Liver (Hepatology and Liver Transplant)

What is liver disease?

Liver problems in children include: 

  • Biliary atresia
  • Autoimmune liver diseases
  • Chronic hepatitis B and C
  • Fatty liver disease

Biliary atresia (BA) is the most common cause of cholestasis in newborns and is the most common indication for liver transplantation in childhood. It is a congenital condition of unknown cause, where the bile ducts draining bile from the liver are not properly developed, resulting in the accumulation of toxic bile which causes severe liver damage. If diagnosed early enough, an operation can help to restore bile flow.

A range of other liver conditions managed by the Hepatology Service include autoimmune liver diseases (autoimmune hepatitis, sclerosing cholangitis), metabolic liver diseases (alpha-1-antitrypsin deficiency, Wilson’s disease, glycogen storage diseases, hereditary fructose intolerance, tyrosinaemia, Niemann-Pick c disease, and inborn errors of metabolism which may require liver transplantation to correct a missing biochemical enzyme). Congenital conditions such as Alagille’s syndrome are commonly seen, while further advances in genetics has seen the identification of a number of rare cholestatic disorders of infancy - Progressive Familial Intrahepatic Cholestasis types 1, 2 and 3.

Fatty liver disease is rapidly becoming a major health crisis due to the increasing epidemics of obesity, type 2 diabetes and metabolic syndrome. Increasingly, children are being seen in the Hepatology Clinic for management of this condition, which in adult patients is becoming a leading indication for liver transplantation.

Although most liver conditions do not have a cure apart from transplantation, they do require careful and regular follow up to manage potential complications. Attention to issues such as portal hypertension (gastrointestinal bleeding, ascites), infection, nutrition and growth/development are important factors in the management of children with chronic liver disease. This requires a multidisciplinary team of nurses/CNC’s, dietitians, social workers, psychologists and pharmacists.

How do we help children who have a liver disease?

The Sydney Children's Hospital's Network provides a complete range of services to children in NSW who have a liver disease, and to some interstate children requiring liver transplant.

At SCH, the Gastroenterology team provide a Hepatology service that includes management of all liver illnesses, up until the need for transplantation arises. Hepatitis B and C patients are looked after by a multi-disciplinary team with the Department of Infectious Diseases. Sydney Children's Hospital provides services to liver patients through its Department of Gastroenterology. See Gastroenterology and hepatology (liver) services at Sydney Children's Hospital. 

CHW provides a nationally funded Liver Transplant service with an interstate referral base and quaternary service in management of complex liver disease in children. CHW has been at the forefront of establishing a state-wide service for children with chronic hepatitis B and C. Under the auspices of the Agency for Clinical Integration (ACI) and in conjunction with gastroenterologists at SCH and JHH plus a wide range of health care providers, guidelines have been developed for screening, identification and management of these affected children. CHW currently manages approximately 100 cases of chronic hepatitis B and C per annum. Around 20 patients are seen each fortnight at the CHW Hepatitis Clinic.

The CHW Liver Transplant Service is a Nationally Funded Centre (NFC). The Service provides all children’s liver transplants in NSW as well as those for ACT and Western Australia. Children from other states are able to access this service as required according to patient/family and referring physician preference; other paediatric transplant services are provided in Victoria and Queensland. Up until 5 years ago the majority of children from South Australia were transplanted at CHW, and on-going care of these children continues in collaboration with local physicians.CHW has the largest paediatric liver transplant service in Australia. As such it has also developed a tertiary and quaternary hepatology service for paediatric patients with a wide range of liver disorders, many of whom don’t require liver transplantation.