Following Kasai hepatic portoenterostomy (HPE), most patients with biliary atresia will eventually require liver transplantation due to progressive cirrhosis and liver failure. Preventing liver transplantation, or even delaying eventual liver transplantation, is the key to improving long-term outcomes. This review first examines the commonly used adjuvant therapies in post-HPE biliary atresia and the strength of the evidence supporting these therapies. Next, it examines the evolving frontiers of management through a comprehensive evaluation of both recently completed and ongoing clinical trials in biliary atresia. Promising therapies used in other cholestatic liver diseases with potential benefit in biliary atresia are discussed. Improving post-HPE management is critical to prevent complications, delay liver transplantation, and ultimately improve the long-term survival of patients with biliary atresia.
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http://dx.doi.org/10.3389/fped.2022.1007813 | DOI Listing |
Hepatol Commun
November 2024
Paediatric Liver, GI and Nutrition Centre and Mowatlabs, King's College Hospital, London, UK.
Background: The Kasai portoenterostomy (KPE) aims to re-establish bile flow in biliary atresia (BA); however, BA remains the commonest indication for liver transplantation in pediatrics. Gut microbiota-host interplay is increasingly associated with outcomes in chronic liver disease. This study characterized fecal microbiota and fatty acid metabolites in BA.
View Article and Find Full Text PDFCureus
December 2024
Department of Pharmacy, Mesco College of Pharmacy, Hyderabad, IND.
Biliary atresia (BA) is a serious hepatobiliary disorder that occurs due to progressive inflammation and scarring obstruction in the bile ducts, posing a threat to life. This condition usually appears in infants, and timely identification is fundamental for a better prognosis. If left untreated, individuals will inevitably experience liver damage and mortality.
View Article and Find Full Text PDFEur J Pediatr Surg
January 2025
Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, JODHPUR, India.
Introduction Indocyanine Green (ICG) fluorescence guided surgery (FGS) is reported extensively in adult operations, but its safety and applications in Pediatric populations remain to be comprehensively understood. The dose, administration protocols and intraoperative imaging benefits in Pediatric hepatobiliary operations are not clear. Objectives To identify the feasibility and applications of ICG Fluorescence Guided Surgery (FGS) in hepatobiliary surgeries (for biliary atresia, choledochal cyst, and cholelithiasis) in children.
View Article and Find Full Text PDFNeurosurg Focus
January 2025
1Department of Pediatric Neurosurgery, Hôpital Necker - Enfants Malades, Assistance Publique-Hôpitaux de Paris.
Objective: Craniosynostoses are an underrecognized cause of intracranial hypertension (ICH), especially when associated with congenital syndromes. Alagille syndrome (ALGS) is a multisystem disorder with typical facial features and hepatobiliary, cardiac, vascular, skeletal, and ocular manifestations. The occurrence of craniosynostosis in ALGS is rare and can be associated with chronic ICH, requiring craniofacial surgery to increase the intracranial volume.
View Article and Find Full Text PDFMymensingh Med J
January 2025
Dr Subir Ananda Biswas, Resident, Department of Paediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh; E-mail:
Cholestatic jaundice is a potentially serious condition that requires early diagnosis for proper management. Fat-soluble vitamin (FSV) deficiency develops as a consequence of cholestasis. Vitamin D deficiency is common and remains a challenge in patients with cholestasis.
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