Translational Research in Biliary Atresia: News from Mice and Men

This article presents a review of the literature on available BA animal models and gives a glimpse of future developments. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text
Source: European Journal of Pediatric Surgery - Category: Surgery Authors: Tags: Review Article Source Type: research

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Biliary atresia (BA) is a fatal condition resulting in the lack of effective biliary drainage leading invariably to liver failure and cirrhosis within a year, and it is often lethal within a few months in the absence of corrective surgery or liver transplantation. In fact, BA is the most common indication for pediatric liver transplantation. Herein, we present a rare case of unexpected infant death due to BA diagnosed only postmortem in a context of child neglect and carelessness on the part of the parents. It emerged from the clinical history that after a few months, the parents no longer took their daughter to any medic...
Source: The American Journal of Forensic Medicine and Pathology - Category: Forensic Medicine Tags: Original Articles Source Type: research
s M Abstract In contrast to gallstones, which are increasingly diagnosed in children and adolescents, congenital or perinatally acquired malformations of the biliary tree are rare entities. The surgical treatment of these diseases frequently represents a technical challenge. For children with biliary atresia Kasai portoenterostomy is a therapeutic option in the first weeks of life. Most affected children, however, require a liver transplantation before they enter adulthood. Segmental dilatations of the bile duct carry the risk of ascending infections and malignant transformation. This requires early...
Source: Der Chirurg - Category: Surgery Authors: Tags: Chirurg Source Type: research
We appreciate the letter from Dr. Davenport and Dr. Macdonald from Kings College Hospital regarding our single center 26-year experience and long-term outcomes for patients who underwent HPE for biliary atresia. The aim of our study was to determine perioperative risk factors associated with progression to liver transplantation following HPE [1]. Our results, similar to those of Shneider [2], showed total bilirubin at 3  months following HPE was the strongest predictor of need for transplantation.
Source: Journal of Pediatric Surgery - Category: Surgery Authors: Source Type: research
CONCLUSION: LDLT is a safe procedure in a selected group of BA patients with PH, however, further long-term clinical investigations and mechanical researches are needed. PMID: 31588186 [PubMed - in process]
Source: International Journal of Medical Sciences - Category: Biomedical Science Tags: Int J Med Sci Source Type: research
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
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, ...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
Biliary atresia (BA) is the most common reason for pediatric liver transplant. BA's varied presentation, natural history, and treatment with the Kasai portoenterostomy have been well described; however, when BA starts relative to birth has not been clearly defined. In this review, we discuss laboratory, imaging, and clinical data which suggest that most if not all forms of BA may start before birth. This early onset has implications in terms of delivering treatments earlier and identifying possible factors underlying BA's etiology.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Review Article Source Type: research
ConclusionsThis is the first report on the relationships between morphometrically analyzed PV branches and the postoperative course in BA patients. The portal venous system is involved as the primary lesion in BA.
Source: Pediatric Surgery International - Category: Surgery Source Type: research
ConclusionNon-KP initial procedures were limited. Clear criteria regarding the timing of diagnosis or patient condition could not be determined. Reasons for not initially selecting KP varied, but late diagnosis was predominant. Further study is needed to create guidelines for the initial treatment of BA patients.
Source: Pediatric Surgery International - Category: Surgery Source Type: research
This study used computational fluid dynamics to analyze portal vein hemodynamics in the management of post-transplant portal vein stenosis. To perform computational fluid dynamics analyses, three-dimensional portal vein model was created using computed tomographic DICOM data. The inlet flow condition was set according the flow velocity measured on Doppler ultrasonography. Finally, portal vein flow was simulated on a fluid analysis software (Software Cradle, Japan).ResultsAn 18-month-old girl underwent liver transplantation using a left lateral graft for biliary atresia. At the post-transplant 1-week evaluation, the computa...
Source: Journal of Gastrointestinal Surgery - Category: Surgery Source Type: research
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