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Impact of Hepatoblastoma on Infectious Complications Following Pediatric Liver Transplantation.
Pediatr Transplant
February 2025
Division of Infectious Diseases, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA.
Background: Liver transplantation is the standard therapy for end-stage liver disease in pediatric patients with biliary atresia (BA), congenital and metabolic conditions, and for an unresectable malignant tumor like hepatoblastoma (HB). BA is the leading indication for pediatric liver transplantation, while HB is the most common childhood liver cancer. Despite improved outcomes through advanced surgical techniques and novel immunosuppression, pediatric liver transplantation (pLT) is complicated by post-transplant infections.
View Article and Find Full Text PDFProgressive Familial Intrahepatic Cholestasis Type 2 in an Infant: Diagnostic Challenges and Multidisciplinary Management.
Cureus
December 2024
Internal Medicine, Nishtar Medical University, Multan, PAK.
Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rare genetic disorder characterized by severe intrahepatic cholestasis, which often manifests in infancy with progressive liver dysfunction. We present the case of a 3-month-old infant with a one-month history of jaundice, vomiting, and bloody stools, presenting a unique set of diagnostic challenges. Initial clinical and laboratory findings indicated significant liver dysfunction, prompting further imaging and genetic analysis.
View Article and Find Full Text PDFPotential therapeutic effect of dimethyl fumarate on Treg/Th17 cell imbalance in biliary atresia.
Clin Immunol
January 2025
Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai Key Laboratory of Birth Defect, and Key Laboratory of Neonatal Disease, Ministry of Health, Shanghai 201102, China. Electronic address:
The imbalance between Tregs and proinflammatory Th17 cells in children with biliary atresia (BA) causes immune damage to cholangiocytes. Dimethyl fumarate (DMF), an immunomodulatory drug, regulates the Treg/Th17 balance in diseases like multiple sclerosis (MS). This study explores DMF's effect on Treg/Th17 balance in BA and its potential mechanism.
View Article and Find Full Text PDFPost-Kasai cholangitis evaluation and management strategies: Review of the literature with insights from the Swiss Biliary Atresia Registry.
Semin Pediatr Surg
January 2025
Swiss Pediatric Liver Center, Geneva University Hospitals, Geneva, Switzerland; Division of Child and Adolescent Surgery, Department of Pediatrics, Gynecology, and Obstetrics, Geneva University Hospitals, University of Geneva, Geneva, Switzerland.
Cholangitis, defined as the inflammation of the bile ducts, is the most frequent complication after Kasai hepatoportoenterostomy in patients with biliary atresia (BA). This review seeks to provide a comprehensive synthesis of current knowledge on diagnosing and managing BA-associated cholangitis while identifying gaps in the existing literature. A scoping literature review was conducted to gather global insights into the definition, evaluation, and management of post-Kasai cholangitis, illustrated through data from the Swiss Biliary Atresia Registry (SBAR).
View Article and Find Full Text PDFGenetics of biliary atresia: Approaches, pathological insights and challenges.
Semin Pediatr Surg
January 2025
Department of Surgery, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong SAR, China; Dr Li Dak-Sum Research Centre, The University of Hong Kong - Karolinska Institutet Collaboration in Regenerative Medicine, Hong Kong, China. Electronic address:
Biliary atresia (BA) is a severe neonatal cholestatic disorder marked by fibro-obliteration of the extrahepatic and intrahepatic bile ducts. It is the most common cause of pediatric end-stage liver disease and the leading indication for liver transplantation in children. There is significant heterogeneity in the etiology, involving various genetic and environmental factors such as viral infection, immune dysregulation and genetic predisposition to defective hepatobiliary development.
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