Dr Kam Choy Chen

Designation

Paediatric Gastroenterologist

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Institution

Hospital Tunku Azizah Kuala Lumpur

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Qualification

MBBS (UM)

MRCPCH (UK)

 

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Special field of interest

Paediatric Gastroenterology, Hepatology and Nutrition

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Abstract

Title:

Cholestatic Liver Disease in Children

Body:

Cholestatic liver disease is a significant cause of morbidity and mortality. In children, the causes of cholestatic liver diseases are multiple and varies according to age, it can be congenital or acquired, including biliary duct obstruction, infection, drug-induced, autoimmune and metabolic liver diseases. Cholestasis is impairment in bile flow resulting in the accumulation of the component of bile (bile acids, bilirubin, cholesterol, phospholipid). Persistent cholestasis will result in clinical symptoms and laboratory abnormalities. Infection and drug-induced cholestasis usually are self-limiting and required short term support, patients will recover fully without any clinical sequelae most of the time. However diseases such as biliary atresia, metabolic liver diseases (progressive familiar intrahepatic cholestasis disease, bile acid synthetic disorders), primary sclerosing cholangitis will required long term medical support or even liver transplantation ultimately. Medical management includes supportive care for complications of chronic cholestasis including faltering growth and malnutrition, pruritus, and portal hypertension (gastrointestinal bleeding, ascites). There are limited effective interventions to prevent progressive liver damage, ultimately patient will require liver transplantation.