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Current management of biliary atresia
Deirdre A Kelly1, Mark Davenport
1The Liver Unit, Birmingham Children's Hospital NHS Trust, Birmingham, UK. Deirdre.Kelly@bch.nhs.uk
Insights
Extra-hepatic biliary atresia (EHBA) is a rare condition causing prolonged jaundice in newborns. Early diagnosis and surgery, alongside medical management, significantly improve survival rates and quality of life for affected infants.
Area of Science:
- Pediatric Surgery
- Hepatology
- Neonatology
Background:
- Extra-hepatic biliary atresia (EHBA) affects approximately 1 in 15,000 live births in the UK.
- Presentation typically involves prolonged jaundice in term infants, indicative of obstructive jaundice.
Purpose of the Study:
- To review the current management strategies for EHBA.
- To highlight the impact of early diagnosis and specialized surgical centers on patient outcomes.
Main Methods:
- Review of current medical and surgical management protocols for EHBA.
- Analysis of survival and quality of life data following interventions.
Main Results:
- Improved public and professional education has led to earlier diagnosis and surgical intervention before 8 weeks of age.
- Centralization of EHBA surgery to specialized UK centers has increased survival rates to over 90%.
- Over 50% of infants undergoing Kasai portoenterostomy achieve jaundice clearance and an 80% chance of reaching adolescence without liver transplantation.
Conclusions:
- Early diagnosis and surgical intervention, complemented by medical management, are crucial for improving outcomes in EHBA.
- Liver transplantation offers a 90% chance of a normal life for children with intractable complications.
- Specialized care centers have significantly enhanced survival and quality of life for infants with this potentially fatal disease.
Abstract:
Extra-hepatic biliary atresia occurs in approximately 1:15,000 live births leading to about 50 new cases/year in the UK. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice. Management has been improved by public and professional education to encourage early referral and diagnosis to facilitate initial surgery before 8 weeks of age. Surgical management is complementary and includes an attempt to restore biliary flow (the Kasai portoenterostomy) and liver transplantation if necessary. Medical management consists of antibiotics, ursodeoxycholic acid to encourage bile flow, fat soluble vitamin supplementation and nutritional support. Centralising surgery to specialised centres has improved survival of this potentially fatal disease to over 90% in the UK. Over half of infants undergoing portoenterostomy will clear the jaundice and have a greater than 80% chance of a good quality of life, reaching adolescence without transplantation. For those children developing intractable complications of cirrhosis and portal hypertension, liver transplantation provides a 90% chance of achieving normal life.
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