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Liver disease
Neonatal conditions
Biliary Atresia
Biliary atresia is unique to babies, affecting about 1 in 16,000 live births. In this condition, bile ducts within and outside the liver are progressively damaged by inflammation, and become blocked. The condition is suspected in babies who fail to clear the jaundice after two weeks of age and also have pale or white stools. Special tests are required to establish the diagnosis and an early referral to one of the liver specialist centres in the country is recommended, as the outcome of the surgery depends on the expertise of the surgeon and time of the operation. Corrective surgery, called a Kasai-portoenterostomy, seeks to establish bile drainage by using a loop of bowel. This is a complicated procedure and is carried out at three specialist centres in England. The cause of biliary atresia is not very clear and despite corrective surgery a large number of children still need liver transplantation.
Neonatal Hepatitis
This group includes most babies with prolonged neonatal jaundice who tend to have coloured stools and after detailed tests are not found to have any definitive cause of jaundice. Most of these babies lose jaundice after a few months and are not left with any long term damage. Vitamin and nutritional supplements are required while the baby is jaundiced.
Neonatal haemochromatosis
Neonatal haemochromatosis is a condition in which iron builds up in all body tissues but particularly the liver, causing hepatitis which progresses so rapidly that the baby often dies within a few days of birth or in many cases is stillborn. Liver transplantation is required. It is not the same gene as haemochromatosis in adults. Recently usage of intravenous immunoglobulins in the antenatal period has been shown to reduce the severity of liver disease in the newborn baby.
Inheritance patterns
Not yet established but there is a very high recurrence in the affected families.
Prenatal diagnosis
Not available, but close follow up of pregnancies at risk may identify an affected fetus in the third trimester. Abnormalities described are placental or fetal oedema.
Viral infections
| Other childhood liver diseases 
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