Biliary atresia

Summary Biliary atresia is a rare disease of infancy, which has changed within 30 years from being fatal to being a disorder for which effective palliative surgery or curative liver transplantation, or both, are available. Good outcomes for infants depend on early referral and timely Kasai portoente...

Full description

Saved in:
Bibliographic Details
Published in:The Lancet (British edition) 2009-11, Vol.374 (9702), p.1704-1713
Main Authors: Hartley, Jane L, Dr, Davenport, Mark, Prof, Kelly, Deirdre A, Prof
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - epidemiology
Abnormalities, Multiple - genetics
Alcohol use
Algorithms
Biliary Atresia - diagnosis
Biliary Atresia - epidemiology
Biliary Atresia - etiology
Biliary Atresia - surgery
Biological and medical sciences
Changes
Children & youth
Data collection
Diagnosis, Differential
Disease Progression
Early Diagnosis
General aspects
Genes
Genetic Predisposition to Disease - epidemiology
Genetic Predisposition to Disease - genetics
Humans
Infant, Newborn
Infants
Internal Medicine
Jaundice, Neonatal - etiology
Liver
Liver cirrhosis
Liver diseases
Liver Transplantation
Medical sciences
Neonatal Screening
Palliative Care
Patient Selection
Portoenterostomy, Hepatic
Prognosis
Quality of life
Rare Diseases
Referral and Consultation
Risk Factors
Studies
Transplants & implants
Treatment Outcome
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Summary Biliary atresia is a rare disease of infancy, which has changed within 30 years from being fatal to being a disorder for which effective palliative surgery or curative liver transplantation, or both, are available. Good outcomes for infants depend on early referral and timely Kasai portoenterostomy, and thus a high index of suspicion is needed for investigation of infants with persistent jaundice. In centres with much experience of treating this disorder, up to 60% of children will achieve biliary drainage after Kasai portoenterostomy and will have serum bilirubin within the normal range within 6 months. 80% of children who attain satisfactory biliary drainage will reach adolescence with a good quality of life without undergoing liver transplantation. Although much is known about management of biliary atresia, many aspects are poorly understood, including its pathogenesis. Several hypotheses exist, implicating genetic predisposition and dysregulation of immunity, but the cause is probably multifactorial, with obliterative extrahepatic cholangiopathy as the common endpoint. Researchers are focused on identification of relevant genetic and immune factors and understanding serum and hepatic factors that drive liver fibrosis after Kasai portoenterostomy. These factors might become therapeutic targets to halt the inevitable development of cirrhosis and need for liver transplantation.
ISSN:0140-6736
1474-547X
DOI:10.1016/S0140-6736(09)60946-6