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Liver disease
Other childhood liver diseases
The following list is not exhaustive but gives examples of other liver conditions which may appear in childhood:
Progressive familial intrahepatic cholestasis (PFIC)
A group of disorders where jaundice and itching are the main symptoms. This usually starts in the first few months of life. The defect usually lies in the transport of bile acids from liver cells to the biliary tree. All the conditions have a genetic basis. It is usual to treat the symptoms but if this fails, then liver transplantation has been successful.
Inheritance patterns
Autosomal recessive.
Prenatal diagnosis
Not available. However antenatal diagnosis is available for families with an affected sibling
Alagille syndrome
This is a rare multisystem disease affecting liver, heart, kidneys with typical facial features (triangular facial expression, deep-set eyes, prominent forehead). The presentation is variable from life threatening heart disease to very mild facial features. The liver disease severity is also variable, from minimal symptoms of itching, and jaundice to the need for liver transplantation in the first few years of life. A genetic defect has been identified but antenatal diagnosis is not routinely available.
Inheritance patterns
Autosomal dominant with variable penetrance.
Prenatal diagnosis
Prenatal diagnosis is possible.
Choledochal cyst
A disorder of the bile ducts, usually presents as abdominal pain, jaundice or recurrent fever. Diagnosis is usually by ultrasound of the liver. The treatment is surgery with good outcome.
Hepatoblastoma
Hepatoblastoma is a liver tumour of childhood. When chemotherapy is combined with surgery the outlook is very good. In a small number of patients where it is not possible to remove the tumour after chemotherapy, liver transplantation is needed.
Autoimmune hepatitis
A rare liver disease of childhood usually presents in teenage but may also affect young children. Mostly non specific symptoms of malaise, lethargy or jaundice. There is usually a family history of autoimmune conditions like thyroiditis, diabetes or arthritis. The diagnosis is based on the presence of autoantibodies in blood with high immunoglobulins and liver biopsy showing variable degree of inflammation. The treatment is immunosuppression with Prednisolone and Azathioprine. The prognosis is good, but treatment is long term.
Budd Chiari syndrome
A very rare liver disease, either seen as a complication after chemotherapy or a result of thrombotic tendency that causes blockage of veins in the liver. Can present as acute liver failure. If supportive treatment fails, then liver transplantation may be necessary.
Galactosaemia
Galactosaemia is a metabolic condition affecting the liver due to absence of an enzyme that metabolises the milk sugar, galactose. The usual presentation is neonatal jaundice with vomiting or septicaemia. The symptoms resolve once galactose is eliminated from the diet.
Inheritance patterns
Autosomal recessive.
Neonatal conditions
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