Management of Biliary Atresia in France 1986 to 2015: Long-term Results

This study analyses the prognosis of biliary atresia (BA) in France since 1986, when both Kasai operation (KOp) and liver transplantation (LT) became widely available. Methods: The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed. Results: A total of 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin ≤20 μmol/L) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6–199) was stable over time. Survival with native liver after KOp was 41%, 35%, 26%, and 22% at 5, 10, 20, and 30 years, stable in the 4 cohorts. 25-year survival with native liver was 38%, 27%, 22%, and 19% in patients operated in the first, second, third month of life or later, respectively (P = 0.0001). Center caseloads had a significant impact on results in the 1986 to 1996 cohort only. 16%, 7%, 7%, and 8% of patients died without LT in the 4 cohorts (P = 0.0001). A total of 753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. Five-year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively (P 
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research

Related Links:

Abstract Tamgal J, Damrongmanee A, Khorana J, Tepmalai K, Ukarapol N. Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia. Turk J Pediatr 2019; 61: 7-12. The aim of this study was to assess the probability of survival with native liver (SNL) and the rate of esophageal variceal bleeding (EVB) as well as their potential risk factors, in patients diagnosed with Biliary Atresia (BA), who underwent the hepaticoportoenterostomy (HPE) by retrospectively reviewing medical records between 2007 and 2016. The subjects were classified as poor outcomes if they ...
Source: The Turkish Journal of Pediatrics - Category: Pediatrics Authors: Tags: Turk J Pediatr Source Type: research
Abstract The disease phenotype in biliary atresia (BA) is caused by a fibro-inflammatory process leading to destruction of cholangiocytes, obstruction of ductular pathways and eventual progression to liver cirrhosis. The first line of management is a Kasai portoenterostomy (KPE) followed by liver transplantation (LT) in some children. Several factors have been postulated to affect the outcome of KPE and/or the subsequent progression of liver disease. However, no biomarkers have been identified in the liver for BA. We aimed to address this deficit. Whole transcriptome mRNA sequencing was performed for 29 samples (2...
Source: Molecular Biology Reports - Category: Molecular Biology Authors: Tags: Mol Biol Rep Source Type: research
Conclusion: BA is an uncommon cause of infantile cholestasis in Saudi Arabia. Our study provides a snapshot of the epidemiology of BA in Saudi Arabia that is characterized by late referral to pediatric gastroenterologists and poor outcome without LT.
Source: The Saudi Journal of Gastroenterology - Category: Gastroenterology Authors: Source Type: research
We report the case of a 10-day old boy with biliary atresia (BA) who was delivered by caesarean section at 33 weeks 4 days of gestation (birth weight, 2135 g). At birth, his direct bilirubin level was high, and stools were light yellow. Abdominal ultrasonography showed no triangular cord sign, and duodenal fluid examination showed no bile. We performed a Kasai procedure (KP) 27 days after birth. His liver biopsy showed intrahepatic bile duct reduction. No bile excretion occurred postoperatively. We performed a redo KP 83 days after birth. Postoperative bile excretion was good, and his jaundice promptly resolved. The pati...
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
Discussion The liver is one of the largest organs in the body, weighing just over 3 pounds in an adult. It is found in the upper right abdomen, under the right dome of the diaphragm. Grossly, it has asymmetric lobes with the right being larger than the left. The lobes are separated by a fibrous connective tissue band that also anchors the liver in the abdominal cavity. The gallbladder is located on the inferior surface of the liver and stores bile, which is then released into the duodenum. Microscopically, the liver cells are arranged in lobules with canals carrying blood vessels and bile ducts. At any moment about 10-13% ...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Biliary Atresia (BA), is an idiopathic neonatal cholangiopathy, characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts [1]. The surgical procedure, Kasai portoenterostomy (KP), aims to restore bile flow, and alleviate jaundice. Liver transplantation (LT) is performed in cases where KP is unable to salvage the native liver, with complications including jaundice, cholangitis, portal hypertension and/or synthetic failure [2]. Five and 10 year UK native liver survival (NLS) rates in BA, have been documented as 46% and 40% respectively [3].
Source: Journal of Hepatology - Category: Gastroenterology Authors: Source Type: research
Biliary atresia (BA) is an idiopathic neonatal cholangiopathy characterized by progressive inflammatory obliteration of the intrahepatic or extrahepatic bile ducts.1 The surgical procedure, Kasai portoenterostomy (KP), aims to restore bile flow, and alleviate jaundice. Liver transplantation (LT) is performed in cases where KP is unable to salvage the native liver, with complications including jaundice, cholangitis, portal hypertension (PHT) and/or synthetic failure.2 Five and 10-year UK native liver survival (NLS) rates in BA have been documented as 46% and 40%, respectively.
Source: Journal of Hepatology - Category: Gastroenterology Authors: Tags: Research Article Source Type: research
Abstract OBJECTIVES: Liver transplant has been used as a curative approach for children with end-stage liver diseases. Here, we describe the underlying causes for pediatric liver transplant performed at the Shiraz Organ Transplantation Center, Nemazee Hospital, Shiraz, Iran. MATERIALS AND METHODS: In this cross-sectional des-criptive study, children
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - Category: Transplant Surgery Authors: Tags: Exp Clin Transplant Source Type: research
We report a patient with recurrent cholangitis following HPE who developed multiple large biliary cystic lesions. Percutaneous drainage resulted in resolution of jaundice and cholangitis but capping of drains resulted in recurrence of symptoms. Therefore, internal drainage of the bile lakes was accomplished using cystjejunostomy to the roux limb of the previous portoenterostomy. At 18 months of follow-up, the patient has remained jaundice free with only one recurrent cholangitis episode which occurred after stopping her antibiotic prophylaxis. Although this patient will likely require liver transplantation in the next few ...
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
We report a patient with recurrent cholangitis following HPE who developed multiple large biliary cystic lesions. Percutaneous drainage resulted in resolution of jaundice and cholangitis but capping of drains resulted in recurrence of symptoms. Therefore, internal drainage of the bile lakes was accomplished using cystjejunostomy to the roux limb of the previous portoenterostomy. At 18 months of follow-up, the patient has remained jaundice free with only one recurrent cholangitis episode which occurred after stopping her antibiotic prophylaxis. Although this patient will likely require liver transplantation in the next few ...
Source: Journal of Pediatric Surgery Case Reports - Category: Surgery Source Type: research
More News: Atresia | Bilirubin | France Health | Gastroenterology | Jaundice | Liver | Liver Transplant | Nutrition | Pediatrics | Study | Transplants | Urology & Nephrology