Quantitative Liver Fibrosis Using Collagen Hybridizing Peptide to Predict Native Liver Survival in Biliary Atresia: A Pilot Study

Conclusion: Our results suggest that quantitative assessment of fibrosis at the time of KPE holds promise as an earlier predictor of LTx requirement in BA. A larger study is justified to assess quantitative fibrosis as a BA prognostic tool.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research

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Objectives: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of “normal GGT” BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. Methods: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were ca...
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
Biliary atresia (BA), which is the most important cause of liver cirrhosis in children, is characterized by progressive fibro-obliterative inflammation involving the extra- and intrahepatic bile ducts in infancy.1 A successful Kasai portoenterostomy procedure establishes bile drainage and may  prevent hepatic fibrogenesis and the need for liver transplantation. However, the procedure is unsuccessful in a significant portion of patients, who have persistent jaundice and liver dysfunction. Intestinal bile acids modulate the gut microbiota and may have a role in disease progression in BA.
Source: Pediatrics and Neonatology - Category: Perinatology & Neonatology Authors: Tags: Short Communication Source Type: research
To identify changes in demographics, outcomes, and risk factors for patient and graft loss in patients with biliary atresia undergoing liver transplantation since Pediatric End-Stage Liver Disease implementation (2002).
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Original Articles Source Type: research
AbstractIntroductionTreatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait ‐list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients.MethodsUsing the Nationwide Inpatient Sample database, we identified all adults aged at least 18 years old who underwent heart transplantation across U.S. hospitals from 2004 to 2014. We t...
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: ORIGINAL ARTICLE Source Type: research
AbstractBackgroundVery few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis.AimsTo evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia.MethodsAll children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA  =...
Source: Digestive Diseases and Sciences - Category: Gastroenterology Source Type: research
Conclusions. VG interposition for PV reconstruction in LDLT appears to be a feasible alternative option with acceptable outcomes for patients with BA. Achieving sufficient PV flow is essential to preventing PVCs after LDLT.
Source: Transplantation - Category: Transplant Surgery Tags: Original Clinical Science—Liver Source Type: research
Danielle Roscoe, 35, saved her son Otis by donating part of her liver. Otis had biliary atresia and doctors in Leeds warned the baby boy would die without a transplant.
Source: the Mail online | Health - Category: Consumer Health News Source Type: news
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 et.al [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
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