Reply to Letter to the Editor

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

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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
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
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
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
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