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Chapter: Paediatrics: Gastroenterology and nutrition

Paediatrics: Liver transplantation

Indications for liver transplantation, Clinical features requiring consideration for transplantation, Post-transplant complications.

Liver transplantation

 

Indications for liver transplantation

 

The commonest underlying conditions leading to irreversible liver failure and transplant are:

Fulminant hepatic failure: e.g. viral, toxic, Wilson’s disease.

 

Biliary atresia.

 

Chronic end-stage liver disease: e.g. post-viral hepatitis with cirrhosis.

 

Liver based metabolic conditions: e.g. Wilson’s disease, α1-antitrypsin deficiency, Crigler–Najjar syndrome, tyrosinaemia.

 

·Acute liver failure following a liver transplant: e.g. primary non-function of transplant or hepatic artery thrombosis.

 

Neonatal hepatitis.

 

Autoimmune hepatitis.

 

Unresectable tumour confined to the liver: e.g. hepatoblastoma.

 

Clinical features requiring consideration for transplantation

 

Bleeding varices due to portal hypertension.

 

Failure of growth or development.

 

Resistant ascites.

 

Hepatic encephalopathy.

 

Poor quality of life: e.g. pruritis, lethargy.

 

Coagulopathy (PTT >2 x normal).

 

Multi-organ failure: e.g. hepatorenal syndrome, hepatopulmonary syndrome.

 

 

Preparation for transplant

 

Requires multidisciplinary evaluation to include the following:

Nutritional support.

 

Development and psychological assessment of child and family.

 

Education and counseling.

 

Ensure vaccinations are current: e.g. MMR, varicella, hepatitis A and B.

 

Cardiac evaluation (ECG, echocardiogram).

 

·Abdominal US (patency of major hepatic blood vessels).

 

Post-transplant complications

 

Primary non-function of the liver (<5%).

 

Hepatic artery thrombosis (10–15%).

 

Biliary leaks and strictures (20%).

 

Acute rejection (50%).

 

Chronic rejection (5–10%).

 

Sepsis (main cause of death).

 

Prognosis

 

Long-term studies indicate normal psychosocial development and quality of life in survivors. Patients require lifelong immunosuppression drug ther-apy, e.g. ciclosporin or tacrolimus.

1yr survival is 90%.

 

   5yr survival is 80%.

 

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Paediatrics: Gastroenterology and nutrition : Paediatrics: Liver transplantation |


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