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Introduction Introduction
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Neonatal cholestasis Neonatal cholestasis
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Biliary atresia Biliary atresia
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α1-Antitrypsin deficiency α1-Antitrypsin deficiency
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Genetic cholestasis Genetic cholestasis
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Syndromic cholestasis Syndromic cholestasis
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Nonsyndromic cholestasis Nonsyndromic cholestasis
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Management of cholestasis Management of cholestasis
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Neonatal liver failure Neonatal liver failure
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Gestational alloimmune liver disease Gestational alloimmune liver disease
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Haemophagocytic lymphohistiocytosis Haemophagocytic lymphohistiocytosis
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Metabolic liver disease Metabolic liver disease
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Galactosaemia Galactosaemia
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Tyrosinaemia type 1 Tyrosinaemia type 1
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Glycogen storage diseases Glycogen storage diseases
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Mitochondrial disease Mitochondrial disease
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Liver disease in older children Liver disease in older children
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Autoimmune liver disease Autoimmune liver disease
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Cystic fibrosis Cystic fibrosis
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Wilson disease Wilson disease
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Transition to adulthood Transition to adulthood
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Further reading Further reading
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15.24.7 Liver and biliary diseases in infancy and childhood
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Published:January 2020
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Abstract
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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