for these children. Without surgical treatment these patients die during the first two years of life from the progression of the liver disease, oesophageal bleeding or infection. In this article we would like to provide a brief overview of these diseases, describe the main diagnostic criteria, and we also want to turn the attention of pediatricians and neonatologists on diagnostic difficulties during the diagnostic period. Despite of the rarity of these diseases, biliary atresia should be on the list of differential diagnoses. Thanks to early diagnosis, performance of Kasai portoenterostomy and the possibility of liver transplantation was made significant progress in the treatment of children with those diseases in the last decade.

This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5-15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) postcholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)-(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.

- by Rajamani Krishna
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- Treatment Outcome, Adolescent, Child, Pediatric Surgery
- by Jorge Luiz Santos
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- Anthropometry, Pediatric Surgery, Infant, Liver Transplantation
- by Michael Narkewicz
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- Infant, Pediatric, Newborn Infant, Prognosis

Sixty-five children underwent liver transplantation (LTx) from March 1995 to December 2002. Cirrhosis due to biliary atresia was the main indication, and hepatic artery thrombosis (HAT) the most common vascular complication (n ϭ 5). Other vascular problems were portal vein thrombosis and stenosis. Another patient developed hepatomegaly and ascites due to a late stenosis of the left hepatic vein anastomosis. The two cases of venous stenosis were successfully treated by percutaneous angioplasty. One graft with HAT was saved, but four children died awaiting retransplant.

- by M. Zanotelli
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- Adolescent, Child, Infant, Liver Transplantation

Little is known about the safety of pediatric cardiac surgery in children with end-stage liver disease. We reviewed our experience with 4 patients with biliary atresia or Alagille's syndrome who underwent repair of ventricular septal defect and tricuspid regurgitation, atrioventricular canal, subaortic stenosis, or supravalvular aortic stenosis. One patient died on postoperative day 2. All other patients survived to discharge. At follow-up, 1 patient died at home awaiting liver transplantation and the remaining patients are doing well. One patient received a successful liver transplant. Pediatric cardiac surgery in children with end-stage liver disease can be done safely, albeit with a higher mortality.

- by Joel Hardin
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- Cardiac Surgery, Congenital Heart Defects, Aortic stenosis, Follow-up studies

Background: Biliary atresia (BA) is a progressive obliterative cholangiopathy leading to liver fibrosis and cirrhosis. The aspartate aminotransferase-to-platelet ratio index (APRi) has been used in other liver diseases and in older children with BA as a surrogate marker of liver fibrosis. The aim of this study was to calculate APRi at time of presentation and relate this to operative findings and early outcome. Methods: Prospective single surgeon cohort study of infants with BA (January 1999-December 2010). Initial APRi values were related to other biochemical indices and liver appearance at the time of Kasai portoenterostomy. Data are expressed as median (interquartile range). Non-parametric comparison was performed and a P-value of≤0.05 was regarded as significant. Results: Overall 260 infants were included in the study. Median APRi was 0.67 (0.43-1.12) at a median age of surgery of 58 (range 14-209) days. APRi correlated with age (r s =0.44; Pb0.0001), spleen size (r s =0.48; Pb0.0001) and bilirubin (r s =0.45; Pb0.0001). Liver assessment at operation was divided into cirrhosis [n=28 (10.8%)] or non-cirrhosis. Using a cutoff value of 1.22 [AUC 0.83 (95% CI 0.73-0.90)] showed a sensitivity of 75% and a specificity of 84% for macroscopic cirrhosis. Native liver survival was significantly different but improved only for those in the lowest APRi quartile (b0.43; Pb0.009). APRi values at presentation had no significant association with later development of significant oesophageal varices. Conclusion: APRi at the time of KP is a useful adjunct in evaluating severity of liver disease in BA at presentation.

- by Mark Davenport
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- Prospective studies, Child, Liver Cirrhosis, Pediatric Surgery
- by Jules Tolboom
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- Survival Analysis, Child, Portal hypertension, Liver

Inherited syndromes of intrahepatic cholestasis and biliary atresia are the most common causes of chronic liver disease and the prime indication for liver transplantation in children. Our understanding of the pathogenesis of these diseases has increased substantially by the discovery of genetic mutations in children with intrahepatic cholestasis and the findings that inflammatory circuits are operative at the time of

- by jorge noreno dos santos
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- Genetics, Mutation, Liver Transplantation, Trypsin

The purpose of this study is to classify biliary atresia (BA) with a ''cyst at porta'' according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a ''cyst at porta'' at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.

- by Niraj Kumari
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- Pediatric Surgery, Infant, Newborn Infant, Management Strategy

Objective: Connective tissue growth factor (CTGF) has been proposed to play a key role in the pathogenesis of hepatic fibrosis in biliary atresia (BA). The aim of the present study was to determine the single nucleotide polymorphism (SNP) in the promoter region of CTGF gene in a Thai population, and to investigate the possible role of CTGF promoter polymorphism in

- by Apiradee Theamboonlers and +1
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- Polymorphism, Statistical Analysis, Thailand, Disease susceptibility

Objective To determine the prognostic factors and optimal approaches to the diag- nosis and management of biliary atresia, the leading indication for liver transplantation in children. Study design A retrospective study was performed of all children who underwent hepatoportoenterostomy (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months was

- by Kathleen Schwarz
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- Pediatrics, Treatment Outcome, United States, Infant

To provide an updated review on extrahepatic biliary atresia, focusing mainly on its etiopathogenesis, diagnosis, treatment and prognosis. MEDLINE and PubMed databases were searched using the following keywords: biliary atresia, etiopathogenesis, diagnosis, treatment, prognosis, children. Extrahepatic biliary atresia is the main indication for liver transplantation among pediatric patients. As to its etiology, cytomegalovirus, reovirus and rotavirus have been widely investigated as possible triggers of the immunomediated obstruction of the biliary tree. The immune response, especially the predominant TH1 and interferon-gamma responses, genetic susceptibility and disorders related to the embryonic development of the biliary tree can play a role in the etiopathogenesis of extrahepatic biliary atresia. Yet today, portoenterostomy is the only available treatment, with better results when performed in the first 2 months of life. As to prognosis, all untreated children eventually die due ...

- by Cláudia Alexandra Pontes Ivantes
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- Forecasting, Child, Liver Transplantation, Prognosis

The major blood supply of extrahepatic biliary ductal system is by
cystic artery, which supplies to cystic duct, common hepatic duct
and the upper part of the common bile duct. The lower part of bile
duct is supplied by gastroduodenal artery. The present study is
aimed at determining the anatomical variations of blood supply of
extrahepatic biliary ductal system and its clinicalimplications. The
present study of the blood supply of extrahepatic biliary ductal
system has been conducted on 30 cadavers. After exposing the
abdominal cavity and removing peritoneal fat, extrahepatic biliary
system and its blood supply was carefully dissectedand relevant
photographs were taken. Special attention was given to the
relationship of the cystic artery to the Calot’s triangle. Along with
the arteries, the variations in the venous drainagewere also noted.
Major importance of knowledge of blood supply of extrahepatic
biliary ductal system may well lie in the understanding of the
etiology of postoperative bile duct strictures and in their
prevention. A clear understanding of the major arterial supply to
the extrahepatic bile ducts should be of assistance in biliary
reconstruction and treating hemobilia.

Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in the pediatric population. The outcomes of liver transplantation for biliary atresia, however, have not been formally examined on a national scale. The objective of this study was to identify pretransplant variables that predict patient survival after primary liver transplantation for biliary atresia. A cohort of 1,976 pediatric patients undergoing primary liver transplantation for biliary atresia between 1/1988 to 12/2003 was enrolled from the United Network for Organ Sharing database after excluding patients with a history of multiorgan transplant or previous liver transplant. Follow-up data up to 16 years post-OLT was available. The 5-and 10-year actuarial survival rates of patients that underwent liver transplantation for biliary atresia in the United States are 87.2% and 85.8%, respectively, and the 5-and 10-year graft actuarial survival rates are 76.2% and 72.7%, respectively. Early deaths (<90 days post-OLT) were more often caused by graft failure (P ‫؍‬ 0.01), whereas late deaths (>90 days post-OLT) were more often due to malignancy (P < 0.01). An analysis of outcomes over time demonstrated a decrease in post-OLT survival and an increase in the number of OLTs done for biliary atresia at an increasing number of centers. A multivariate analysis revealed that cadaveric partial/reduced liver grafts, a history of life support at the time of OLT, and decreased age were independent predictors of increased post-OLT mortality. In conclusion, OLT is an effective treatment for biliary atresia. Certain pretransplant variables may help predict patient survival following liver transplantation for biliary atresia. (Liver Transpl 2005;11:1193-1200.) Abbreviations: OLT, orthotopic liver transplantation; PELD, Pediatric End-Stage Liver Disease; UNOS, United Network for Organ Sharing.

- by Roshan Shrestha
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- Survival Analysis, Treatment Outcome, Adolescent, Transplantation

Biliary atresia is an obstructive cholangiopathy of unknown etiology. Although the adaptive immune system has been shown to regulate the obstruction of bile ducts in a rotavirus-induced mouse model, little is known about the virus-induced inflammatory response. Here, we hypothesized that cholangiocytes secrete chemoattractants in response to rotavirus. To test this hypothesis, we infected cholangiocyte and macrophage cell lines with rhesus rotavirus type A (RRV), quantified cytokines and chemokines and measured the migration of splenocytes. We also used PCR and immunostaining to search for new cellular targets of RRV in the liver. We found that RRV-infected cholangiocytes induced the mRNA expression for chemokines, but conditioned media failed to promote chemotaxis of splenocytes. Analyzing livers after viral challenge, we detected RRV in hepatic macrophages and demonstrated that media from RRV-infected macrophages have high concentrations of cytokines and chemokines and induced che...

- by Cláudia Alexandra Pontes Ivantes
- •
- Immunology, Cytokines, Macrophages, Cell line

Background and Aim: Congenital portosystemic veno-venous malformations are rare abnomalities that often remain undiagnosed. Typically they are classified by their anatomical characteristics according to Morgan (extrahepatic, Abernethy malformations type Ia,b and II) and Park (intrahepatic, types 1-4). However, their clinical presentation is less dependent on the anatomical type. Method: We reviewed the clinical characteristics of six cases drawn from our files (from 1970 to 2006). Results: One patient, a 25-year-old male, had extrahepatic shunting whereby the liver receives only arterial blood because the portal vein (PV) connects with the inferior caval vein (ICV) (Abernethy Ib); he presented with episodes of jaundice and pruritus. Three patients had extrahepatic shunting with patent intrahepatic portal veins, but with shunting of splenomesenterial blood towards the ICV (Abernethy II); these included a 66-year-old male with hepatic encephalopathy, a 17-year-old female with (porto?-)pulmonary hypertension without portal hypertension, and a 33-year-old female with epidsodes of acute pain secondary to spontaneous bleeding within a primary liver tumor. Two patients had intrahepatic shunting; these included an 8-year-old boy who was diagnosed incidentally during work-up for abnormal liver enzymes with a communication between right PV and ICV (Park type 1), and a 59-year-old male with multiple PV-ICV-shunts in several liver segments (Park, type 4) who presented with hepatic encephalopathy. Conclusion: Patients often present with signs of hepatic shunting (encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, and/or hypoglycemia) with relative sparing of the synthetic liver function in the absence of portal hypertension. Some shunts present with space-occupying lesions (focal nodular hyperplasia, hepatocellular carcinoma, nodular regenerative hyperplasia, etc.) or biliary atresia. Finally, some cases are detected incidentally.

- by Jacques Pirenne and +1
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- Adolescent, Child, Portal hypertension, Liver

Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.

- by Joanne Hojsak
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- Treatment Outcome, Congenital Heart Defects, Paediatric Anaesthesia, Infant

Hepatocyte &amp;amp;amp;amp;amp;amp;amp;amp;amp;quot;ballooning&amp;amp;amp;amp;amp;amp;amp;amp;amp;quot; is an often used but ill defined term in liver pathology to designate a special form of liver cell degeneration associated with cell swelling and enlargement found particularly in steatohepatitis. Alterations of the intermediate filament cytoskeleton of the hepatocyte may contribute to the pathogenesis of this microscopic change. Ballooning degeneration is considered a hallmark of steatohepatitis, but enlarged hepatocytes may also be observed in a variety of other acute and chronic liver diseases. The intermediate filament cytoskeleton was investigated using keratin 8 and 18 immunohistochemistry in liver diseases associated with enlarged or ballooned hepatocytes. Keratin 8/18 immunostaining was drastically reduced or lost in the cytoplasm of ballooned hepatocytes in alcoholic and non-alcoholic steatohepatitis, chronic cholestatic conditions, ischemia/reperfusion injury and in ballooned hepatocytes in chronic hepatitis C cases with concurrent steatohepatitis. In contrast, substantial decrease or loss of keratin 8/18 immunostaining was not noted in cases of acute hepatitis, giant cell hepatitis, chronic hepatitis B, or autoimmune hepatitis. Loss of keratin 8/18 immunostaining can serve as an objective marker of a specific type of ballooning degeneration of hepatocytes. Oxidative stress may be a common denominator in the pathogenesis of keratin filament alterations in these conditions.

- by Kurt Zatloukal
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- Immunohistochemistry, Oxidative Stress, Hepatology, Ubiquitin
- by D. Stamm
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- Fathers, Infant, Liver Transplantation, Parents
- by Mark Davenport
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- Endocrinology, Genetics, Cardiology, Hematology
- by Michel Lallier
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- Pediatrics, Surgery, Immunology, Treatment

The presence of ductal plate malformation (DPM+) on liver histology in children with biliary atresia (BA) is a marker of early intrauterine disease onset and an indication of an unfavorable prognosis. We studied the prognostic value of DPM in infants with BA after hepatoportoenterostomy (HPE). We reviewed 28 BA patients who underwent HPE in a single medical center. We examined the time of jaundice onset after delivery (conjugated hyperbilirubinemia): early onset (fetal phenotype with no jaundice-free interval) vs. late onset (perinatal phenotype with jaundice-free interval) and the presence or absence of DPM (DPM+ or DPM−) histopathology.

- by Tomislav Luetic and +2
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- Algorithms, Treatment Outcome, Infant, Follow-up studies

Background/Purpose: The aim of this study was to investigate the problems and the quality of life during and after pregnancy of the patients who had undergone Kasai operation and to find out a strategy for follow-up during the period of... more

- by Masaki Nio
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- Quality of life, Adolescent, Pregnancy, Child

The role of adjuvant steroid therapy in the postoperative management of patients with biliary atresia (BA) is unclear. To systematically review the literature and perform a meta-analysis to determine the efficacy of adjuvant steroid therapy post-Kasai portoenterostomy (KP) on BA outcome. A systematic review and meta-analysis of randomized trials and⁄or observational studies that examined the role of steroids on BA outcomes published between January 1969 and June 2010 was conducted. Studies were identified using the Medline, PubMed, EMBASE and Cochrane databases. Sixteen observational studies and one randomized controlled trial (RCT) were found. Four of the 16 observational studies (160 participants) and the RCT (73 participants) met the entry criteria and were eligible to be included in the analysis. There was no statistically significant difference in the effect of steroids either on normalizing serum bilirubin levels at six months (pooled OR 1.48 [95% CI 0.67 to 3.28]) or in delay...

- by Collin Barker and +1
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- Treatment Outcome, Liver Transplantation, Bilirubin, Clinical Sciences
- by Federico Sicca
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- Psychology, Pediatrics, Surgery, Behavior

The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) in children and analyze the survival and prognostic factors, and long-term outcome. We reviewed 332 OLTs performed in 280 children between the years 1986 and 2000. Univariate and multivariate analysis were performed on patient and graft survivals according to recipients' and donors' characteristics as well as intraoperative data. The long-term outcome among the 80 children living at 10 years after OLT was studied according to growth, immunosuppressive therapy, and liver and renal functions. Liver graft status was eventually documented by liver biopsy. Status of rehabilitation was assessed by reviewing school performance and employment. Overall patient survival rates at 1, 5, and 10 years were 85, 82, and 82%, respectively, and the corresponding overall graft survival rates were 77, 73, and 71%. In the multivariate analysis, we identified 4 independent prognostic factors: polysplenia syndrome (P ‫؍‬ .03), United Network for Organ Sharing (UNOS) status (P ‫؍‬ .05), donor's age (P ‫؍‬ .01), and perioperative surgical complications (P ‫؍‬ .03). At 10 years after transplant, 80 children were alive and had normal growth rates. Liver histology was abnormal in 73% of these long-term survivors, mainly due to chronic rejection and centrilobular fibrosis. A total of 63 of the 80 children attended normal school and in 55 children (69%) school performance was not delayed. In conclusion, we discovered that a good long-term survival could be achieved after liver transplantation for BA, with a 82% survival rate at 10 years with normal scholastic studies in the majority of recipients. (Liver Transpl 2005;11: 152-160.)

- by Dominique Debray
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- Treatment Outcome, Adolescent, Multivariate Analysis, Child

Biliary atresia (BA) is the end result of a destructive, rhosis. Until recently, BA was uniformly fatal; liver inflammatory process that affects intra-and extrahetransplantation has altered the inevitability of that patic bile ducts, leading to fibrosis and obliteration of outcome, but at a ''cost'' to the patient, who must face the biliary tract with the development of biliary cirrhothe consequences of living with a transplanted organ, sis. It is the commonest cause of chronic cholestasis in and to society in real dollars. Our understanding of the infants and children, and therefore is the most frequent etiology and pathogenesis of BA has remained virtually indication for liver transplantation in this age group. unchanged for two decades. This disorder is of interest The disease occurs worldwide, affecting an estimated 1 to all individuals involved in basic and clinical studies in 8,000 to 12,000 live births. At present, there is no speof diseases of the liver; the rapidly progressive ficific therapy for BA; however, sequential surgical therbroobliterative process may represent a paradigm for apy begins with creation of a hepatoportoenterostomy (HPE); in those with end-stage liver disease, liver trans-other forms of hepatobiliary injury, perhaps reflecting plantation is indicated. Since most candidates are young an inter-relationship between genetic predisposition children of small size, there is a shortage of size-matched and environmental exposure.

- by Frederick Suchy
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- Genetics, Hepatology, Liver Transplantation, Clinical Sciences

The aim of this study was to assess whether serum concentrations of hyaluronic acid (HA), measured at diagnosis is an early biochemical marker of prognosis in biliary atresia. Methods: Serum HA was measured at diagnosis using a radiometric assay in 84 infants with biliary atresia (BA), and related to outcome by 5 years of age. Results: Serum HA was higher in the 29 patients who died or required liver transplant by 5 years of age compared with the 56 who survived to 5 years without transplant (490 Ϯ 216 g/L v 262 Ϯ 163 g/L; P Ͻ .001, 95% confidence intervals of the difference 145 to 311 g/L). Conclusion: High serum concentrations of HA at diagnosis may help to identify at an early stage those patients with BA who have a poor prognosis and will require liver transplant by 5 years of age.

- by Giorgina Mieli-vergani
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- Medicine, Pediatric Surgery, Liver Transplantation, Pediatric

Objective: The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. Methods: Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). Results: KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection.

- by Paul Colombani
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- Surgery, Prediction, Treatment, Medicine

A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group of those who died or received liver transplantation. A score was calculated that assessed nine factors, including laboratory values and complications. The data were assessed at the time complications occurred. The scores were analysed by a trend analysis to see if serial scores predicted the evolution of liver disease. A receiver operating characteristic (ROC) curve was plotted to assess the optimal cut-point for the scoring system. There were 98 patients in the non-transplantation group and 35 in the failure group. The latter group had significantly higher post-operative bilirubin (9.3±7.2 mg/dl versus 3.5±3.1 mg/dl), ALT (136±89 U/l versus 92±88 U/l), prothrombin time, and incidence of cirrhosis, ascites, oesophageal varices, portal hypertension, cholangitis and sepsis than the non-transplantation group (P <0.05). A score of ‡8 had a high sensitivity (96.9%) and specificity (89.5%) for predicting the need for liver transplant. Conclusion: Based on easily available clinical information, our scoring system can predict which pa-tients with biliary atresia who have already undergone a Kasai procedure should be considered for liver transplantation.

- by Jin-cherng Sheu
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- Surgery, Treatment Outcome, Portal hypertension, Liver Cirrhosis
- by inci yildirim
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- Inborn errors of metabolism, TURKISH, Cytomegalovirus, Infant