Cholestatic disease in childhood.

Editor(s): International committee of paediatrician Annales Nestlé Vol.66 / 3,  2008


  • Editorial
  • Diagnosis of Neonatal Cholestasis
  • Familial Cholestatic Syndromes
  • Treatments in Chronic Cholestasis in Children
  • Nutritional Management of Cholestatic Syndromes in Childhood

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  • Editorial

    Author(s): The Editorial Committee

    Cholestasis may be defined physiologically as a reduction in canalicular bile flow. Although it is clinically manifested as jaundice due to conjugated hyperbilirubinemia, its main consequences are due, on the one hand, to the cellular toxicity of accumulated bile acids, ultimately leading to biliary cirrhosis and, on the other hand, to decreased intraluminal bile acid concentration responsible for fat and fat-soluble vitamin malabsorption. Because the hepatobiliary excretory system is functionally immature at birth, the neonate has a particular tendency to develop cholestasis. Indeed, cholestatic diseases have been central in the development of pediatric hepatology. Around biliary atresia, the main cause of cholestasis in infants, multiple other causes have been recognized, related either to anatomic or genetic anomalies or to infectious or toxic agents, with very different prognoses.

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