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Liver and biliary diseases in infancy and childhood 

Liver and biliary diseases in infancy and childhood
Chapter:
Liver and biliary diseases in infancy and childhood
Author(s):

Richard J. Thompson

DOI:
10.1093/med/9780198746690.003.0333
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date: 07 March 2021

Most liver diseases that occur in adults also occur in children, but some present almost exclusively in early childhood. Neonatal jaundice is common and usually short-lived. Onset before 24 h, or continuation beyond 2 weeks, strongly suggests an underlying pathology, when biliary atresia is the most common cause, with liver transplantation allowing some patients to survive to adolescence and adulthood. Some genetic causes of cholestasis (e.g. MDR3 deficiency) can present in adults. A long list of metabolic disorders present with evidence of liver involvement, with later manifesting diseases including those with accumulation of material in the liver. Liver transplantation is an excellent treatment for many of these disorders, and a growing number of metabolic disorders that do not cause liver disease per se are now being successfully managed through liver replacement, with patients surviving into adult life.

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