This syndrome is of unknown aetiology but has been associated with aspirin consumption in childhood. It consists of an acute encephalopathy with cerebral oedema and diffuse microvesicular fatty infiltration of the liver. It occurs mainly in children and the mortality rate is 50%. Benign recurrent intrahepatic cholestasis This condition often presents in children and consists of bouts of cholestatic jaundice with pruritus. Jaundice may last for weeks or months. There is no treatment; cholestyramine 12 g daily may help relieve the itching.
Indian childhood cirrhosis
This condition of children is seen in the Indian subcontinent. The cause is unknown. Eventually there is development of a micronodular cirrhosis with excess copper in the liver.
This disease mainly affects the lung and pancreas. but patients can develop fatty liver. cholestasis and cirrhosis. The aetiology of the liver involvement is unclear.