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Biliary atresia (BA) is an important public health issue because of its severity, remaining the the most common indication for paediatric liver transplantation, and the time-sensitive nature of its management. The aetiology of BA remains elusive, but it develops prenatally and progresses rapidly after birth. It presents with neonatal jaundice, often as prolongation of physiological jaundice. Affected infants often appear superficially well with normal early growth, although hepatomegaly is common if actively sought. Pale stools are usually present by 2 weeks and are the rule by 1 month of age.
Treatment is with a timely Kasai portoenterostomy which, if successful in clearing jaundice, transforms the outlook and postpones the need for urgent liver transplantation, often for many years. The traditional target was to ensure the Kasai was carried out before 60 days old, but it is now evident that it should be done as early as possible to maximise chances of success.1 Unfortunately, despite campaigns to raise professional and community awareness of the importance of investigating prolonged neonatal jaundice, the age at surgery for BA has not fallen in the USA over the last 20 years, or in the UK until very recently. Currently, more than half require urgent liver transplant in their first 2 years. The major cause for delay continues to be failure to recognise and/or appreciate the significance of prolonged jaundice. Some delay may reflect health infrastructure as infants are not routinely examined between the …
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; externally peer reviewed.